Science.gov

Sample records for mammo-tomography cmt system

  1. Design, Implementation, and Characterization of a Dedicated Breast Computed Mammo Tomography System for Enhanced Lesion Imaging

    DTIC Science & Technology

    2007-03-01

    with development of a phantom for this purpose (Figure.7). 9 Figure.7 Schematic of cross phantom with 5mm acrylic spheres arranged in a cross...total of 9 spheres are placed on the sheet (graphic is courtesy of Priti Madhav). Cross phantom studies showed that lesion information may be...measurements of similar Varian devices. A phantom was developed specifically for the measurement of 3D MTF, NPS, and resulting DQE (Figure.9

  2. Types of CMT

    MedlinePlus

    ... Meet our Partners How to Get Involved Donate Charcot-Marie-Tooth Disease (CMT) Share print email share facebook twitter google ... get involved? Contact Us Get Our Emails About Charcot-Marie-Tooth Disease (CMT) Types Of Charcot-Marie-Tooth Disease (CMT) ...

  3. An Analysis of the Symptomatic Domains Most Relevant to Charcot Marie Tooth Neuropathy (CMT) Patients

    ClinicalTrials.gov

    2015-04-28

    Charcot Marie Tooth Disease (CMT); Hereditary Sensory and Motor Neuropathy; Nerve Compression Syndromes; Tooth Diseases; Congenital Abnormalities; Genetic Diseases, Inborn; Heredodegenerative Disorders, Nervous System

  4. Detection of the CMT1A/HNPP recombination hotspot in unrelated patients of European descent.

    PubMed Central

    Timmerman, V; Rautenstrauss, B; Reiter, L T; Koeuth, T; Löfgren, A; Liehr, T; Nelis, E; Bathke, K D; De Jonghe, P; Grehl, H; Martin, J J; Lupski, J R; Van Broeckhoven, C

    1997-01-01

    Charcot-Marie-Tooth type 1 disease (CMT1) and hereditary neuropathy with liability to pressure palsies (HNPP) are common inherited disorders of the peripheral nervous system. The majority of CMT1 patients have a 1.5Mb tandem duplication (CMT1A) in chromosome 17p11.2 while most HNPP patients have a deletion of the same 1.5 Mb region. The CMT1A duplication and HNPP deletion are the reciprocal products of an unequal crossing over event between misaligned flanking CMT1A-REP elements. We analysed 162 unrelated CMT1A duplication patients and HNPP deletion patients from 11 different countries for the presence of a recombination hotspot in the CMT1A-REP sequences. A hotspot for unequal crossing over between the misaligned flanking CMT1A-REP elements was observed through the detection of novel junction fragments in 76.9% of 130 unrelated CMT1A patients and in 71.9% of 32 unrelated HNPP patients. This recombination hotspot was also detected in eight out of 10 de novo CMT1A duplication and in two de novo HNPP deletion patients. These data indicate that the hotspot of unequal crossing over occurs in several populations independently of ethnic background and is directly involved in the pathogenesis of CMT1A and HNPP. We conclude that the detection of junction fragments from the CMT1A-REP element on Southern blot analysis is a simple and reliable DNA diagnostic tool for the identification of the CMT1A duplication and HNPP deletion in most patients. Images PMID:9032649

  5. Detection of the CMT1A/HNPP recombination hotspot in unrelated patients of European descent.

    PubMed

    Timmerman, V; Rautenstrauss, B; Reiter, L T; Koeuth, T; Löfgren, A; Liehr, T; Nelis, E; Bathke, K D; De Jonghe, P; Grehl, H; Martin, J J; Lupski, J R; Van Broeckhoven, C

    1997-01-01

    Charcot-Marie-Tooth type 1 disease (CMT1) and hereditary neuropathy with liability to pressure palsies (HNPP) are common inherited disorders of the peripheral nervous system. The majority of CMT1 patients have a 1.5Mb tandem duplication (CMT1A) in chromosome 17p11.2 while most HNPP patients have a deletion of the same 1.5 Mb region. The CMT1A duplication and HNPP deletion are the reciprocal products of an unequal crossing over event between misaligned flanking CMT1A-REP elements. We analysed 162 unrelated CMT1A duplication patients and HNPP deletion patients from 11 different countries for the presence of a recombination hotspot in the CMT1A-REP sequences. A hotspot for unequal crossing over between the misaligned flanking CMT1A-REP elements was observed through the detection of novel junction fragments in 76.9% of 130 unrelated CMT1A patients and in 71.9% of 32 unrelated HNPP patients. This recombination hotspot was also detected in eight out of 10 de novo CMT1A duplication and in two de novo HNPP deletion patients. These data indicate that the hotspot of unequal crossing over occurs in several populations independently of ethnic background and is directly involved in the pathogenesis of CMT1A and HNPP. We conclude that the detection of junction fragments from the CMT1A-REP element on Southern blot analysis is a simple and reliable DNA diagnostic tool for the identification of the CMT1A duplication and HNPP deletion in most patients.

  6. Proceedings of the workshop on high resolution computed microtomography (CMT)

    SciTech Connect

    1997-02-01

    The purpose of the workshop was to determine the status of the field, to define instrumental and computational requirements, and to establish minimum specifications required by possible users. The most important message sent by implementers was the remainder that CMT is a tool. It solves a wide spectrum of scientific problems and is complementary to other microscopy techniques, with certain important advantages that the other methods do not have. High-resolution CMT can be used non-invasively and non-destructively to study a variety of hierarchical three-dimensional microstructures, which in turn control body function. X-ray computed microtomography can also be used at the frontiers of physics, in the study of granular systems, for example. With high-resolution CMT, for example, three-dimensional pore geometries and topologies of soils and rocks can be obtained readily and implemented directly in transport models. In turn, these geometries can be used to calculate fundamental physical properties, such as permeability and electrical conductivity, from first principles. Clearly, use of the high-resolution CMT technique will contribute tremendously to the advancement of current R and D technologies in the production, transport, storage, and utilization of oil and natural gas. It can also be applied to problems related to environmental pollution, particularly to spilling and seepage of hazardous chemicals into the Earth's subsurface. Applications to energy and environmental problems will be far-ranging and may soon extend to disciplines such as materials science--where the method can be used in the manufacture of porous ceramics, filament-resin composites, and microelectronics components--and to biomedicine, where it could be used to design biocompatible materials such as artificial bones, contact lenses, or medication-releasing implants. Selected papers are indexed separately for inclusion in the Energy Science and Technology Database.

  7. Genetics of Charcot-Marie-Tooth (CMT) Disease within the Frame of the Human Genome Project Success.

    PubMed

    Timmerman, Vincent; Strickland, Alleene V; Züchner, Stephan

    2014-01-22

    Charcot-Marie-Tooth (CMT) neuropathies comprise a group of monogenic disorders affecting the peripheral nervous system. CMT is characterized by a clinically and genetically heterogeneous group of neuropathies, involving all types of Mendelian inheritance patterns. Over 1,000 different mutations have been discovered in 80 disease-associated genes. Genetic research of CMT has pioneered the discovery of genomic disorders and aided in understanding the effects of copy number variation and the mechanisms of genomic rearrangements. CMT genetic study also unraveled common pathomechanisms for peripheral nerve degeneration, elucidated gene networks, and initiated the development of therapeutic approaches. The reference genome, which became available thanks to the Human Genome Project, and the development of next generation sequencing tools, considerably accelerated gene and mutation discoveries. In fact, the first clinical whole genome sequence was reported in a patient with CMT. Here we review the history of CMT gene discoveries, starting with technologies from the early days in human genetics through the high-throughput application of modern DNA analyses. We highlight the most relevant examples of CMT genes and mutation mechanisms, some of which provide promising treatment strategies. Finally, we propose future initiatives to accelerate diagnosis of CMT patients through new ways of sharing large datasets and genetic variants, and at ever diminishing costs.

  8. Causes of Charcot-Marie-Tooth Disease (CMT)

    MedlinePlus

    ... motor and sensory problems in the body’s extremities. Inheritance patterns in CMT Although CMT can look very similar ... from a parent will have the disease, as will the parent. When CMT is passed on in an autosomal dominant pattern, it can be easy to recognize in the ...

  9. New mutations in CMT 1 and HNPP

    SciTech Connect

    Vandenberghe, A.; Boucherat, M.; Bonnebouche, C.

    1994-09-01

    The majority of mutations in CMT 1 (Charcot-Marie-Tooth disease type 1) are due to a duplication of a 1.5 Mb fragment from chromosome 17 containing the PMP22 myelin gene. In addition, micromutations are found in the genes for PMP22 and myelin Po. We collected data from over one hundred families with a duplication in 17p11.2. In about 10% of these families, a de novo mutation was observed. All parents were clinically examined as normal and correct paternity was confirmed. Some families were informative for polymorphic probes located in the duplicated region, and we could deduce a majority of new mutations to be from paternal origin. HNPP (hereditary neuropathy with liability to pressure palsies) is believed to be the reciprocal product of an unequal crossing over underlying the CMT 1 mutation and is due to a deletion of the 1.5 Mb fragment. One new HNPP mutation was found among 7 deleted HNPP families. This mutation is of paternal origin. Clinically assigned CMT 1 patients without a duplication are screened for micromutations applying the SSCP technique. In one family, a de novo mutation was found in the gene for Po.

  10. Analysis of the CMT1A-REP repeat: mapping crossover breakpoints in CMT1A and HNPP.

    PubMed

    Kiyosawa, H; Lensch, M W; Chance, P F

    1995-12-01

    The CMT1A-REP repeat sequence flanks a 1.5 megabase pair (Mb) segment of chromosome 17p11.2-12 which is duplicated in Charcot-Marie-Tooth neuropathy type 1A (CMT1A) and deleted in hereditary neuropathy with liability to pressure palsies (HNPP). The CMT1A-REP repeat is proposed to mediate misalignment and unequal crossover resulting in reciprocal chromosomal rearrangements in CMT1A and HNPP. We have constructed a physical map of the proximal and distal CMT1A-REP repeats. Cloned fragments from CMT1A-REP repeat regions are used to determine the size of the repeats and assess regions of homology. The crossover breakpoints were mapped in series of 30 unrelated CMT1A patients and 22 unrelated HNPP patients. The CMT1A-REP repeat spans approximately 27 kilobase pairs and appears to be continuous. Locations of restriction enzyme sites are highly conserved for the proximal and distal CMT1A-REP repeats. All crossovers mapped within the CMT1A-REP repeat sequence and heterogeneity for breakpoint location demonstrated. Seventy-seven percent (40 to 52) of CMT1A and HNPP chromosomes contained breakpoints which mapped within a 7.9 kb interval, suggesting the presence of a possible 'hotspot'for recombination in CMT1A-REP. DNA sequence analysis for 4 kb of the interval containing the majority of crossovers revealed over 98% sequence identity between proximal and distal CMT1A-REP repeat sequences. Probes useful for molecular-based diagnosis of CMT1A and HNPP are described.

  11. Charcot-Marie-Tooth disease type 1A (CMT1A) and hereditary neuropathy with liability to pressure palsies (HNPP): reliable detection of the CMT1A duplication and HNPP deletion using 8 microsatellite markers in 2 multiplex PCRs.

    PubMed

    Seeman, P; Mazanec, R; Zidar, J; Hrusáková, S; Ctvrtecková, M; Rautenstrauss, B

    2000-10-01

    Charcot-Marie-Tooth disease (CMT) and hereditary neuropathy with liability to pressure palsies (HNPP) are the most frequent inherited disorders of the peripheral nervous system. They are clinically and genetically heterogeneous. A submicroscopic tandem duplication of 1. 5 Mb in chromosome 17p11.2-12 comprising the PMP22 gene is found in 70.7% of autosomal dominant Charcot-Marie-Tooth type 1 (CMT1) patients. A reciprocal deletion is found in 87.6% of HNPP patients. The size of the typical CMT1A duplication is too small for classical cytogenetics and the whole region including the CMT1A-REP elements is sometimes too complex for a single DNA analysis method. We present results of a multiplex PCR of 8 microsatellite markers with multicolour fluorescence primer labelling followed by fragment analysis on an ABI 310 Prism analyzer to simplify the diagnostic procedure. Results for 24 patients can be obtained within 24 h. This method was applied on 92 DNA samples of unrelated patients carrying a typical CMT1A duplication previously confirmed by two colour fluorescence in situ hybridization (FISH, probe c132G8) and EcoRI/SacI Southern blotting (probe pLR7.8). Three alleles of three different sizes were clearly detected at least once in 88 of them (95.6%). Subsequently this analysis was applied on 312 Czech patients and revealed a CMT1A/HNPP rearrangement in 109 out of them.

  12. Physical and transcriptional map in the CMT 1A region

    SciTech Connect

    Chevillard, C.; Passage, E.; Cudrey, C.

    1994-09-01

    The Charcot-Marie-Tooth disease type 1A (CMT1A) has been mapped to the proximal short arm of chromosome 17. CMT1A is the most frequent of the motor and sensory peripheral neuropathies and is associated with a duplication of a 1.5 Mb fragment in proximal 17p12. Several groups have proposed that the gene coding for peripheral myelin protein-22 (PMP-22) as the candidate gene for CMT1A. We have recently published a {open_quote}MegaYAC{close_quote} contig of 6 Mb which covers the CMT1A critical region. In order to isolate new genes localized in this region, we used a {open_quote}physical trapping {close_quote} strategy derived from the direct cDNA selection technique developed by Parimoo et al. This approach has allowed us to construct cDNA {open_quotes}minilibraries{close_quotes} using YAC DNA from the CMT1A region. One of the clones in these minilibraries has been mapped back to the CMT1A duplication. Other potentially interesting clones are in the process of further characterization. Furthermore, we have mapped several Genethon microsatellites in the 6 Mb YAC contig and some are located in the CMT1A duplicated region. These highly polymorphic markers should prove useful for diagnostic testing in CMT1A.

  13. Preparation and characterization of 4-dedimethylamino sancycline (CMT-3) loaded nanostructured lipid carrier (CMT-3/NLC) formulations.

    PubMed

    Yang, Xiaomin; Zhao, Lin; Almasy, Laszlo; Garamus, Vasil M; Zou, Aihua; Willumeit, Regine; Fan, Saijun

    2013-06-25

    Chemically modified tetracyclines (CMTs) have been reported to strongly inhibit proliferation and metastasis of various cancers, but their efficacy is restricted by poor water solubility. In the present study, a hydrophilic 4-dedimethylamino sancycline (CMT-3) loaded nanostructured lipid carrier (CMT-3/NLC) was produced by high pressure homogenization (HPH). The physical properties of CMT-3/NLC formulations were characterized by dynamic light scattering (DLS), high efficiency liquid chromatography (HPLC), atomic force microscopy (AFM), scanning electron microscopy (SEM), small-angle neutron scattering (SANS), small-angle X-ray scattering (SAXS) and wide-angle X-ray powder diffraction (XRD). The lipid and surfactant ingredients, as well as drug/lipid concentrations (m/m) were optimized to produce stable and sustained NLC formulations. In vitro cytotoxicity of CMT-3/NLC against HeLa cells was evaluated by MTT assay. The diameter of CMT-3/NLC was found to increase from 153.1±3.0 nm to a maximum of 168.5±2.0 nm after 30 days of storage, while the entrapment efficiency remained constant at >90%. CMT-3/NLC demonstrated a burst-sustained release profile in release media with different pH, a property attributed to the 3-dimensional structure of CMT-3/NLC. Cell uptake and localization studies indicated that NLC reached the cytoplasm and could thereby facilitate CMT-3 entry into HeLa cells.

  14. First Carlsberg Meridian Telescope (CMT) CCD Catalogue.

    NASA Astrophysics Data System (ADS)

    Bélizon, F.; Muiños, J. L.; Vallejo, M.; Evans, D. W.; Irwin, M.; Helmer, L.

    2003-11-01

    The Carlsberg Meridian Telescope (CMT) is a telescope owned by Copenhagen University Observatory (CUO). It was installed in the Spanish observatory of El Roque de los Muchachos on the island of La Palma (Canary Islands) in 1984. It is operated jointly by the CUO, the Institute of Astronomy, Cambridge (IoA) and the Real Instituto y Observatorio de la Armada of Spain (ROA) in the framework of an international agreement. From 1984 to 1998 the instrument was provided with a moving slit micrometer and with its observations a series of 11 catalogues were published, `Carlsberg Meridian Catalogue La Palma (CMC No 1-11)'. Since 1997, the telescope has been controlled remotely via Internet. The three institutions share this remote control in periods of approximately three months. In 1998, the CMT was upgraded by installing as sensor, a commercial Spectrasource CCD camera as a test of the possibility of performing meridian transits observed in drift-scan mode. Once this was shown possible, in 1999, a second model of CCD camera, built in the CUO workshop with a better performance, was installed. The Spectrasource camera was loaned to ROA by CUO and is now installed in the San Fernando Automatic Meridian Circle in San Juan (CMASF). In 1999, the observations were started of a sky survey from -3deg to +30deg in declination. In July 2002, a first release of the survey was published, with the positions of the observed stars in the band between -3deg and +3deg in declination. This oral communication will present this first release of the survey.

  15. Molecular analyses of unrelated Charcot-Marie-Tooth (CMT) disease patients suggest a high frequency of the CMT1A duplication

    SciTech Connect

    Wise, C.A.; Davis, S.N.; Heju, Z.; Pentao, L.; Patel, P.I.; Lupski, J.R. ); Garcia, C.A. )

    1993-10-01

    Charcot-Marie-Tooth disease (CMT) is the most common inherited peripheral neuropathy. One form of CMT, CMT type 1A, is characterized by uniformly decreased nerve conduction velocities, usually shows autosomal dominant inheritance, and is associated with a large submicroscopic duplication of the p11.2-p12 region of chromosome 17. A cohort of 75 unrelated patients diagnosed clinically with CMT and evaluated by electrophysiological methods were analyzed molecularly for the presence of the CMT1A DNA duplication. Three methodologies were used to assess the duplication: Measurement of dosage differences between RFLP alleles, analysis of polymorphic (GT)[sub n] repeats, and detection of a junction fragment by pulsed-field gel electrophoresis. The CMT1A duplication was found in 68% of the 63 unrelated CMT patients with electrophysiological studies consistent with CMT type 1 (CMT1). The CMT1A duplication was detected as a de novo event in two CMT1 families. Twelve CMT patients who did not have decreased nerve conduction velocities consistent with a diagnosis of CMT type 2 (CMT2) were found not to have the CMT1A duplication. The most informative molecular method was the detection of the CMT1A duplication-specific junction fragment. Given the high frequency of the CMT1A duplication in CMT patients and the high frequency of new mutations, the authors conclude that a molecular test for the CMT1A DNA duplication is very useful in the differential diagnosis of patients with peripheral neuropathies. 61 refs., 4 figs.

  16. CMT4D (NDRG1 mutation): genotype-phenotype correlations.

    PubMed

    Ricard, Emilie; Mathis, Stéphane; Magdelaine, Corinne; Delisle, Marie-Bernadette; Magy, Laurent; Funalot, Benoît; Vallat, Jean-Michel

    2013-09-01

    Charcot-Marie-Tooth (CMT) disease is a heterogeneous condition with a large number of clinical, electrophysiological and pathological phenotypes. More than 40 genes are involved. We report a child of gypsy origin with an autosomal recessive demyelinating phenotype. Clinical data, familial history, and electrophysiological studies were in favor of a CMT4 sub-type. The characteristic N-Myc downstream-regulated gene 1 (NDRG1) mutation responsible for this CMT4D phenotype was confirmed: p.R148X. The exact molecular function of the NDRG1 protein has yet to be elucidated.

  17. A Family Harboring CMT1A Duplication and HNPP Deletion.

    PubMed

    Lee, Jung Hwa; Kang, Hee Jin; Song, Hyunseok; Hwang, Su Jin; Cho, Sun-Young; Kim, Sang-Beom; Kim, Joonki; Chung, Ki Wha; Choi, Byung-Ok

    2007-06-01

    Charcot-Marie-Tooth disease type 1A (CMT1A) is associated with duplication of chromosome 17p11.2-p12, whereas hereditary neuropathy with liability to pressure palsies (HNPP), which is an autosomal dominant neuropathy showing characteristics of recurrent pressure palsies, is associated with 17p11.2-p12 deletion. An altered gene dosage of PMP22 is believed to the main cause underlying the CMT1A and HNPP phenotypes. Although CMT1A and HNPP are associated with the same locus, there has been no report of these two mutations within a single family. We report a rare family harboring CMT1A duplication and HNPP deletion.

  18. CMT for biomedical and other applications

    SciTech Connect

    Spanne, P.

    1997-02-01

    This session includes two presentations describing applications for x-ray tomography using synchrotron radiation for biomedical uses and fluid flow modeling, and outlines advantages for using monoenergetic x-rays. Contrast mechanisms are briefly described and several graphs of absorbed doses and scattering of x-rays are included. Also presented are schematic diagrams of computerized tomographic instrumentation with camera head. A brief description of goals for a real time tomographic system and expected improvements to the system are described. Color photomicrographs of the Berea Sandstone and human bone are provided, as well as a 3-D microtomographic reconstruction of a human vertebra sample.

  19. Locations of crossover breakpoints within the CMT1A-REP repeat in Japanese patients with CMT1A and HNPP.

    PubMed

    Yamamoto, M; Yasuda, T; Hayasaka, K; Ohnishi, A; Yoshikawa, H; Yanagihara, T; Ikegami, T; Yamamoto, T; Ohashi, H; Nishimura, T; Mitsuma, T; Kiyosawa, H; Chance, P F; Sobue, G

    1997-02-01

    The crossover breakpoints for Charcot-Marie-Tooth disease type 1A (CMT1A) and hereditary neuropathy with liability to pressure palsies (HNPP) are located in the CMT1A-REP repeat flanking a 1.5-Mb region of chromosome 17p11.2-12. The precise locations of the breakpoints are heterogeneous, and we analyzed the relative frequency distribution of breakpoints in 33 unrelated Japanese CMT1A and 3 unrelated HNPP families. The CMT1A-REP repeat region was divided into five regions, A, B, C, D and E, based on restriction site differences between the proximal and distal CMT1A-REP repeats. The frequency distribution of breakpoints within the CMT1A-REP repeat in the Japanese patients was 3% in region A, .78% in B/C and 19% in D, which is similar to that in Caucasian patients. This result also indicates that an 8-kb region defined by region B/C is a recombinational hotspot within the CMT1A-REP repeat in Japanese patients.

  20. CHANNEL MORPHOLOGY TOOL (CMT): A GIS-BASED AUTOMATED EXTRACTION MODEL FOR CHANNEL GEOMETRY

    SciTech Connect

    JUDI, DAVID; KALYANAPU, ALFRED; MCPHERSON, TIMOTHY; BERSCHEID, ALAN

    2007-01-17

    This paper describes an automated Channel Morphology Tool (CMT) developed in ArcGIS 9.1 environment. The CMT creates cross-sections along a stream centerline and uses a digital elevation model (DEM) to create station points with elevations along each of the cross-sections. The generated cross-sections may then be exported into a hydraulic model. Along with the rapid cross-section generation the CMT also eliminates any cross-section overlaps that might occur due to the sinuosity of the channels using the Cross-section Overlap Correction Algorithm (COCoA). The CMT was tested by extracting cross-sections from a 5-m DEM for a 50-km channel length in Houston, Texas. The extracted cross-sections were compared directly with surveyed cross-sections in terms of the cross-section area. Results indicated that the CMT-generated cross-sections satisfactorily matched the surveyed data.

  1. Automated and Rapid Determinations of Earthquake Source Parameters in Indonesia: Comparisons with Global CMT Solutions

    NASA Astrophysics Data System (ADS)

    Nakano, M.; Yamashina, T.; Kumagai, H.; Inoue, H.; S.; F.

    2008-12-01

    receiving initial hypocenter information from the GEOFON email alert. Using the displacement seismograms with a total length of 512 s and Green's functions stored in a library, the inversion is performed to estimate the source parameters. Finally, when the result is judged sufficiently accurate, the estimated source parameters are displayed on our web server (http://www.isn.bosai.go.jp/en/index.html). Using the inversion method, we created a CMT catalogue for earthquakes in Indonesia that occurred between July 2006 and May 2008. We obtained CMT solutions for 180 earthquakes with the moment magnitude larger than 5. We compared the source parameters with those obtained by the GCMT project. The average differences in the horizontal source location and depth are 40.7 km and -5.6 km, respectively. The horizontal difference corresponds to twice the grid spacing of the grid search. The moment magnitudes obtained by our method are almost identical to those estimated by the GCMT project. These results indicate that our CMT solutions are consistent with those obtained by the GCMT project. The average time required for the source parameter estimations by this system is 13 minutes after the occurrence of earthquakes, which is much shorter than that required for CMT estimations based on global seismic networks. Seismic monitoring based on our inversion system provides early notification of detailed characterizations of earthquakes including the moment function, which may be useful for identification of tsunami earthquakes and can provide supporting information for tsunami warnings.

  2. Genetic and physical mapping of the genomic region spanning CMT4A

    SciTech Connect

    Othmane, K.B.; Loeb, D.; Roses, A.D.

    1994-09-01

    Autosomal recessive Charcot-Marie-Tooth disease (CMT4) is a severe childhood neuropathy classified into three types: A, B, and C. We previously mapped CMT4A to chromosome 8q13-q21 in four large Tunisian families. Analysis of recombination events suggested the order: cent.-D8S279-(D8S286,D8S164, CMT4A)-D8S84-tel. Families with types B and C were subsequently typed and linkage for these types was excluded for the CMT4A region and other known CMT loci. Recently, the gene for a major peripheral myelin protein (PMP2) was mapped by FISH to chromosome 8q21-q22 and therefore appeared to be a strong candidate gene for CMT4A. We used SSCP analysis, DNA sequencing, FISH and YAC mapping analysis, and demonstrated that PMP2 is not the defect in CMT4A. Using physical mapping data, we sublocalized a new genethon marker (D8S548) to the CMT4A region between D8S286 and D8S164. All affected CMT4A patients were homozygotes for this polymorphic microsatellite as expected from its physical localization. We screened the CEPH megabase YAC library using the closest markers; over 30 YACs were isolated and characterized by PFGE. FISH analysis revealed about 16% chimeras. The YACs span the 8 cM region between D8S279 and PMP2 (mapped distal to D8S84), with a current 1 cM gap between D8S164 and D8S84. We are currently using Alu-PCR and vectorette to develop end clones in order to identify new YACs in the region and further close this gap. Alu-PCR fragments have identified several new microsatellites in the region which can be used for additional mapping of the CMT4A gene.

  3. Transgenic mouse models of CMT1A and HNPP.

    PubMed

    Suter, U; Nave, K A

    1999-09-14

    We have generated several PMP22 animal mutants with altered PMP22 gene dosage. A moderate increase in the number of PMP22 genes led to hypomyelination comparable to CMT1A, whereas high copy numbers of transgenic PMP22 resulted in phenotypes resembling more severe forms of hereditary motor and sensory neuropathies. In contrast, eliminating one of the two normal PMP22 genes by gene targeting caused unstable focal hypermyelination (tomacula) similar to the pathology in HNPP. A related but more severe phenotype was observed in mice that lack PMP22 completely. Detailed analysis of the different PMP22 mutants revealed, in addition to the obvious myelinopathy, distal axonopathy as a characteristic feature. We conclude that the maintenance of axons might be a promising target for therapeutic interventions in these demyelinating hereditary neuropathies. Furthermore, our results strongly support the concept that PMP22-related neuropathies (and most likely also other forms of inherited motor and sensory neuropathies) should be viewed as the consequence of impaired neuron-Schwann cell interactions that are likely already to be operative during development. Such considerations should be taken into account in the design of potential novel treatment strategies.

  4. Molecular mechanisms for CMT1A duplication and HNPP deletion.

    PubMed

    Boerkoel, C F; Inoue, K; Reiter, L T; Warner, L E; Lupski, J R

    1999-09-14

    As the best characterized human genomic disorders, CMT1A and HNPP illustrate several common mechanistic features of genomic rearrangements. These features include the following: (1) Recombination occurs between homologous sequences flanking the duplicated/deleted genomic segment. (2) The evolution of the mammalian genome may result in an architecture consisting of region-specific low-copy repeats that predispose to rearrangement secondary to providing homologous regions as substrate for recombination. (3) Strand exchange occurs preferentially in a region of perfect sequence identity within the flanking repeat sequences. (4) Double-strand breaks likely initiate recombination between the flanking repeats. (5) The mechanism and rate of homologous recombination resulting in DNA rearrangement may differ for male and female gametogenesis. (6) Homologous recombination resulting in DNA rearrangement occurs with high frequency in the human genome. (7) Genomic disorders result from structural features of the human genome and not population specific alleles or founder effects; therefore, genomic disorders appear to occur with equal frequencies in different world populations.

  5. Reliability of the CMT neuropathy score (second version) in Charcot-Marie-Tooth disease.

    PubMed

    Murphy, Sinéad M; Herrmann, David N; McDermott, Michael P; Scherer, Steven S; Shy, Michael E; Reilly, Mary M; Pareyson, Davide

    2011-09-01

    The Charcot-Marie-Tooth neuropathy score (CMTNS) is a reliable and valid composite score comprising symptoms, signs, and neurophysiological tests, which has been used in natural history studies of CMT1A and CMT1X and as an outcome measure in treatment trials of CMT1A. Following an international workshop on outcome measures in Charcot-Marie-Tooth disease (CMT), the CMTNS was modified to attempt to reduce floor and ceiling effects and to standardize patient assessment, aiming to improve its sensitivity for detecting change over time and the effect of an intervention. After agreeing on the modifications made to the CMTNS (CMTNS2), three examiners evaluated 16 patients to determine inter-rater reliability; one examiner evaluated 18 patients twice within 8 weeks to determine intra-rater reliability. Three examiners evaluated 63 patients using the CMTNS and the CMTNS2 to determine how the modifications altered scoring. For inter- and intra-rater reliability, intra-class correlation coefficients (ICCs) were ≥0.96 for the CMT symptom score and the CMT examination score. There were small but significant differences in some of the individual components of the CMTNS compared with the CMTNS2, mainly in the components that had been modified the most. A longitudinal study is in progress to determine whether the CMTNS2 is more sensitive than the CMTNS for detecting change over time.

  6. Rapid diagnosis of CMT1A duplications and HNPP deletions by multiplex microsatellite PCR.

    PubMed

    Choi, Byung-Ok; Kim, Joonki; Lee, Kyung Lyong; Yu, Jin Seok; Hwang, Jung Hee; Chung, Ki Wha

    2007-02-28

    Charcot-Marie-Tooth (CMT) disease and hereditary neuropathy with liability to pressure palsies (HNPP) are frequent forms of genetically heterogeneous peripheral neuropathies. Reciprocal unequal crossover between flanking CMT1A-REPs on chromosome 17p11.2-p12 is a major cause of CMT type 1A (CMT1A) and HNPP. The importance of a sensitive and rapid method for identifying the CMT1A duplication and HNPP deletion is being emphasized. In the present study, we established a molecular diagnostic method for the CMT1A duplication and HNPP deletion based on hexaplex PCR of 6 microsatellite markers (D17S921, D17S9B, D17S9A, D17S918, D17S4A and D17S2230). The method is highly time-, cost- and sample-saving because the six markers are amplified by a single PCR reaction and resolved with a single capillary in 3 h. Several statistical and forensic estimates indicated that most of these markers are likely to be useful for diagnosing the peripheral neuropathies. Reproducibility, as determined by concordance between independent tests, was estimated to be 100%. The likelihood that genotypes of all six markers are homozygous in randomly selected individuals was calculated to be 1.6 x 10(-4) which indicates that the statistical error rate for this diagnosis of HNPP deletion is only 0.016%.

  7. Validation of the CMT Pediatric Scale as an outcome measure of disability

    PubMed Central

    Burns, Joshua; Ouvrier, Robert; Estilow, Tim; Shy, Rosemary; Laurá, Matilde; Pallant, Julie F.; Lek, Monkol; Muntoni, Francesco; Reilly, Mary M.; Pareyson, Davide; Acsadi, Gyula; Shy, Michael E.; Finkel, Richard S.

    2012-01-01

    Objective Charcot-Marie-Tooth disease (CMT) is a common heritable peripheral neuropathy. There is no treatment for any form of CMT although clinical trials are increasingly occurring. Patients usually develop symptoms during the first two decades of life but there are no established outcome measures of disease severity or response to treatment. We identified a set of items that represent a range of impairment levels and conducted a series of validation studies to build a patient-centered multi-item rating scale of disability for children with CMT. Methods As part of the Inherited Neuropathies Consortium, patients aged 3–20 years with a variety of CMT types were recruited from the USA, UK, Italy and Australia. Initial development stages involved: definition of the construct, item pool generation, peer review and pilot testing. Based on data from 172 patients, a series of validation studies were conducted, including: item and factor analysis, reliability testing, Rasch modeling and sensitivity analysis. Results Seven areas for measurement were identified (strength, dexterity, sensation, gait, balance, power, endurance), and a psychometrically robust 11-item scale constructed (Charcot-Marie-Tooth disease Pediatric Scale: CMTPedS). Rasch analysis supported the viability of the CMTPedS as a unidimensional measure of disability in children with CMT. It showed good overall model fit, no evidence of misfitting items, no person misfit and it was well targeted for children with CMT. Interpretation The CMTPedS is a well-tolerated outcome measure that can be completed in 25-minutes. It is a reliable, valid and sensitive global measure of disability for children with CMT from the age of 3 years. PMID:22522479

  8. Al-Si-Mn Alloy Coating on Aluminum Substrate Using Cold Metal Transfer (CMT) Welding Technique

    NASA Astrophysics Data System (ADS)

    Rajeev, G. P.; Kamaraj, M.; Bakshi, S. R.

    2014-06-01

    The cold metal transfer (CMT) process was explored as a weld overlay technique for synthesizing Al-Si-Mn alloy coating on a commercially pure Al plate. The effect of welding speed on the bead geometry, deposition rate, and the dilution were studied and the best parameter was used to synthesize the coatings. The CMT process can be used to produce thick coatings (>2.5 mm) without porosity and with low dilution levels. The Vickers hardness number of the Al substrate increased from 28 in the bulk to 57 in the coating. It is suggested that the CMT process can be an effective and energy-efficient technique for depositing thick coatings and is useful in weld repair of aluminum alloy components.

  9. Factors Affecting Phenotype Variability in a Family with CMT2B: Gender and LRSAM1 Genotype

    PubMed Central

    Peddareddygari, Leema Reddy; Oberoi, Kinsi; Vellore, Jaasrini Reddy; Grewal, Raji P.

    2016-01-01

    Charcot-Marie-Tooth disease type 2 (CMT2) is an autosomal dominant axonal neuropathy caused by mutations in various genes. The subtype CMT2B results from missense mutations in RAB7A, member RAS oncogene family gene, whereas missense mutations in the Leucine-rich repeat and sterile alpha motif-containing protein 1 (LRSAM1) gene cause CMT2P. We describe the genotype/phenotype analysis of a family in which a previously described mutation in the RAB7A gene and a novel mutation in the LRSAM1 gene were identified. In this family, none of the individuals had ulceromutilating features, and there was a marked variability in the age of onset. We discuss the possible etiology of the observed phenotypic variability including the role of gender and possible RAB7A/LRSAM1 gene interactions. PMID:27462242

  10. Diagnostic laboratory testing for Charcot Marie Tooth disease (CMT): the spectrum of gene defects in Norwegian patients with CMT and its implications for future genetic test strategies

    PubMed Central

    2013-01-01

    Background Current genetic test algorithms for Charcot Marie Tooth (CMT) disease are based on family details and comprehensive clinical and neurophysiological data gathered under ideal conditions for clinical assessment. However, in a diagnostic laboratory setting relying on external test requisitions and patient samples, such conditions are not always met. Our objective was therefore to perform a retrospective evaluation of the data given in laboratory request forms and to assess their quality and applicability with regard to the recommended algorithms for CMT diagnostics. As we are the main test centre for CMT in Norway our results also provide an overview of the spectrum of gene defects in the Norwegian CMT population. Methods Genetic testing was performed according to polyneuropathy type; demyelinating/mixed: PMP22 duplication, MPZ, EGR2, LITAF, NEFL, PMP22, GJB1, axonal: MFN2, MPZ, NEFL, and GJB1. Results Diagnostic testing of index patients was requested in 435 of the 549 cases. Seventy-two (16.6%) positive molecular genetic findings were made. The majority (94.6%) of mutation positive cases showed disease onset before 50 years of age. PMP22 (duplication), MPZ, GJB1 and MFN2 mutations constituted 95.8% of the positive findings. Within the nerve conduction study groups, mutation detection rates were; demyelinating 33.8%; mixed 29.0%; axonal 8.8%; unspecified 16.5%. Conclusion We suggest a simplified algorithm intended for referral centres, dealing with DNA/blood samples, which involves the assessment of age at onset and neurophysiological data followed by testing of four genes; PMP22 (duplication), MPZ, GJB1 and MFN2. Patients negative for mutations in those four genes should be subjected to evaluation at an interdisciplinary inherited neuropathy clinic with the capacity for extended molecular genetic analysis by next generation sequencing. PMID:24053775

  11. MFN2 point mutations occur in 3.4% of Charcot-Marie-Tooth families. An investigation of 232 Norwegian CMT families

    PubMed Central

    2010-01-01

    Background Point mutations in the mitofusin 2 (MFN2) gene has been identified exclusively in Charcot-Marie-Tooth type 2 (CMT2), and in a single family with intermediate CMT. MFN2 point mutations are probably the most common cause of CMT2. Methods Two-hundred and thirty-two consecutive unselected and unrelated CMT families with available DNA from all regions in Norway were included. We screened for point mutations in the MFN2 gene. Results We identified four known and three novel point mutations in 8 unrelated CMT families. The novel point mutations were not found in 100 healthy controls. This corresponds to 3.4% (8/232) of CMT families have point mutations in the MFN2 gene. The phenotypes were compatible with CMT1 in two families, CMT2 in four families, intermediate CMT in one family and distal Hereditary Motor Neuropathy (dHMN) in one family. This corresponds to 2.3% of CMT1, 5.5% of CMT2, 12.5% of intermediate CMT and 6.7% of dHMN families have a point mutation in the MFN2 gene. Point mutations in the MFN2 gene is likely to be the fourth most common cause to CMT after duplication of the peripheral myelin protein 22 (PMP22) gene, and point mutations in the Connexin32 (Cx32) and myelin protein zero (MPZ) genes. Conclusions The identified known and novel point mutations in the MFN2 gene expand the clinical spectrum from CMT2 and intermediate CMT to also include possibly CMT1 and the dHMN phenotypes. Thus, genetic analyses of the MFN2 gene should not be restricted to persons with CMT2. PMID:20350294

  12. Copy number variations are a rare cause of non-CMT1A Charcot-Marie-Tooth disease.

    PubMed

    Huang, Jia; Wu, Xingyao; Montenegro, Gladys; Price, Justin; Wang, Gaofeng; Vance, Jeffery M; Shy, Michael E; Züchner, Stephan

    2010-05-01

    Hereditary peripheral neuropathies present a group of clinically and genetically heterogeneous entities. All known forms, including the various forms of Charcot-Marie-Tooth disease (CMT) are characterized as Mendelian traits and over 35 genes have been identified thus far. The mutational mechanism of the most common CMT type, CMT1A, is a 1.5 Mb chromosomal duplication at 17p12 that contains the gene PMP22. Only recently it has been realized that such copy number variants (CNV) are a widespread phenomenon and important for disease. However, it is not known whether CNVs play a wider role in hereditary peripheral neuropathies outside of CMT1A. In a phenotypically heterogeneous sample of 97 patients, we performed the first high-density CNV study of 34 genomic regions harboring known genes for hereditary peripheral neuropathies including the 17p12 duplication region, with comparative genomic hybridization (CGH) microarrays. We identified three CNVs that affected coding exons. A novel shorter form of a PMP22 duplication was detected in a CMT1A family previously tested negative in a commercial test. Two other CNVs in MTMR2 and ARHGEF10 are likely not disease associated. Our results indicate that CNVs are a rare cause for non-CMT1A CMT. Their potential relevance as disease modifiers remains to be evaluated. The present study design cannot rule out that specific CMT forms exist where CNVs play a larger role.

  13. Metagenome and metatranscriptome data for Rifle CMT-03 laboratory microcosm experiment completed in April 2014

    DOE Data Explorer

    Jewell, Talia [Lawrence Berkeley National Lab. (LBNL), Berkeley, CA (United States); Karaoz, Ulas [Lawrence Berkeley National Lab. (LBNL), Berkeley, CA (United States); Bill, Markus [Lawrence Berkeley National Lab. (LBNL), Berkeley, CA (United States); Chakraborty, Romy [Lawrence Berkeley National Lab. (LBNL), Berkeley, CA (United States); Brodie, Eoin L [Lawrence Berkeley National Lab. (LBNL), Berkeley, CA (United States); Williams, Kenneth Hurst [Lawrence Berkeley National Lab. (LBNL), Berkeley, CA (United States); Beller, Harry R [Lawrence Berkeley National Lab. (LBNL), Berkeley, CA (United States)

    2014-04-01

    Sediment samples were collected during installation of monitoring borehole CMT-03. Microcosms were constructed and inoculated under anerobic conditions with these sediments and anaerobic Rifle artificial groundwater. Microcosm metagenomes and metatranscriptomes were sampled every 5 days for a period of 20 days. The dataset gives gene-level annotations, binning, metagenomic and metatranscriptomic coverages for these microcosms.

  14. Topically applied CMT-2 enhances wound healing in streptozotocin diabetic rat skin.

    PubMed

    Ramamurthy, N S; Kucine, A J; McClain, S A; McNamara, T F; Golub, L M

    1998-11-01

    Delayed wound healing is one of the complications of diabetes mellitus, exhibited by increased wound collagenase and decreased granulation tissues. The current study compared wound healing in normal and diabetic rats, and the effects of topically applied 1% or 3% concentrations of chemically modified tetracycline-2 (CMT-2) on 6-mm circular full-thickness skin wounds healed by secondary intention. On day 7 after wounding, tissues were removed for biochemical analysis and histology. The wound granulation tissue hydroxyproline was less in the untreated diabetic rat with increased collagenase and gelatinase. Treating the diabetic rat wounds with 3% CMT-2 increased the wound hydroxyproline and decreased activities of gelatinase and collagenase. There was a delay in wound filling by granulation tissue in diabetic rats. In CMT-2-treated diabetic rats, the volume of granulation tissue was greater than that in untreated diabetic rats. CMT-2 appears to normalize wound healing in diabetic rats and may be a valuable adjunct in the treatment of chronic wounds.

  15. Detection of mosaicism in a CMT1a patient using FISH

    SciTech Connect

    Sourour, E.; Thompson, P.; MacMillan, J.; Upadhyaya, M.

    1994-09-01

    Charcot-Marie-Tooth disease type 1 (CMT1) is the most common hereditary motor and sensory neuropathy, and is characterized by peroneal muscular atrophy, pes cavus, loss of deep tendon reflexes and reduced motor nerve conduction velocities. CMT1a segregates as an autosomal dominant condition and shows complete linkage and association with a large submicroscopic duplication on chromosome 17p11.2-p12. We have detected a mosaicism in the father of an affected CMT1a patient. The affected individuals in this family were found to be homozygous with DNA markers YAW409, p132GBRI which are duplicated in CMT1a patients. FISH analysis with YAC clone (Y49H7), carried on 45 interphases from the affected father, revealed that the duplication was only present in 24 of these interphases. However, this duplication was found in all 50 interphases screened from the father`s affected son and, as expected, none of the 50 interphases derived from a non-CMT case had any evidence of this duplication. The affected father had had difficulty with his balance from age 40, with numerous falls. His median and motor nerve conduction velocities were normal. There was no obvious history fo the disorder in the previous generations. The affected boy had foot drop prior to his 10th birthday. He had loss of sensation in his feet and sensorineural deafness from childhood and had a median motor conduction velocity of 33 meters/second. The findings based on FISH analysis suggest that the mosaicism may have occurred early in embryogenesis leading to the disease in the father.

  16. High frequency of mutations in codon 98 of the peripheral myelin protein Po gene in 20 French CMT1 patients

    SciTech Connect

    Rougher, H.; LeGuern, E. Gouider, R.

    1996-03-01

    Charcot-Marie-Tooth disease, characterized by distal muscle weakness and amyotrophy, decreased or absent tendon reflexes, and high arched feet, is the most common inherited peripheral neuropathy, with a prevalence of 1 in 2,500. Two types of CMT have been distinguished on the basis of nerve conduction velocities. CMT type 1 is the most frequent, with markedly slowed velocities ({<=}40 m/s) associated with hypertrophic onion bulb changes on nerve biopsy. Autosomal dominant CMT1 is genetically heterogeneous: CMT1A is caused by a 1.5-Mb duplication in 17p11.2 and, more rarely, by a point mutation in tha PMP22 (peripheral myelin protein, 22 kD) gene located in the duplicated region; CMT1B results from mutations in the Po (peripheral myelin protein zero) gene in 1q22-23. Forty-five percent (7/16) of the published mutations associated with CMT1 occur in exon 3 of Po. In order to determine the cause of CMT1 in 20 unrelated patients without 17p11.2 duplications, mutations were sought in exon 3 of Po with three techniques: nonradioactive SSCP, automated sequencing, and PCR enzymatic restriction. 18 refs., 2 figs.

  17. Biochemical characterization of protein quality control mechanisms during disease progression in the C22 mouse model of CMT1A

    PubMed Central

    Chittoor, Vinita G.; Sooyeon, Lee; Rangaraju, Sunitha; Nicks, Jessica R.; Schmidt, Jordan T.; Madorsky, Irina; Narvaez, Diana C.; Notterpek, Lucia

    2013-01-01

    Charcot–Marie–Tooth disease type 1A (CMT1A) is a hereditary demyelinating neuropathy linked with duplication of the peripheral myelin protein 22 (PMP22) gene. Transgenic C22 mice, a model of CMT1A, display many features of the human disease, including slowed nerve conduction velocity and demyelination of peripheral nerves. How overproduction of PMP22 leads to compromised myelin and axonal pathology is not fully understood, but likely involves subcellular alterations in protein homoeostatic mechanisms within affected Schwann cells. The subcellular response to abnormally localized PMP22 includes the recruitment of the ubiquitin–proteasome system (UPS), autophagosomes and heat-shock proteins (HSPs). Here we assessed biochemical markers of these protein homoeostatic pathways in nerves from PMP22-overexpressing neuropathic mice between the ages of 2 and 12 months to ascertain their potential contribution to disease progression. In nerves of 3-week-old mice, using endoglycosidases and Western blotting, we found altered processing of the exogenous human PMP22, an abnormality that becomes more prevalent with age. Along with the ongoing accrual of misfolded PMP22, the activity of the proteasome becomes compromised and proteins required for autophagy induction and lysosome biogenesis are up-regulated. Moreover, cytosolic chaperones are consistently elevated in nerves from neuropathic mice, with the most prominent change in HSP70. The gradual alterations in protein homoeostatic response are accompanied by Schwann cell de-differentiation and macrophage infiltration. Together, these results show that while subcellular protein quality control mechanisms respond appropriately to the presence of the overproduced PMP22, with aging they are unable to prevent the accrual of misfolded proteins. PMID:24175617

  18. Expressing hNF-LE397K results in abnormal gaiting in a transgenic model of CMT2E

    PubMed Central

    Dale, Jeffrey M.; Villalon, Eric; Shannon, Stephen G.; Barry, Devin M.; Markey, Rachel M.; Garcia, Virginia B.; Garcia, Michael L.

    2012-01-01

    Charcot-Marie-Tooth disease (CMT) is the most commonly inherited peripheral neuropathy. CMT disease signs include distal limb neuropathy, abnormal gaiting, exacerbation of neuropathy, sensory defects, and deafness. We generated a novel line of CMT2E mice expressing a hNF-LE397K transgene, which displayed muscle atrophy of the lower limbs without denervation, proximal reduction in large caliber axons, and decreased nerve conduction velocity. In this study, we demonstrated that hNF-LE397K mice developed abnormal gait of the hind limbs. The identification of severe gaiting defects in combination with previously observed muscle atrophy, reduced axon caliber, and decreased nerve conduction velocity suggests that hNF-LE397K mice recapitulate many of clinical signs associated with CMT2E. Therefore, hNF-LE397K mice provide a context for potential therapeutic intervention. PMID:22288874

  19. Mutation analysis of the nerve specific promoter of the peripheral myelin protein 22 gene in CMT1 disease and HNPP.

    PubMed

    Nelis, E; De Jonghe, P; De Vriendt, E; Patel, P I; Martin, J J; Van Broeckhoven, C

    1998-07-01

    We analysed the nerve specific promoter of the peripheral myelin protein 22 gene (PMP22) in a set of 15 unrelated patients with Charcot-Marie-Tooth type 1 disease (CMT1) and 16 unrelated patients with hereditary neuropathy with liability to pressure palsies (HNPP). In these patients no duplication/deletion nor a mutation in the coding region of the CMT1/ HNPP genes was detected. In one autosomal dominant CMT1 patient, we identified a base change in the non-coding exon 1A of PMP22 which, however, did not cosegregate with the disease in the family. This study indicates that mutations in the nerve specific PMP22 promoter and 5' untranslated exon will not be a common genetic cause of CMT1A and HNPP.

  20. CMT: A Constrained Multi-Level Thresholding Approach for ChIP-Seq Data Analysis

    PubMed Central

    Rezaeian, Iman; Rueda, Luis

    2014-01-01

    Genome-wide profiling of DNA-binding proteins using ChIP-Seq has emerged as an alternative to ChIP-chip methods. ChIP-Seq technology offers many advantages over ChIP-chip arrays, including but not limited to less noise, higher resolution, and more coverage. Several algorithms have been developed to take advantage of these abilities and find enriched regions by analyzing ChIP-Seq data. However, the complexity of analyzing various patterns of ChIP-Seq signals still needs the development of new algorithms. Most current algorithms use various heuristics to detect regions accurately. However, despite how many formulations are available, it is still difficult to accurately determine individual peaks corresponding to each binding event. We developed Constrained Multi-level Thresholding (CMT), an algorithm used to detect enriched regions on ChIP-Seq data. CMT employs a constraint-based module that can target regions within a specific range. We show that CMT has higher accuracy in detecting enriched regions (peaks) by objectively assessing its performance relative to other previously proposed peak finders. This is shown by testing three algorithms on the well-known FoxA1 Data set, four transcription factors (with a total of six antibodies) for Drosophila melanogaster and the H3K4ac antibody dataset. PMID:24736605

  1. Characterization of methane hydrate host sediments using synchrotron-computed microtomography (CMT)

    USGS Publications Warehouse

    Jones, K.W.; Feng, H.; Tomov, S.; Winters, W.J.; Prodanovic, M.; Mahajan, D.

    2007-01-01

    The hydrate-sediment interaction is an important aspect of gas hydrate studies that needs further examination. We describe here the applicability of the computed microtomography (CMT) technique that utilizes an intense X-ray synchrotron source to characterize sediment samples, two at various depths from the Blake Ridge area (a well-known hydrate-prone region) and one from Georges Bank, that once contained methane trapped as hydrates. Detailed results of the tomographic analysis performed on the deepest sample (667??m) from Blake Ridge are presented as 2-D and 3-D images which show several mineral constituents, the internal grain/pore microstructure, and, following segmentation into pore and grain space, a visualization of the connecting pathways through the pore-space of the sediment. Various parameters obtained from the analysis of the CMT data are presented for all three sediment samples. The micro-scale porosity values showed decreasing trend with increasing depth for all three samples that is consistent with the previously reported bulk porosity data. The 3-D morphology, pore-space pathways, porosity, and permeability values are also reported for all three samples. The application of CMT is now being expanded to the laboratory-formed samples of hydrate in sediments as well as field samples of methane hydrate bearing sediments.

  2. Polytherapy with a combination of three repurposed drugs (PXT3003) down-regulates Pmp22 over-expression and improves myelination, axonal and functional parameters in models of CMT1A neuropathy.

    PubMed

    Chumakov, Ilya; Milet, Aude; Cholet, Nathalie; Primas, Gwenaël; Boucard, Aurélie; Pereira, Yannick; Graudens, Esther; Mandel, Jonas; Laffaire, Julien; Foucquier, Julie; Glibert, Fabrice; Bertrand, Viviane; Nave, Klaus-Armin; Sereda, Michael W; Vial, Emmanuel; Guedj, Mickaël; Hajj, Rodolphe; Nabirotchkin, Serguei; Cohen, Daniel

    2014-12-10

    Charcot-Marie-Tooth disease type 1A (CMT1A) is the most common inherited sensory and motor peripheral neuropathy. It is caused by PMP22 overexpression which leads to defects of peripheral myelination, loss of long axons, and progressive impairment then disability. There is no treatment available despite observations that monotherapeutic interventions slow progression in rodent models. We thus hypothesized that a polytherapeutic approach using several drugs, previously approved for other diseases, could be beneficial by simultaneously targeting PMP22 and pathways important for myelination and axonal integrity. A combination of drugs for CMT1A polytherapy was chosen from a group of authorised drugs for unrelated diseases using a systems biology approach, followed by pharmacological safety considerations. Testing and proof of synergism of these drugs were performed in a co-culture model of DRG neurons and Schwann cells derived from a Pmp22 transgenic rat model of CMT1A. Their ability to lower Pmp22 mRNA in Schwann cells relative to house-keeping genes or to a second myelin transcript (Mpz) was assessed in a clonal cell line expressing these genes. Finally in vivo efficacy of the combination was tested in two models: CMT1A transgenic rats, and mice that recover from a nerve crush injury, a model to assess neuroprotection and regeneration. Combination of (RS)-baclofen, naltrexone hydrochloride and D-sorbitol, termed PXT3003, improved myelination in the Pmp22 transgenic co-culture cellular model, and moderately down-regulated Pmp22 mRNA expression in Schwannoma cells. In both in vitro systems, the combination of drugs was revealed to possess synergistic effects, which provided the rationale for in vivo clinical testing of rodent models. In Pmp22 transgenic CMT1A rats, PXT3003 down-regulated the Pmp22 to Mpz mRNA ratio, improved myelination of small fibres, increased nerve conduction and ameliorated the clinical phenotype. PXT3003 also improved axonal regeneration and

  3. Diagnosis of CMT1A duplications and HNPP deletions by interphase FISH: Implications for testing in the cytogenetics laboratory

    SciTech Connect

    Shaffer, L.G.; Kennedy, G.M.; Spikes, A.S.

    1997-03-31

    Charcot-Marie-Tooth (CMT) disease type 1A is an inherited peripheral neuropathy characterized by slowly progressive distal muscle wasting and weakness, decreased nerve conduction velocities, and genetic linkage to 17p12. Most (>98%) CMT1A cases are caused by a DNA duplication of a 1.5-Mb region in 17p12 containing the PMP22 gene. The reciprocal product of the CMT1A duplication is a 1.5-Mb deletion which causes hereditary neuropathy with liability to pressure palsies (HNPP). The most informative current diagnostic testing requires pulsed-field gel electrophoresis to detect DNA rearrangement-specific junction fragments. We investigated the use of interphase FISH for the detection of duplications and deletions for these disorders in the clinical molecular cytogenetics laboratory. Established cell lines or blood specimens from 23 individuals with known molecular diagnoses and 10 controls were obtained and scored using a two-color FISH assay. At least 70%, of CMT1A cells displayed three signals consistent with duplications. Using this minimum expected percentile to make a CMT1A duplication diagnosis, all patients with CMT1A showed a range of 71-92% of cells displaying at least three signals. Of the HNPP cases, 88% of cells displayed only one hybridization signal, consistent with deletions. The PMP22 locus from normal control individuals displayed a duplication pattern in {approximately}9% of cells, interpreted as replication of this locus. The percentage of cells showing replication was significantly lower than in those cells displaying true duplications. We conclude that FISH can be reliably used to diagnose CMT1A and HNPP in the clinical cytogenetics laboratory and to readily distinguish the DNA rearrangements associated with these disorders from individuals without duplication or deletion of the PMP22 locus. 43 refs., 4 figs., 2 tabs.

  4. Diagnosis of CMT1A duplications and HNPP deletions by interphase FISH: implications for testing in the cytogenetics laboratory.

    PubMed

    Shaffer, L G; Kennedy, G M; Spikes, A S; Lupski, J R

    1997-03-31

    Charcot-Marie-Tooth (CMT) disease type 1A is an inherited peripheral neuropathy characterized by slowly progressive distal muscle wasting and weakness, decreased nerve conduction velocities, and genetic linkage to 17p12. Most (> 98%) CMT1A cases are caused by a DNA duplication of a 1.5-Mb region in 17p12 containing the PMP22 gene. The reciprocal product of the CMT1A duplication is a 1.5-Mb deletion which causes hereditary neuropathy with liability to pressure palsies (HNPP). The most informative current diagnostic testing requires pulsed-field gel electrophoresis to detect DNA rearrangement-specific junction fragments. We investigated the use of interphase FISH for the detection of duplications and deletions for these disorders in the clinical molecular cytogenetics laboratory. Established cell lines or blood specimens from 23 individuals with known molecular diagnoses and 10 controls were obtained and scored using a two-color FISH assay. At least 70% of CMT1A cells displayed three signals consistent with duplications. Using this minimum expected percentile to make a CMT1A duplication diagnosis, all patients with CMT1A showed a range of 71-92% of cells displaying at least three signals. Of the HNPP cases, 88% of cells displayed only one hybridization signal, consistent with deletions. The PMP22 locus from normal control individuals displayed a duplication pattern in approximately 9% of cells, interpreted as replication of this locus. The percentage of cells showing replication was significantly lower than in those cells displaying true duplications. We conclude that FISH can be reliably used to diagnose CMT1A and HNPP in the clinical cytogenetics laboratory and to readily distinguish the DNA rearrangements associated with these disorders from individuals without duplication or deletion of the PMP22 locus.

  5. High Resolution X-ray CMT Imaging of Supercritical CO2 in Porous Media: Experimental Challenges, Solutions, and Results

    NASA Astrophysics Data System (ADS)

    Herring, A. L.; Andersson, L.; Newell, D. L.; Carey, J. W.; Wildenschild, D.

    2013-12-01

    Geologic carbon dioxide (CO2) sequestration has been proposed as a climate change mitigation strategy to limit emissions of CO2 to the atmosphere from large fossil-fuel burning CO2 point sources; however, there are concerns associated with the long-term stability of a mobile subsurface CO2 plume. Capillary trapping of supercritical CO2 (scCO2), wherein the CO2 is held within the pore structure of the geologic matrix by capillary forces, is a more secure form of subsurface storage than structural trapping, which relies on an impermeable caprock to contain the buoyant CO2 plume. To understand the multiphase physics of CO2 transport, and to subsequently produce quantitative estimates of potential CO2 capillary trapping, it is necessary to study field, core, and pore-scale processes. X-ray computed microtomography (x-ray CMT) allows for three-dimensional (3D) in-situ visualization of fluid phases within and the physical structure of a porous medium at the pore-scale. We have designed and built a mobile experimental set-up capable of running at pressures up to 2000 PSI and temperatures up to 50°C, made with materials that are compatible with corrosive fluids. Our experimental procedure includes pressurizing, mixing, and separating fluids; and subsequently running immiscible drainage and imbibition flow experiments with brine and supercritical CO2. With this set-up and procedure, we successfully conducted a brine-scCO2 drainage experiment in Bentheimer sandstone at 1200 PSI and 36°C, and confirmed and quantified CO2 flow in the sandstone core via synchrotron-based x-ray CMT with a resolution of 4.65 μm at the Advanced Photon Source at Argonne National Laboratory. We have proven that we can observe, on a pore-scale basis, the movement of supercritical CO2 within a porous media. The properties of supercritical CO2 (e.g. viscosity, density, interfacial tension and solubility in brine) vary significantly with changes in pressure and temperature; consequently, precise

  6. Loss of Coupling Distinguishes GJB1 Mutations Associated with CNS Manifestations of CMT1X from Those Without CNS Manifestations.

    PubMed

    Abrams, Charles K; Goman, Mikhail; Wong, Sarah; Scherer, Steven S; Kleopa, Kleopas A; Peinado, Alejandro; Freidin, Mona M

    2017-01-10

    CMT1X, an X-linked inherited neuropathy, is caused by mutations in GJB1, which codes for Cx32, a gap junction protein expressed by Schwann cells and oligodendrocytes. Many GJB1 mutations cause central nervous system (CNS) abnormality in males, including stable subclinical signs and, less often, short-duration episodes characterized by motor difficulties and altered consciousness. However, some mutations have no apparent CNS effects. What distinguishes mutations with and without CNS manifestations has been unclear. Here we studied a total of 14 Cx32 mutations, 10 of which are associated with florid episodic CNS clinical syndromes in addition to peripheral neuropathy. The other 4 mutations exhibit neuropathy without clinical or subclinical CNS abnormalities. These "PNS-only" mutations (Y151C, V181M, R183C and L239I) form gap junction plaques and produce levels of junctional coupling similar to those for wild-type Cx32. In contrast, mutants with CNS manifestations (F51L, E102del, V139M, R142Q, R142W, R164W T55I, R164Q and C168Y) either form no morphological gap junction plaques or, if they do, produce little or no detectable junctional coupling. Thus, PNS and CNS abnormalities may involve different aspects of connexin function.

  7. Loss of Coupling Distinguishes GJB1 Mutations Associated with CNS Manifestations of CMT1X from Those Without CNS Manifestations

    PubMed Central

    Abrams, Charles K.; Goman, Mikhail; Wong, Sarah; Scherer, Steven S.; Kleopa, Kleopas A.; Peinado, Alejandro; Freidin, Mona M.

    2017-01-01

    CMT1X, an X-linked inherited neuropathy, is caused by mutations in GJB1, which codes for Cx32, a gap junction protein expressed by Schwann cells and oligodendrocytes. Many GJB1 mutations cause central nervous system (CNS) abnormality in males, including stable subclinical signs and, less often, short-duration episodes characterized by motor difficulties and altered consciousness. However, some mutations have no apparent CNS effects. What distinguishes mutations with and without CNS manifestations has been unclear. Here we studied a total of 14 Cx32 mutations, 10 of which are associated with florid episodic CNS clinical syndromes in addition to peripheral neuropathy. The other 4 mutations exhibit neuropathy without clinical or subclinical CNS abnormalities. These “PNS-only” mutations (Y151C, V181M, R183C and L239I) form gap junction plaques and produce levels of junctional coupling similar to those for wild-type Cx32. In contrast, mutants with CNS manifestations (F51L, E102del, V139M, R142Q, R142W, R164W T55I, R164Q and C168Y) either form no morphological gap junction plaques or, if they do, produce little or no detectable junctional coupling. Thus, PNS and CNS abnormalities may involve different aspects of connexin function. PMID:28071741

  8. Localization of a gene (CMT2A) for autosomal dominant Charcot-Marie-Tooth disease type 2 to chromosome 1p and evidence of genetic heterogeneity

    SciTech Connect

    Othmane, K.B.; Loprest, L.J.; Wilkinson, K.M. ); Middleton, L.T. )

    1993-08-01

    Charcot-Marie-Tooth (CMT) disease type 2 (CMT2) is an inherited peripheral neuropathy characterized by variable age of onset and normal or slightly diminished nerve conduction velocity. CMT2 is pathologically and genetically distinct from CMT type 1 (CMT1). While CMT1 has been shown to be genetically heterogeneous, no chromosomal localization has been established for CMT2. The authors have performed pedigree linkage analysis in six large autosomal dominant CMT2 families and have demonstrated linkage and heterogeneity to a series of microsatellites (D1S160, D1S170, D1S244, D1S228 and D1S199) in the distal region of the short arm of chromosome 1. Significant evidence for heterogeneity was found using admixture analyses and the two-point lod scores. Admixture analyses using the multipoint results for the markers D1S244, D1S228, and D1S199 supported the two-point findings. Three families, DUK662, DUK1241, and 1523 gave posterior probabilities of 1.0, 0.98, and 0.88 of being of the linked type. Multipoint analysis examining the [open quotes]linked[close quotes] families showed that the most favored location for the CMT2A gene is within the interval flanked by D1S244 and D1S228 (odds approximately 70:1 of lying within versus outside that interval). These findings suggest that the CMT2 phenotype is secondary to at least two different genes and demonstrate further heterogeneity in the CMT phenotype.

  9. CMT2D neuropathy is linked to the neomorphic binding activity of glycyl-tRNA synthetase

    PubMed Central

    He, Weiwei; Bai, Ge; Zhou, Huihao; Wei, Na; White, Nicholas M.; Lauer, Janelle; Liu, Huaqing; Shi, Yi; Dumitru, Calin Dan; Lettieri, Karen; Shubayev, Veronica; Jordanova, Albena; Guergueltcheva, Velina; Griffin, Patrick R.; Burgess, Robert W.; Pfaff, Samuel L.; Yang, Xiang-Lei

    2015-01-01

    Summary Selective neuronal loss is a hallmark of neurodegenerative diseases, which counter-intuitively are often caused by mutations in widely-expressed genes1. Charcot-Marie-Tooth (CMT) diseases are the most common hereditary peripheral neuropathies, for which there are no effective therapies2,3. A subtype of the diseases—CMT2D—is caused by dominant mutations in GARS, encoding the ubiquitously expressed enzyme glycyl-tRNA synthetase (GlyRS). Despite the broad requirement of GlyRS for protein biosynthesis in all cells, mutations in this gene cause a selective degeneration of peripheral axons leading to deficits in distal motor function4. How mutations in GlyRS (GlyRSCMT2D) are linked to motor neuron vulnerability has remained elusive. Here we report that GlyRSCMT2D acquires a neomorphic binding activity that directly antagonizes an essential signaling pathway for motor neuron survival. We find that CMT2D mutations alter the conformation of GlyRS, enabling GlyRSCMT2D to bind the Neuropilin 1 (Nrp1) receptor. This aberrant interaction competitively interferes with the binding of the cognate ligand vascular endothelial growth factor (VEGF) to Nrp1. Genetic reduction of Nrp1 in mice worsens CMT2D symptoms, whereas enhanced expression of VEGF improves motor function. These findings link the selective pathology of CMT2D to the neomorphic binding activity of GlyRSCMT2D that antagonizes the VEGF/Nrp1 interaction, and indicate the VEGF/Nrp1 signaling axis is an actionable target for treating CMT2D. PMID:26503042

  10. A recurrent loss-of-function alanyl-tRNA synthetase (AARS) mutation in patients with Charcot-Marie-Tooth disease type 2N (CMT2N).

    PubMed

    McLaughlin, Heather M; Sakaguchi, Reiko; Giblin, William; Wilson, Thomas E; Biesecker, Leslie; Lupski, James R; Talbot, Kevin; Vance, Jeffery M; Züchner, Stephan; Lee, Yi-Chung; Kennerson, Marina; Hou, Ya-Ming; Nicholson, Garth; Antonellis, Anthony

    2012-01-01

    Charcot-Marie-Tooth (CMT) disease comprises a heterogeneous group of peripheral neuropathies characterized by muscle weakness and wasting, and impaired sensation in the extremities. Four genes encoding an aminoacyl-tRNA synthetase (ARS) have been implicated in CMT disease. ARSs are ubiquitously expressed, essential enzymes that ligate amino acids to cognate tRNA molecules. Recently, a p.Arg329His variant in the alanyl-tRNA synthetase (AARS) gene was found to segregate with dominant axonal CMT type 2N (CMT2N) in two French families; however, the functional consequence of this mutation has not been determined. To investigate the role of AARS in CMT, we performed a mutation screen of the AARS gene in patients with peripheral neuropathy. Our results showed that p.Arg329His AARS also segregated with CMT disease in a large Australian family. Aminoacylation and yeast viability assays showed that p.Arg329His AARS severely reduces enzyme activity. Genotyping analysis indicated that this mutation arose on three distinct haplotypes, and the results of bisulfite sequencing suggested that methylation-mediated deamination of a CpG dinucleotide gives rise to the recurrent p.Arg329His AARS mutation. Together, our data suggest that impaired tRNA charging plays a role in the molecular pathology of CMT2N, and that patients with CMT should be directly tested for the p.Arg329His AARS mutation.

  11. CMT Source Inversions for Massive Data Assimilation in Global Adjoint Tomography

    NASA Astrophysics Data System (ADS)

    Lei, W.; Ruan, Y.; Bozdag, E.; Lefebvre, M. P.; Smith, J. A.; Modrak, R. T.; Komatitsch, D.; Song, X.; Liu, Q.; Tromp, J.; Peter, D. B.

    2015-12-01

    Full Waveform Inversion (FWI) is a vital tool for probing the Earth's interior and enhancing our knowledge of the underlying dynamical processes [e.g., Liu et al., 2012]. Using the adjoint tomography method, we have successfully obtained a first-generation global FWI model named M15 [Bozdag et al., 2015]. To achieve higher resolution of the emerging new structural features and to accommodate azimuthal anisotropy and anelasticity in the next-generation model, we expanded our database from 256 to 4,224 earthquakes. Previous studies have shown that ray-theory-based Centroid Moment Tensor (CMT) inversion algorithms can produce systematic biases in earthquake source parameters due to tradeoffs with 3D crustal and mantle heterogeneity [e.g., Hjorleifsdottir et al., 2010]. To reduce these well-known tradeoffs, we performed CMT inversions in our current 3D global model before resuming the structural inversion with the expanded database. Initial source parameters are selected from the global CMT database [Ekstrom et al., 2012], with moment magnitudes ranging from 5.5 to 7.0 and occurring between 1994 and 2015. Data from global and regional networks were retrieved from the IRIS DMC. Synthetic seismograms were generated based on the spectral-element-based seismic wave propagation solver (SPECFEM3D GLOBE) in model M15. We used a source inversion algorithm based on a waveform misfit function while allowing time shifts between data and synthetics to accommodate additional unmodeled 3D heterogeneity [Liu et al., 2004]. To accommodate the large number of earthquakes and time series (more than 10,000,000 records), we implemented a source inversion workflow based on the newly developed Adaptive Seismic Data Format (ASDF) [Krischer, Smith, et al., 2015] and ObsPy [Krischer et al., 2015]. In ASDF, each earthquake is associated with a single file, thereby eliminating I/O bottlenecks in the workflow and facilitating fast parallel processing. Our preliminary results indicate that errors

  12. Fine mapping of de novo CMT1A and HNPP rearrangements within CMT1A-REPs evidences two distinct sex-dependent mechanisms and candidate sequences involved in recombination.

    PubMed

    Lopes, J; Ravisé, N; Vandenberghe, A; Palau, F; Ionasescu, V; Mayer, M; Lévy, N; Wood, N; Tachi, N; Bouche, P; Latour, P; Ruberg, M; Brice, A; LeGuern, E

    1998-01-01

    The molecular mechanism resulting in the duplication or deletion of a 1.5 Mb region of 17p11.2-p12, associated, respectively, with Charcot-Marie-Tooth type 1A (CMT1A) and hereditary neuropathy with liability to pressure palsies (HNPP), has been proposed to be an unequal crossing-over during meiosis between the two chromosome 17 homologues generated by misalignment of the proximal and distal CMT1A-REP repeats, two homologous sequences flanking the 1.5 Mb CMT1A/HNPP monomer unit. In a recent study of a large series of de novo cases of CMT1A and HNPP, two distinct sex-dependent mechanisms were identified. Rearrangements of paternal origin, essentially duplications, were indeed generated by unequal meiotic crossing-over between the two chromosome 17 homologues, but duplications and deletions of maternal origin resulted from an intrachromosomal process, either unequal sister chromatid exchange or, in the case of deletion, excision of an intrachromatidal loop. In order to determine how these recombinations occur, 24 de novo crossover breakpoints were localized within the 1.7 kb rearrangement hot spot by comparing the sequences of the parental CMT1A-REPs with the chimeric copy in affected offspring. Nineteen out of 21 paternal crossovers were found in a 741 bp hot spot. All the breakpoints of maternal origin (n = 3), however, were located outside this interval, but in closely flanking sequences, supporting the hypothesis that two distinct sex-dependent mechanisms are involved. Several putative recombination promoting sequences in the hot spot, which are rare or absent in the surrounding 7.8 kb, were identified.

  13. Loss of Fig4 in both Schwann cells and motor neurons contributes to CMT4J neuropathy

    PubMed Central

    Vaccari, Ilaria; Carbone, Antonietta; Previtali, Stefano Carlo; Mironova, Yevgeniya A.; Alberizzi, Valeria; Noseda, Roberta; Rivellini, Cristina; Bianchi, Francesca; Del Carro, Ubaldo; D'Antonio, Maurizio; Lenk, Guy M.; Wrabetz, Lawrence; Giger, Roman J.; Meisler, Miriam H.; Bolino, Alessandra

    2015-01-01

    Mutations of FIG4 are responsible for Yunis-Varón syndrome, familial epilepsy with polymicrogyria, and Charcot-Marie-Tooth type 4J neuropathy (CMT4J). Although loss of the FIG4 phospholipid phosphatase consistently causes decreased PtdIns(3,5)P2 levels, cell-specific sensitivity to partial loss of FIG4 function may differentiate FIG4-associated disorders. CMT4J is an autosomal recessive neuropathy characterized by severe demyelination and axonal loss in human, with both motor and sensory involvement. However, it is unclear whether FIG4 has cell autonomous roles in both motor neurons and Schwann cells, and how loss of FIG4/PtdIns(3,5)P2-mediated functions contribute to the pathogenesis of CMT4J. Here, we report that mice with conditional inactivation of Fig4 in motor neurons display neuronal and axonal degeneration. In contrast, conditional inactivation of Fig4 in Schwann cells causes demyelination and defects in autophagy-mediated degradation. Moreover, Fig4-regulated endolysosomal trafficking in Schwann cells is essential for myelin biogenesis during development and for proper regeneration/remyelination after injury. Our data suggest that impaired endolysosomal trafficking in both motor neurons and Schwann cells contributes to CMT4J neuropathy. PMID:25187576

  14. A novel NDRG1 mutation in a non-Romani patient with CMT4D/HMSN-Lom.

    PubMed

    Piscosquito, Giuseppe; Magri, Stefania; Saveri, Paola; Milani, Micaela; Ciano, Claudia; Farina, Laura; Taroni, Franco; Pareyson, Davide

    2017-03-01

    Charcot-Marie-Tooth disease type 4D (CMT4D), also known as hereditary motor and sensory neuropathy Lom type (HMSNL), is an autosomal recessive, early onset, severe demyelinating neuropathy with hearing loss, caused by N-Myc downstream-regulated gene 1 (NDRG1) mutations. CMT4D is rare with only three known mutations, one of which (p.Arg148Ter) is found in patients of Romani ancestry and accounts for the vast majority of cases. We report a 38-year-old Italian female with motor development delay, progressive neuropathy, and sensorineural deafness. Magnetic resonance imaging showed slight atrophy of cerebellum, medulla oblongata, and upper cervical spinal cord. She had a novel homozygous NDRG1 frameshift mutation (c.739delC; p.His247ThrfsTer74). The identification of this NDRG1 mutation confirms that CMT4D is not a private Romani disease and should be considered in the differential diagnosis of recessive demyelinating CMT.

  15. Mutations in noncoding regions of GJB1 are a major cause of X-linked CMT

    PubMed Central

    Tomaselli, Pedro J.; Rossor, Alexander M.; Horga, Alejandro; Jaunmuktane, Zane; Carr, Aisling; Saveri, Paola; Piscosquito, Giuseppe; Pareyson, Davide; Laura, Matilde; Blake, Julian C.; Poh, Roy; Polke, James; Houlden, Henry

    2017-01-01

    Objective: To determine the prevalence and clinical and genetic characteristics of patients with X-linked Charcot-Marie-Tooth disease (CMT) due to mutations in noncoding regions of the gap junction β-1 gene (GJB1). Methods: Mutations were identified by bidirectional Sanger sequence analysis of the 595 bases of the upstream promoter region, and 25 bases of the 3′ untranslated region (UTR) sequence in patients in whom mutations in the coding region had been excluded. Clinical and neurophysiologic data were retrospectively collected. Results: Five mutations were detected in 25 individuals from 10 kindreds representing 11.4% of all cases of CMTX1 diagnosed in our neurogenetics laboratory between 1996 and 2016. Four pathogenic mutations, c.-17G>A, c.-17+1G>T, c.-103C>T, and c.-146-90_146-89insT were detected in the 5′UTR. A novel mutation, c.*15C>T, was detected in the 3′ UTR of GJB1 in 2 unrelated families with CMTX1 and is the first pathogenic mutation in the 3′UTR of any myelin-associated CMT gene. Mutations segregated with the phenotype, were at sites predicted to be pathogenic, and were not present in the normal population. Conclusions: Mutations in noncoding DNA are a major cause of CMTX1 and highlight the importance of mutations in noncoding DNA in human disease. Next-generation sequencing platforms for use in inherited neuropathy should therefore include coverage of these regions. PMID:28283593

  16. Fate of Schwann cells in CMT1A and HNPP: evidence for apoptosis.

    PubMed

    Erdem, S; Mendell, J R; Sahenk, Z

    1998-06-01

    The fate of Schwann cells in Charcot-Marie-Tooth (CMT) neuropathies was addressed in this study of nerve biopsies from patients with proven PMP22 duplications and deletions. In frozen sections, apoptotic nuclei were detected using the TUNEL method. In adjacent sections, anti-neurofilament 68kD antibody was used as an axonal marker, while the antibodies to NKH-1 and low-affinity nerve growth factor receptor P75NTR were used as Schwann cell markers. In addition, plastic sections were used to determine the densities of myelinated fibers and Schwann cell nuclei. In all biopsies from CMT1A, TUNEL-positive nuclei appeared in clusters. In adjacent sections, areas of TUNEL-positive nuclei matched with areas devoid of neurofilaments and NKH-1-positive Schwann cell silhouettes, suggesting that the apoptotic nuclei belonged to nonmyelinating Schwann cells. In addition, quantitative studies on plastic-embedded sections showed a significantly reduced number of total Schwann cells compared with controls, strongly favoring a loss of Schwann cell by apoptosis. In HNPP, the number of total Schwann cells was increased and a significant Schwann cell apoptosis was observed in only 2 patients. Examination of plastic sections and teased nerve preparations from these cases suggested that the Schwann cell apoptosis might be related to the regenerative state of the nerve resulting from the process of sprout pruning. No strict correlation between p75NTR expression and apoptosis was found. These studies indicate that factors regulating Schwann cell number in early postnatal development continue to be important for Schwann cell survival throughout life.

  17. The LITAF/SIMPLE I92V sequence variant results in an earlier age of onset of CMT1A/HNPP diseases.

    PubMed

    Sinkiewicz-Darol, Elena; Lacerda, Andressa Ferreira; Kostera-Pruszczyk, Anna; Potulska-Chromik, Anna; Sokołowska, Beata; Kabzińska, Dagmara; Brunetti, Craig R; Hausmanowa-Petrusewicz, Irena; Kochański, Andrzej

    2015-01-01

    Charcot-Marie-Tooth disease type 1A (CMT1A) and hereditary neuropathy with liability to pressure palsies (HNPP) represent the most common heritable neuromuscular disorders. Molecular diagnostics of CMT1A/HNPP diseases confirm clinical diagnosis, but their value is limited to the clinical course and prognosis. However, no biomarkers of CMT1A/HNPP have been identified. We decided to explore if the LITAF/SIMPLE gene shared a functional link to the PMP22 gene, whose duplication or deletion results in CMT1A and HNPP, respectively. By studying a large cohort of CMT1A/HNPP-affected patients, we found that the LITAF I92V sequence variant predisposes patients to an earlier age of onset of both the CMT1A and HNPP diseases. Using cell transfection experiments, we showed that the LITAF I92V sequence variant partially mislocalizes to the mitochondria in contrast to wild-type LITAF which localizes to the late endosome/lysosomes and is associated with a tendency for PMP22 to accumulate in the cells. Overall, this study shows that the I92V LITAF sequence variant would be a good candidate for a biomarker in the case of the CMT1A/HNPP disorders.

  18. Synergistic growth inhibitory effect of deracoxib with doxorubicin against a canine mammary tumor cell line, CMT-U27

    PubMed Central

    BAKIREL, Tülay; ALKAN, Fulya Üstün; ÜSTÜNER, Oya; ÇINAR, Suzan; YILDIRIM, Funda; ERTEN, Gaye; BAKIREL, Utku

    2016-01-01

    Cyclooxygenase (COX) inhibitors have been shown to exert anti-angiogenic and anti-tumor activities on many types of malignant tumors. These anticancer properties make it worthwhile to examine the possible benefit of combining COX inhibitors with other anti-cancer agents. In the present study, we evaluated the potential of deracoxib (DER) in potentiating antitumor activity of doxorubicin (DOX) in canine mammary carcinoma cells (CMT-U27). DER (50–250 µM) enhanced the antiproliferative activity of DOX by reducing the IC50 (approximately 3- to 3.5 fold). Interaction analysis of the data showed that combinations of DOX at 0.9 µM with DER (100–250 µM) produced synergism in the CMT-U27 cell line, with a ratio index ranging from 1.98 to 2.33. In additional studies identifying the mechanism of observed synergistic effect, we found that DER strongly potentiated DOX-caused G0/G1 arrest in cell cycle progression. Also, DER (100–250 µM) augmented apoptosis induction with approximately 1.35- and 1.37- fold increases in apoptotic response caused by DOX in the cells. DER enhanced the antiproliferative effect of DOX in conjunction with induction of apoptosis by modulation of Bcl-2 expression and changes in the cell cycle of the CMT-U27 cell line. Although the exact molecular mechanism of the alterations in the cell cycle and apoptosis observed with DER and DOX combinations require further investigations, the results suggest that the synergistic effect of DOX and DER combinations in CMT therapy may be achieved at relatively lower doses of DOX with lesser side effects. Therefore, combining DER with DOX may prove beneficial in the clinical treatment of canine mammary cancer. PMID:26822118

  19. SNM Movement Detection/Radiation Sensors and Advanced Materials Portfolio Review, CdMnTe (CMT) Gamma Ray Detectors

    SciTech Connect

    Bolotnikov,A.

    2009-06-02

    The project goals are: (1) Develop CMT radiation detectors - Demonstrate feasibility (Phase 1 is complete) and Improve material properties and device performance; (2) This project will lead to novel radiation detectors - high detection efficiency, high energy-resolution, ambient-temperature operation, and low production cost; and (3) Such detectors are needed in areas of nonproliferation and national security for detection of SNM. Research highlights are: (1) We achieved our Phase-I goal - Demonstration of CMT detector performance approaching that of CZT detectors; (2) Demonstrated that In-doped CMT is much closer to its anticipated performance as radiation detectors than other alternative materials, TlBr and HgI{sub 2} - Large crystal volumes, 10{sup 10}{Omega}{center_dot}cm, 3 x 10{sup -3}cm{sup 2}/V, and stable response; and (3) Conducted material and device characterization experiments - Detectors: I-V, {mu}{sub e}, ({mu}{tau}){sub e}, internal E fields, energy spectra, and high-resolution x-ray response mapping data and Materials - DLTS, TCT, PL, EPDs, XRD, PCD and IR transmission.

  20. Application of whole-exome sequencing for detecting copy number variants in CMT1A/HNPP.

    PubMed

    Jo, H-Y; Park, M-H; Woo, H-M; Han, M H; Kim, B-Y; Choi, B-O; Chung, K W; Koo, S K

    2016-08-01

    Large insertions and deletions (indels), including copy number variations (CNVs), are commonly seen in many diseases. Standard approaches for indel detection rely on well-established methods such as qPCR or short tandem repeat (STR) markers. Recently, a number of tools for CNV detection based on next-generation sequencing (NGS) data have also been developed; however, use of these methods is limited. Here, we used whole-exome sequencing (WES) in patients previously diagnosed with CMT1A or HNPP using STR markers to evaluate the ability of WES to improve the clinical diagnosis. Patients were evaluated utilizing three CNV detection tools including CONIFER, ExomeCNV and CEQer, and array comparative genomic hybridization (aCGH). We identified a breakpoint region at 17p11.2-p12 in patients with CMT1A and HNPP. CNV detection levels were similar in both 6 Gb (mean read depth = 80×) and 17 Gb (mean read depth = 190×) data. Taken together, these data suggest that 6 Gb WES data are sufficient to reveal the genetic causes of various diseases and can be used to estimate single mutations, indels, and CNVs simultaneously. Furthermore, our data strongly indicate that CNV detection by NGS is a rapid and cost-effective method for clinical diagnosis of genetically heterogeneous disorders such as CMT neuropathy.

  1. Quantification of soil heterogeneity induced by corroding metal objects, using X-ray computed micro tomography (CMT)

    NASA Astrophysics Data System (ADS)

    Afanasyev, Michael; Heimovaara, Timo; van Paassen, Leon

    2014-05-01

    Metal objects in soil, such as pipelines and sheet pile walls are subject to corrosion, that causes extensive economic damage - the annual direct costs of metal corrosion are estimated as 3-4% of the gross domestic product (GDP) of both developed and developing countries. Corrosion of the metal object results in the diffusion of corrosion products away from the original metal surface, where the corrosion products combine with dissolved species and precipitate, altering the properties of the porous medium. The result is a system composed of the uncorroded metal, the Dense Product Layer (DPL) composed of iron corrosion products, the Transformed Medium (TM) which is a mix of the corrosion products and compounds coming from the surrounding soil and the unaltered soil. Naturally occurring DPL's were reported to reduce the corrosion rate of metal objects in soil and studies of metal archaeological artifacts show that it is possible that microbiota plays a role in the process, controlling the rate and location of reprecipitation of corrosion products. However, the dynamics of such complex processes in soil are not yet fully understood and experimental results that can be used to calibrate and verify numerical models of corrosion processes in porous media are scarce. In this study, we explore the potential of X-ray computed microtomography (CMT) in quantifying the changes occurring in soil around corroding metal objects. Metal coupons were incubated in sand and scanned using a Phoenix Nanotom® s nanofocus computed tomography system. Using objects of known density in the samples, the measurements were density-calibrated and the increase in density and accompanying reduction in porosity due to reprecipitation of corrosion products were quantified. Our results demonstrate the potential of X-ray tomography in non-destructive quantification of corrosion processes in porous media. We suggest using smaller samples to increase resolution of the measurements and to use

  2. Selected items from the Charcot-Marie-Tooth (CMT) Neuropathy Score and secondary clinical outcome measures serve as sensitive clinical markers of disease severity in CMT1A patients.

    PubMed

    Mannil, Manoj; Solari, Alessandra; Leha, Andreas; Pelayo-Negro, Ana L; Berciano, José; Schlotter-Weigel, Beate; Walter, Maggie C; Rautenstrauss, Bernd; Schnizer, Tuuli J; Schenone, Angelo; Seeman, Pavel; Kadian, Chandini; Schreiber, Olivia; Angarita, Natalia G; Fabrizi, Gian Maria; Gemignani, Franco; Padua, Luca; Santoro, Lucio; Quattrone, Aldo; Vita, Giuseppe; Calabrese, Daniela; Young, Peter; Laurà, Matilde; Haberlová, Jana; Mazanec, Radim; Paulus, Walter; Beissbarth, Tim; Shy, Michael E; Reilly, Mary M; Pareyson, Davide; Sereda, Michael W

    2014-11-01

    This study evaluates primary and secondary clinical outcome measures in Charcot-Marie-Tooth disease type 1A (CMT1A) with regard to their contribution towards discrimination of disease severity. The nine components of the composite Charcot-Marie-Tooth disease Neuropathy Score and six additional secondary clinical outcome measures were assessed in 479 adult patients with genetically proven CMT1A and 126 healthy controls. Using hierarchical clustering, we identified four significant clusters of patients according to clinical severity. We then tested the impact of each of the CMTNS components and of the secondary clinical parameters with regard to their power to differentiate these four clusters. The CMTNS components ulnar sensory nerve action potential (SNAP), pin sensibility, vibration and strength of arms did not increase the discriminant value of the remaining five CMTNS components (Ulnar compound motor action potential [CMAP], leg motor symptoms, arm motor symptoms, leg strength and sensory symptoms). However, three of the six additional clinical outcome measures - the 10m-timed walking test (T10MW), 9 hole-peg test (9HPT), and foot dorsal flexion dynamometry - further improved discrimination between severely and mildly affected patients. From these findings, we identified three different composite measures as score hypotheses and compared their discriminant power with that of the CMTNS. A composite of eight components CMAP, Motor symptoms legs, Motor symptoms arms, Strength of Legs, Sensory symptoms), displayed the strongest power to discriminate between the clusters. As a conclusion, five items from the CMTNS and three secondary clinical outcome measures improve the clinical assessment of patients with CMT1A significantly and are beneficial for upcoming clinical and therapeutic trials.

  3. Isolation of novel genes from the CMT1A duplication/HNPP deletion critical region in 17p11.2-p12.

    PubMed

    Murakami, T; Sun, Z S; Lee, C C; Lupski, J R

    1997-01-01

    Charcot-Marie-Tooth disease type 1A (CMT1A) is associated with a 1.5-Mb tandem DNA duplication in chromosome 17p11.2-p12, while hereditary neuropathy with liability to pressure palsies (HNPP) is associated with a 1.5-Mb deletion at this locus. The 1.5-Mb CMT1A monomer unit duplicated in CMT1A and deleted in HNPP is flanked by low-copy repeats termed CMT1A-REPs. Both diseases appear to be caused by an altered copy number of the peripheral myelin protein 22 gene (PMP22), which lies within the critical region. To identify additional genes rapidly, we used a cosmid contig of this region and reciprocal probing of arrayed chromosome 17-specific cosmid and cDNA libraries. Three cDNA clones were identified within the CMT1A duplication/HNPP deletion region and one just proximal to the critical region. The cDNA for human heme A:farnesyltransferase (COX10) mapped 10 kb centromeric to the distal CMT1A-REP. The other two cDNA clones from within the critical interval mapped to cosmid 126D1 at the mfd41 (D17S261) DNA marker, and their conceptual translation showed homology to 60S ribosomal protein L9 (RPL9) and chromosomal protein RMSA-1 (RMSA-1). A gene that is homologous to human peroxisome proliferator activated receptor alpha (hPPARA) was identified near the proximal CMT1A-REP.

  4. The PpCMT chromomethylase affects cell growth and interacts with the homolog of LIKE HETEROCHROMATIN PROTEIN 1 in the moss Physcomitrella patens.

    PubMed

    Dangwal, Meenakshi; Kapoor, Sanjay; Kapoor, Meenu

    2014-02-01

    Chromomethylases (CMTs) are plant-specific cytosine DNA methyltransferases that are involved in maintenance of CpNpG methylation. In seed plants, histone methylation and interaction of CMT with LIKE HETEROCHROMATIN PROTEIN 1 (LHP1) is essential for recruitment of CMT to target sites. LHP1 has been characterized as a putative component of the POLYCOMB REPRESSIVE COMPLEX1 (PRC1) in plants, and functions downstream of PRC2 to maintain genes in repressed state for orchestrated development. In the present study, we show that targeted disruption of PpCMT results in an approximately 50% reduction in global cytosine methylation levels. This affects growth of apical cells, predominantly growth of side branch initials emerging from chloronema cells. In some places, these cells develop thick walls with plasmolyzed cellular contents. Transcript accumulation patterns of genes involved in apical cell extension and metabolism of hemicelluloses, such as xyloglucans, in the primary cell walls decreased many fold in ppcmt mutant lines, as determined by real-time PCR. Using yeast two-hybrid method and bimolecular fluorescence complementation assay, we show that PpCMT and PpLHP1 interact through their chromo domains, while PpLHP1 homodimerizes through its chromo shadow domain. The results presented in this study provide insight into the role of the single chromomethylase, PpCMT, in proliferation of protonema filaments, and shed light on the evolutionary conservation of proteins interacting with these methylases in the early land plant, Physcomitrella patens.

  5. Screening of the 17p11.2--p12 region in a large cohort of patients with Charcot-Marie-Tooth (CMT) disease or hereditary neuropathy with liability to pressure palsies (HNPP).

    PubMed

    Kabzinska, D; Pierscinska, J; Kochanski, A

    2009-01-01

    Within the last decade, numerous methods have been applied to detect the most common mutation in patients affected with Charcot-Marie-Tooth (CMT) disease, i.e. submicroscopic duplication in the 17p11.2--p12 region. In 1993, another neuropathy - known as hereditary neuropathy with liability to pressure palsies (HNPP) - has been shown to be caused by a 17p11.2--p12 deletion. Historically, Southern blot analysis was the first approach to identify CMT1A duplication or HNPP deletion. This time- and labor-consuming method requires prior selection of DNA samples. In fact, only CMT patients affected with the demyelinating form of CMT1 have been screened for CMT1A duplication. After the 17p11.2--p12 duplication was identified in the CMT1 families, subsequent studies revealed additional axonal features in the patients harboring the 17p11.2--p12 duplication. Thus it seems reasonable to test all patients affected with CMT for the presence of the 17p11.2--p12 duplication. To evaluate the utility of real-time polymerase chain reaction (Q-PCR) and restriction fragment length polymorphism PCR (RFLP-PCR), we screened a large group of 179 families with the diagnosis of CMT/HNPP for the presence of the 17p11.2--p12 duplication/deletion. Due to a high frequency of CMT1A duplication in familial cases of CMT, we propose (in contrast to the previous studies) to perform Q-PCR analysis in all patients diagnosed with CMT.

  6. Radiosynthesis and validation of 18F-FP-CMT, a phenyltropane with superior properties for imaging the dopamine transporter in living brain

    PubMed Central

    Cumming, Paul; Maschauer, Simone; Riss, Patrick J; Tschammer, Nuska; Fehler, Stefanie K; Heinrich, Markus R; Kuwert, Torsten; Prante, Olaf

    2014-01-01

    To date there is no validated, 18F-labeled dopamine transporter (DAT) radiotracer with a rapid kinetic profile suitable for preclinical small-animal positron emission tomography (PET) studies in rodent models of human basal ganglia disease. Herein we report radiosynthesis and validation of the phenyltropane 18F-FP-CMT. Dynamic PET recordings were obtained for 18F-FP-CMT in six untreated rats, and six rats pretreated with the high-affinity DAT ligand GBR 12909; mean parametric maps of binding potential (BPND) relative to the cerebellum reference region, and maps of total distribution volume (VT) relative to the metabolite-corrected arterial input were produced. 18F-FP-CMT BPND maps showed peak values of ∼4 in the striatum, versus ∼0.4 in the vicinity of the substantia nigra. Successive truncation of the PET recordings indicated that stable BPND estimates could be obtained with recordings lasting only 45 minutes, reflecting rapid kinetics of 18F-FP-CMT. Pretreatment with GBR 12909 reduced the striatal binding by 72% to 76%. High-performance liquid chromatography analysis revealed rapid metabolism of 18F-FP-CMT to a single, non-brain penetrant hydrophilic metabolite. Total distribution of volume calculated relative to the metabolite-corrected arterial input was 4.4 mL/g in the cerebellum. The pharmacological selectivity of 18F-FP-CMT, rapid kinetic profile, and lack of problematic metabolites constitute optimal properties for quantitation of DAT in rat, and may also predict applicability in human PET studies. PMID:24714035

  7. Mild recurrent neuropathy in CMT1B with a novel nonsense mutation in the extracellular domain of the MPZ gene

    PubMed Central

    Lagueny, A; Latour, P; Vital, A; Le Masson, G; Rouanet, M; Ferrer, X; Vital, C; Vandenberghe, A

    2001-01-01

    Clinical, electrophysiological, and neuropathological features are reported associated with a novel heterozygote point mutation in the extracellular domain of the MPZ gene, where a transversion at codon 71 in exon 3 leads to a codon stop: Glu71stop (ie GAA→TAA). A 36 year old woman developed a mild recurrent neuropathy after intensive manual work. The motor nerve conduction velocities were slow without conduction blocks and the nerve biopsy showed signs of demyelination-remyelination, axonal loss, and regular uncompacted myelin lamellae. She inherited the mutation from her father who displayed the same mutation with a normal phenotype. This nonsense mutation may cause a dosage difference of normal P0, and is probably underrepresented in the current mutation data bases. This report further extends the phenotype of MPZ mutations and also emphasises that mild phenotype of CMT1B may be more frequent than has been appreciated.

 PMID:11160475

  8. [A case of Charcot-Marie-Tooth disease (CMT) type 1 complicated by diabetes mellitus (DM) showing bilateral phrenic nerve palsy].

    PubMed

    Takakura, Yuka; Furuya, Hirokazu; Yamashita, Ken-ichiro; Murai, Hiroyuki; Araki, Takehisa; Kikuchi, Hitoshi; Ohyagi, Yasumasa; Yamada, Takeshi; Kira, Jun-ichi

    2002-04-01

    We here report a 44-year-old woman with Charcot-Marie-Tooth disease (CMT) type 1 who showed severe bilateral phrenic nerve palsy (PNP). She had chronic progressive distal dominant muscle weakness and atrophy since early in her second decade and had been unable to walk by herself due to weakness of the legs since she was 40-years old. At that time, she was diagnosed with diabetes mellitus (DM). She also had difficulty breathing when she was in a supine position. On admission, sural nerve biopsy showed a marked decrease of large and small myelinated fibers and numerous onion bulb formations, which are compatible with CMT type 1. Chest X-ray showed bilateral elevation of the diaphragm, which was more marked on the right side, indicating bilateral PNP. Since it is reported that CMT patients show demyelination of the phrenic nerve subclinically, and DM itself may facilitate the development of PNP, periodic evaluations of respiratory function may thus be useful for preventing respiratory failure in patients with CMT, especially when it is complicated with DM.

  9. CMT-linked loss-of-function mutations in GDAP1 impair store-operated Ca(2+) entry-stimulated respiration.

    PubMed

    González-Sánchez, Paloma; Pla-Martín, David; Martínez-Valero, Paula; Rueda, Carlos B; Calpena, Eduardo; Del Arco, Araceli; Palau, Francesc; Satrústegui, Jorgina

    2017-02-21

    GDAP1 is an outer mitochondrial membrane protein involved in Charcot-Marie-Tooth (CMT) disease. Lack of GDAP1 gives rise to altered mitochondrial networks and endoplasmic reticulum (ER)-mitochondrial interactions resulting in a decreased ER-Ca(2+) levels along with a defect on store-operated calcium entry (SOCE) related to a misallocation of mitochondria to subplasmalemmal sites. The defect on SOCE is mimicked by MCU silencing or mitochondrial depolarization, which prevent mitochondrial calcium uptake. Ca(2+) release from de ER and Ca(2+) inflow through SOCE in neuroblastoma cells result in a Ca(2+)-dependent upregulation of respiration which is blunted in GDAP1 silenced cells. Reduced SOCE in cells with CMT recessive missense mutations in the α-loop of GDAP1, but not dominant mutations, was associated with smaller SOCE-stimulated respiration. These cases of GDAP1 deficiency also resulted in a decreased ER-Ca(2+) levels which may have pathological implications. The results suggest that CMT neurons may be under energetic constraints upon stimulation by Ca(2+) mobilization agonists and point to a potential role of perturbed mitochondria-ER interaction related to energy metabolism in forms of CMT caused by some of the recessive or null mutations of GDAP1.

  10. CMT-linked loss-of-function mutations in GDAP1 impair store-operated Ca2+ entry-stimulated respiration

    PubMed Central

    González-Sánchez, Paloma; Pla-Martín, David; Martínez-Valero, Paula; Rueda, Carlos B.; Calpena, Eduardo; del Arco, Araceli; Palau, Francesc; Satrústegui, Jorgina

    2017-01-01

    GDAP1 is an outer mitochondrial membrane protein involved in Charcot-Marie-Tooth (CMT) disease. Lack of GDAP1 gives rise to altered mitochondrial networks and endoplasmic reticulum (ER)-mitochondrial interactions resulting in a decreased ER-Ca2+ levels along with a defect on store-operated calcium entry (SOCE) related to a misallocation of mitochondria to subplasmalemmal sites. The defect on SOCE is mimicked by MCU silencing or mitochondrial depolarization, which prevent mitochondrial calcium uptake. Ca2+ release from de ER and Ca2+ inflow through SOCE in neuroblastoma cells result in a Ca2+-dependent upregulation of respiration which is blunted in GDAP1 silenced cells. Reduced SOCE in cells with CMT recessive missense mutations in the α-loop of GDAP1, but not dominant mutations, was associated with smaller SOCE-stimulated respiration. These cases of GDAP1 deficiency also resulted in a decreased ER-Ca2+ levels which may have pathological implications. The results suggest that CMT neurons may be under energetic constraints upon stimulation by Ca2+ mobilization agonists and point to a potential role of perturbed mitochondria-ER interaction related to energy metabolism in forms of CMT caused by some of the recessive or null mutations of GDAP1. PMID:28220846

  11. Mechanisms for nonrecurrent genomic rearrangements associated with CMT1A or HNPP: rare CNVs as a cause for missing heritability.

    PubMed

    Zhang, Feng; Seeman, Pavel; Liu, Pengfei; Weterman, Marian A J; Gonzaga-Jauregui, Claudia; Towne, Charles F; Batish, Sat Dev; De Vriendt, Els; De Jonghe, Peter; Rautenstrauss, Bernd; Krause, Klaus-Henning; Khajavi, Mehrdad; Posadka, Jan; Vandenberghe, Antoon; Palau, Francesc; Van Maldergem, Lionel; Baas, Frank; Timmerman, Vincent; Lupski, James R

    2010-06-11

    Genomic rearrangements involving the peripheral myelin protein gene (PMP22) in human chromosome 17p12 are associated with neuropathy: duplications cause Charcot-Marie-Tooth disease type 1A (CMT1A), whereas deletions lead to hereditary neuropathy with liability to pressure palsies (HNPP). Our previous studies showed that >99% of these rearrangements are recurrent and mediated by nonallelic homologous recombination (NAHR). Rare copy number variations (CNVs) generated by nonrecurrent rearrangements also exist in 17p12, but their underlying mechanisms are not well understood. We investigated 21 subjects with rare CNVs associated with CMT1A or HNPP by oligonucleotide-based comparative genomic hybridization microarrays and breakpoint sequence analyses, and we identified 17 unique CNVs, including two genomic deletions, ten genomic duplications, two complex rearrangements, and three small exonic deletions. Each of these CNVs includes either the entire PMP22 gene, or exon(s) only, or ultraconserved potential regulatory sequences upstream of PMP22, further supporting the contention that PMP22 is the critical gene mediating the neuropathy phenotypes associated with 17p12 rearrangements. Breakpoint sequence analysis reveals that, different from the predominant NAHR mechanism in recurrent rearrangement, various molecular mechanisms, including nonhomologous end joining, Alu-Alu-mediated recombination, and replication-based mechanisms (e.g., FoSTeS and/or MMBIR), can generate nonrecurrent 17p12 rearrangements associated with neuropathy. We document a multitude of ways in which gene function can be altered by CNVs. Given the characteristics, including small size, structural complexity, and location outside of coding regions, of selected rare CNVs, their identification remains a challenge for genome analysis. Rare CNVs may potentially represent an important portion of "missing heritability" for human diseases.

  12. X-linked Charcot-Marie-Tooth (CMT) neuropathies (CMTX1, CMTX2, CMTX3) show different clinical phenotype and molecular genetics

    SciTech Connect

    Ionasescu, V.V.; Searby, C.C.; Ionasescu, R.

    1994-09-01

    The purpose of this study was to compare the X-linked dominant type CMTX1 (20 families) with X-linked recessive types CMTX2 and CMTX3 (2 families). The clinical phenotype was consistent with CMT peripheral neuropathy in all cases including distal weakness, atrophy and sensory loss, pes cavus and areflexia. Additional clinicial involvement of the central nervous system was present in one family with CMTX2 (mental retardation) and one family with CMTX3 (spastic paraparesis). Tight genetic linkage to Xq13.1 was present in 20 families with CMTX1 (Z=34.07 at {theta}=0) for the marker DXS453. Fifteen of the CMTX1 families showed point mutations of the connexin 32 coding region (5 nonsense mutations, 8 missense mutations, 2 deletions). Five CMTX1 neuropathy families showed no evidence of point mutations of the CX32 coding sequence. These findings suggest that the CMTX1 neuropathy genotype in these families may be the result of promoter mutations, 3{prime}-untranslated region mutations or exon/intron splice site mutations or a mutation with a different type of connexin but which has close structural similarities to CX32. No mutations of the CX32 coding region were found in the CMTX2 or CMTX3 families. Linkage to Xq13.1 was excluded in both families. Genetic linkage to Xp22.2 was present in the CMTX2 family (Z=3.54 at {theta}=0) for the markers DXS987 and DXS999. Suggestion of linkage to Xq26 (Z=1.81 at {theta}=0) for the marker DXS86 was present in the CMTX3 family.

  13. GJB1-associated X-linked Charcot-Marie-Tooth disease, a disorder affecting the central and peripheral nervous systems.

    PubMed

    Abrams, Charles K; Freidin, Mona

    2015-06-01

    Charcot-Marie-Tooth disease (CMT) is a group of inherited diseases characterized by exclusive or predominant involvement of the peripheral nervous system. Mutations in GJB1, the gene encoding Connexin 32 (Cx32), a gap-junction channel forming protein, cause the most common X-linked form of CMT, CMT1X. Cx32 is expressed in Schwann cells and oligodendrocytes, the myelinating glia of the peripheral and central nervous systems, respectively. Thus, patients with CMT1X have both central and peripheral nervous system manifestations. Study of the genetics of CMT1X and the phenotypes of patients with this disorder suggest that the peripheral manifestations of CMT1X are likely to be due to loss of function, while in the CNS gain of function may contribute. Mice with targeted ablation of Gjb1 develop a peripheral neuropathy similar to that seen in patients with CMT1X, supporting loss of function as a mechanism for the peripheral manifestations of this disorder. Possible roles for Cx32 include the establishment of a reflexive gap junction pathway in the peripheral and central nervous system and of a panglial syncitium in the central nervous system.

  14. Characterization of non-CG genomic hypomethylation associated with gamma-ray-induced suppression of CMT3 transcription in Arabidopsis thaliana.

    PubMed

    Kim, Ji Eun; Lee, Min Hee; Cho, Eun Ju; Kim, Ji Hong; Chung, Byung Yeoup; Kim, Jin-Hong

    2013-12-01

    Ionizing radiation causes various epigenetic changes, as well as a variety of DNA lesions such as strand breaks, cross-links, oxidative damages, etc., in genomes. However, radiation-induced epigenetic changes have rarely been substantiated in plant genomes. The current study investigates whether DNA methylation of Arabidopsis thaliana genome is altered by gamma rays. We found that genomic DNA methylation decreased in wild-type plants with increasing doses of gamma rays (5, 50 and 200 Gy). Irradiation with 200 Gy significantly increased the expression of transcriptionally inactive centromeric 180-bp (CEN) and transcriptionally silent information (TSI) repeats. This increase suggested that there was a substantial release of transcriptional gene silencing by gamma rays, probably by induction of DNA hypomethylation. High expression of the DNA demethylase ROS1 and low expression of the DNA methyltransferase CMT3 supported this hypothesis. Moreover, Southern blot analysis following digestion of genomic DNA with methylation-sensitive enzymes revealed that the DNA hypomethylation occured preferentially at CHG or CHH sites rather than CG sites, depending on the radiation dose. Unlike CEN and TSI repeats, the number of Ta3, AtSN1 and FWA repeats decreased in transcription but increased in non-CG methylation. In addition, the cmt3-11 mutant showed neither DNA hypomethylation nor transcriptional activation of silenced repeats upon gamma irradiation. Furthermore, profiles of genome-wide transcriptomes in response to gamma rays differed between the wild-type and cmt3-11 mutant. These results suggest that gamma irradiation induced DNA hypomethylation preferentially at non-CG sites of transcriptionally inactive repeats in a locus-specific manner, which depends on CMT3 activity.

  15. IGF-I and retinoic acid regulate the distribution pattern of IGFBPs synthesized by the canine mammary tumor cell line CMT-U335.

    PubMed

    Oosterlaken-Dijksterhuis, M A; Kwant, M M; Slob, A; Hellmén, E; Mol, J A

    1999-03-01

    Stromal-epithelial interactions modulate growth and development in normal and neoplastic mammary gland. The release of IGF binding proteins (IGFBPs) by the stromal compartment of the mammary gland may play a modulating role in the IGF-mediated proliferation of mammary epithelium. Therefore, the IGFBP-expression pattern of the canine mammary tumor cell line U335 (CMT-U335), which has a mesenchymal phenotype, was determined. In addition, the effects of IGFs and all trans retinoic acid (RA) on DNA synthesis, and IGFBP secretion and distribution were examined. The IGFBPs secreted by CMT-U335 were characterized as IGFBP-2, -4, -5, and -6. Moreover, CMT-U335 appeared to be a suitable mammary mesenchymal cell line for study of the regulatory factors of IGFBP expression and the mechanism(s) involved. IGFs and RA enhanced IGFBP concentrations in cell-conditioned medium with IGF-I and RA having an additive effect. The IGF-I-stimulated DNA synthesis, however, was inhibited by RA. The difference between IGF-I and RA was an enhanced IGFBP-5 binding to the extracellular matrix (ECM) by RA, whereas IGF-I reduced binding to the ECM. Because high doses of insulin had no significant effects on IGFBP concentrations in the medium, it is concluded that IGF-I-induced changes in IGFBP concentrations are not mediated by type-IIGF receptors and may be the consequence of IGFBP redistribution.

  16. Improved testing for CMT1A and HNPP using multiplex ligation-dependent probe amplification (MLPA) with rapid DNA preparations: comparison with the interphase FISH method.

    PubMed

    Slater, Howard; Bruno, Damien; Ren, Hua; La, Phung; Burgess, Trent; Hills, Louise; Nouri, Sara; Schouten, Jan; Choo, K H Andy

    2004-08-01

    Charcot-Marie-Tooth disease type 1A (CMT1A) and hereditary neuropathy with liability to pressure palsies (HNPP) are the two most common peripheral neuropathies, with incidences of about 1 in 2,500. Several techniques can be used to detect the typical 1.5-Mb duplication or deletion associated with these respective conditions, but none combines simplicity with high sensitivity. MLPA is a new technique for measuring sequence dosage. We have assessed its performance for the detection of the specific 1.5-Mb duplication/deletion by prospectively testing 50 patients referred with differential diagnoses of CMT or HNPP. Probes were designed to evaluate the TEKT3, PMP22, and COX10 genes within the CMT1A/HNPP region. We have compared the results with our existing fluorescence in situ hybridization (FISH) assay, which was performed in parallel. There was concordance of results for 49 patients. Of note, one patient showed an intermediate multiplex ligation-dependent probe amplification (MLPA) result with an abnormal FISH result, which is consistent with mosaicism. The assay works equally well with either purified DNA or rapid DNA preparations made by direct cell lysis. The use of the latter significantly reduces the cost of the assay. MLPA is a sensitive, specific, robust, and cost-effective technique suitable for fast, high-throughput testing and offers distinct advantages over other testing methods.

  17. A novel Gypsy founder mutation, p.Arg1109X in the CMT4C gene, causes variable peripheral neuropathy phenotypes

    PubMed Central

    Gooding, R; Colomer, J; King, R; Angelicheva, D; Marns, L; Parman, Y; Chandler, D; Bertranpetit, J; Kalaydjieva, L

    2005-01-01

    Background: Linkage, haplotype and sequencing analysis in a large Spanish Gypsy kindred with multiple members affected by autosomal recessive peripheral neuropathy led to the identification of a novel mutation, p.Arg1109X, in the CMT4C gene. The screening of further unrelated patients, and of a panel of ethnically matched controls, showed that p.Arg1109X is an ancestral mutation which occurs in Gypsy populations across Europe and is the most common cause of autosomal recessive Charcot–Marie–Tooth disease in Spanish Gypsies. Objective: To report the identification of a novel Gypsy founder mutation causing autosomal recessive CMT4C disease in a sample of homozygous affected individuals. Results: The mutation was associated with a surprisingly broad spectrum of neuropathy phenotypes, with variation in the age at onset, rate of progression, severity of muscle and sensory involvement, the presence of scoliosis, and cranial nerve involvement. Conclusions: Ascertainment and further studies of CMT4C patients in this population will provide a unique opportunity for characterising the full range of clinical manifestations of the disease in a genetically homogeneous sample. PMID:16326826

  18. Development and Optimization of a Dedicated, Hybrid Dual-Modality SPECT-CmT System for Improved Breast Lesion Diagnosis

    DTIC Science & Technology

    2008-01-01

    bars were mounted on either end of the plate to secure the CmT tube and detector. The CmT system was laterally offset 5cm from the center of rotation...Motor Camera P ro je ct io ns R ec on st ru ct io ns 7 At the end , a sequential scan where SPECT and CmT images would be separately...controlled together from one computer during image acquisition. This software was originally promised to be written up by the end of Year 1. However

  19. CMT for materials science

    SciTech Connect

    Kinney, J.

    1997-02-01

    This session is comprised of two articles by John Kinney describing biomedical and other uses for computerized tomography. In the first article, Kinney describes the use of a three-dimensional x-ray tomographic microscope to image the trabecular bone architecture of the proximal tibias of rats in vivo. Research in this field may help to detect the earliest stages of hypoestrogenemic bone loss and may help to more rapidly test the effectiveness of new clinical treatments for this major public health problem. The second article describes recent advances in X-ray tomography using synchrotron radiation to evaluate microstructures in ceramic matrix composites, bone loss in osteoporosis, and the development of carries lesions in teeth.

  20. A 1.5-Mb cosmid contig of the CMT1A duplication/HNPP deletion critical region in 17p11.2-p12

    SciTech Connect

    Murakami, Tatsufumi; Lupski, J.R.

    1996-05-15

    Charcot-Marie-Tooth disease type 1A (CMT1A) is associated with a 1.5-Mb tandem duplication in chromosome 17p11.2-p12, and hereditary neuropathy with liability to pressure palsies (HNPP) is associated with a 1.5-Mb deletion at this locus. Both diseases appear to result from an altered copy number of the peripheral myelin protein-22 gene, PMP22, which maps within the critical region. To identify additional genes and characterize chromosomal elements, a 1.5-Mb cosmid contig of the CMT1A duplication/HNPP deletion critical region was assembled using a yeast artificial chromosome (YAC)-based isolation and binning strategy. Whole YAC probes were used for screening a high-density arrayed chromosome 17-specific cosmid library. Selected cosmids were spotted on dot blots and assigned to bins defined by YACs. This binning of cosmids facilitated the subsequent fingerprint analysis. The 1.5-Mb region was covered by 137 cosmids with a minimum overlap set of 52 cosmids assigned to 17 bins and 9 contigs. 20 refs., 2 figs.

  1. A 1.5-Mb cosmid contig of the CMT1A duplication/HNPP deletion critical region in 17p11.2-p12.

    PubMed

    Murakami, T; Lupski, J R

    1996-05-15

    Charcot-Marie-Tooth disease type 1A (CMT1A) is associated with a 1. 5-Mb tandem duplication in chromosome 17p11.2-p12, and hereditary neuropathy with liability to pressure palsies (HNPP) is associated with a 1.5-Mb deletion at this locus. Both diseases appear to result from an altered copy number of the peripheral myelin protein-22 gene, PMP22, which maps within the critical region. To identify additional genes and characterize chromosomal elements, a 1.5-Mb cosmid contig of the CMT1A duplication/HNPP deletion critical region was assembled using a yeast artificial chromosome (YAC)-based isolation and binning strategy. Whole YAC probes were used for screening a high-density arrayed chromosome 17-specific cosmid library. Selected cosmids were spotted on dot blots and assigned to bins defined by YACs. This binning of cosmids facilitated the subsequent fingerprint analysis. The 1.5-Mb region was covered by 137 cosmids with a minimum overlap set of 52 cosmids assigned to 17 bins and 9 contigs.

  2. Enigma of earthquakes at ridge-transform-fault plate boundaries - Distribution of non-double couple parameter of Harvard CMT solutions. [Centroid Moment Tensors

    SciTech Connect

    Kawakatsu, Hitoshi )

    1991-06-01

    The distribution of the non-double couple parameter of shallow earthquakes reported in the Harvard CMT catalogue shows systematic characteristics depending on the epicentral locations and types of fault mechanisms. The authors suggest that they can be explained by the presence of subevents with different double couple mechanisms in a single rupture sequence. The earthquakes at the ridge-transform-fault plate boundaries show a particularly interesting pattern. It is suggested that two types of faulting expected in the area (i.e., normal faults at ridges and strike slip faults at transform-faults) tend to occur almost simultaneously, although this hypothesis needs to be delineated by careful analyses using bodywave waveforms.

  3. Matrix and impurity element distributions in CdHgTe (CMT) and (Cd,Zn)(Te,Se) compounds by chemical analysis

    NASA Astrophysics Data System (ADS)

    Capper, P.; O'Keefe, E. S.; Maxey, C.; Dutton, D.; Mackett, P.; Butler, C.; Gale, I.

    1996-04-01

    This review describes several of the main techniques used to determine matrix element distributions and those which can provide a survey of impurity levels and assess deliberate doping concentrations in Cd xHg 1 - xTe and CdTe-based substrate materials. The most widely used method to non-destructively determine x is that of Fourier transform infrared (FTIR) spectrometry and lateral x variations in current bulk, LPE and MOVPE material measured by this technique will be presented. Auger electron spectrometry (AES) has been used on bevelled samples to assess variations in x with depth and interface widths in LPE, MOVPE and MBE layers and examples will be given. Near IR spectrometry is also now being used to monitor the variations in Zn and Se content, in CdZnTe and CdTeSe respectively, and results in this area will be described along with measurements of Zn on the micro-scale using AES. All of these techniques need to be calibrated against an absolute chemical analysis technique and we have used atomic absorption spectrometry (AAS). The latter technique also provides the accurate measure of dopant and impurity elements to standardise other techniques. Secondary ion mass spectrometry (SIMS) is mainly used for the determination of dopant depth distributions while laser scan mass spectrometry (LSMS) has the unique capability of providing a survey of low levels of impurities in thin epitaxial layers. Depth profiles of arsenic and iodine in MOVPE heterostructures, using SIMS, will be given. Impurity surveys, using LSMS, in bulk CMT and substrate materials and in CMT epitaxial layers grown by LPE, MOVPE and MBE will be described. Reported glow discharge mass spectrometry (GDMS) results on substrate materials will be compared to the present results.

  4. The 1.4-Mb CMT1A duplication/HNPP deletion genomic region reveals unique genome architectural features and provides insights into the recent evolution of new genes.

    PubMed

    Inoue, K; Dewar, K; Katsanis, N; Reiter, L T; Lander, E S; Devon, K L; Wyman, D W; Lupski, J R; Birren, B

    2001-06-01

    Duplication and deletion of the 1.4-Mb region in 17p12 that is delimited by two 24-kb low copy number repeats (CMT1A-REPs) represent frequent genomic rearrangements resulting in two common inherited peripheral neuropathies, Charcot-Marie-Tooth disease type 1A (CMT1A) and hereditary neuropathy with liability to pressure palsy (HNPP). CMT1A and HNPP exemplify a paradigm for genomic disorders wherein unique genome architectural features result in susceptibility to DNA rearrangements that cause disease. A gene within the 1.4-Mb region, PMP22, is responsible for these disorders through a gene-dosage effect in the heterozygous duplication or deletion. However, the genomic structure of the 1.4-Mb region, including other genes contained within the rearranged genomic segment, remains essentially uncharacterized. To delineate genomic structural features, investigate higher-order genomic architecture, and identify genes in this region, we constructed PAC and BAC contigs and determined the complete nucleotide sequence. This CMT1A/HNPP genomic segment contains 1,421,129 bp of DNA. A low copy number repeat (LCR) was identified, with one copy inside and two copies outside of the 1.4-Mb region. Comparison between physical and genetic maps revealed a striking difference in recombination rates between the sexes with a lower recombination frequency in males (0.67 cM/Mb) versus females (5.5 cM/Mb). Hypothetically, this low recombination frequency in males may enable a chromosomal misalignment at proximal and distal CMT1A-REPs and promote unequal crossing over, which occurs 10 times more frequently in male meiosis. In addition to three previously described genes, five new genes (TEKT3, HS3ST3B1, NPD008/CGI-148, CDRT1, and CDRT15) and 13 predicted genes were identified. Most of these predicted genes are expressed only in embryonic stages. Analyses of the genomic region adjacent to proximal CMT1A-REP indicated an evolutionary mechanism for the formation of proximal CMT1A-REP and the

  5. Homologous DNA exchanges in humans can be explained by the yeast double-strand break repair model: a study of 17p11.2 rearrangements associated with CMT1A and HNPP.

    PubMed

    Lopes, J; Tardieu, S; Silander, K; Blair, I; Vandenberghe, A; Palau, F; Ruberg, M; Brice, A; LeGuern, E

    1999-11-01

    Rearrangements in 17p11.2, responsible for the 1.5 Mb duplications and deletions associated, respectively, with autosomal dominant Charcot-Marie-Tooth type 1A disease (CMT1A) and hereditary neuropathy with liability to pressure palsies (HNPP) are a suitable model for studying human recombination. Rearrangements in 17p11.2 are caused by unequal crossing-over between two homologous 24 kb sequences, the CMT1A-REPs, that flank the disease locus and occur in most cases within a 1.7 kb hotspot. We sequenced this hotspot in 28 de novo patients (25 CMT1A and three HNPP), in order to localize precisely, at the DNA sequence level, the crossing-overs. We show that some chimeric CMT1A-REPs in de novo patients (10/28) present conversion of DNA segments associated with the crossing-over. These rearrangements can be explained by the double-strand break (DSB) repair model described in yeast. Fine mapping of the de novo rearrangements provided evidence that the successive steps of this model, heteroduplex DNA formation, mismatch correction and gene conversion, occurred in patients. Furthermore, the model explains 17p11.2 recombinations between chromosome homologues as well as between sister chromatids. In addition, defective mismatch repair of the heteroduplex DNA, observed in two patients, resulted in two heterozygous chimeric CMT1A-REPs which can be explained, as in yeast, by post-meiotic segregation. This work supports the hypothesis that the DSB repair model of DNA exchange may apply universally from yeasts to humans.

  6. Near-regional CMT and multiple-point source solution of the September 5, 2012, Nicoya, Costa Rica Mw 7.6 (GCMT) earthquake

    NASA Astrophysics Data System (ADS)

    Quintero, Ronnie; Zahradník, Jiri; Sokos, Efthimios

    2014-11-01

    We use acceleration data from the Observatorio Vulcanologico y Sismologico, Universidad Nacional de Costa Rica (OVSICORI-UNA) and Laboratorio de Ingenieria Sismica, Universidad de Costa Rica (LIS-UCR) seismic network for the relocation and moment-tensor solution of the September 5, 2012, 14:42:03.35 UTC, Nicoya, Costa Rica earthquake (Mw 7.6 GCMT). Using different relocation methods we found a stable earthquake hypocenter, near the original OVSICORI-UNA location in the Nicoya Peninsula, NW Costa Rica at Lat 9.6943°N, Lon 85.5689°W, depth 15.3 km, associated with the subduction of the Cocos plate under Caribbean plate. Acceleration records at OVSICORI-UNA and LIS-UCR stations (94-171 km), at 0.03 < f < 0.06 Hz were used in the waveform inversion for a single-point centroid moment tensor (CMT). Using spatial grid search the centroid position was found at the depth of 30 km, situated at Lat 10.0559°N, Lon 85.4778°W, i.e. of about 41 km NNE from the epicenter. The centroid time is 14:42:18.89 UTC, i.e. 15.54 s later relative to the location-based origin time. The nodal plane (strike 318°, dip 27° and rake 115°) is the fault plane that agrees with the geometry of the subducted slab at Nicoya, NNW Costa Rica. Increasing the maximum studied frequency from 0.06 to 0.15 Hz, the multiple point source inversion model leads to two subevents. The first one was located near the centroid and the second subevent was situated 20 km along strike and 10 km down dip from the first subevent and 6 s later. The uncertainty of the source model was carefully examined using complementary inversion methods, viz the iterative deconvolution and non-negative least squares.

  7. Effect of intramammary infusion of recombinant bovine GM-CSF and IL-8 on CMT score, somatic cell count, and milk mononuclear cell populations in Holstein cows with Staphylococcus aureus subclinical mastitis.

    PubMed

    Kiku, Yoshio; Ozawa, Tomomi; Takahashi, Hideyuki; Kushibiki, Shiro; Inumaru, Shigeki; Shingu, Hiroyuki; Nagasawa, Yuya; Watanabe, Atsushi; Hata, Eiji; Hayashi, Tomohito

    2017-03-09

    The effect of intramammary infusion of recombinant bovine granulocyte-macrophage colony-stimulating factor (rbGM-CSF) and interleukin-8 (rbIL-8) on mononuclear cell populations in quarters, somatic cell count (SCC) and the California Mastitis Test (CMT) score were investigated. From the selected cows with naturally occurring Staphylococcus aureus subclinical mastitis, one quarter of each cow were selected for the infusions of rbGM-CSF (400 μg/5 mL/quarter, n = 9), rbIL-8 (1 mg/5 mL/quarter, n = 9), and phosphate-buffered saline (5 mL/quarter, n = 7). The CMT score of both cytokines post infusion temporarily increased between days 0 and 1 and significantly decreased between days 7 and 14 compared to the preinfusion level. The SCC on day 14 after infusions of rbGM-CSF tended to be lower than that of the control group. The percentage of CD14+ cells increased on days 1 and 2 post infusion of rbGM-CSF. The percentage of CD4+ and CD8+ cells also increased on days 2 and 3, suggesting that the infusion of rbGM-CSF enhanced cellular immunity in the mammary gland. In contrast, the percentage of CD14+ cells decreased on days 0.25 and 1 post infusion of rbIL-8. No significant changes in the percentages of CD4+ and CD8+ cells in milk after infusion of rbIL-8 were evident during the experimental period, which suggested that rbIL-8 had little effect on the function of T cells in the mammary gland. These results indicated that rbGM-CSF and rbIL-8 decreased the CMT score by a different mechanism and may have a potential as therapeutic agents for subclinical mastitis.

  8. CMT for soil science applications

    SciTech Connect

    Clausnitzer, V.; Hopmans, J.W.

    1997-02-01

    Today, x-ray computed microtomography provides us with the ability to noninvasively measure porous-media properties at a scale approaching 10 {mu}m. In contrast, traditional measurement techniques are either destructive or invasive, while still providing only limited information. Because the output from x-ray CT is directly related to density and atomic number, it is well suited for phase identification and concentration measurements.

  9. Progress in molecular diagnosis of Charcot-Marie-Tooth-disease type 1 (CMT 1, HMSN I) and hereditary neuropathy with liability to pressure palsies (HNPP) by fluorescence in situ hybridization (FISH)-detection of a potential genetic mosaicism

    SciTech Connect

    Bathke, K.; Liehr. T.; Ekici, A.

    1994-09-01

    We tested 20 CMT 1 patients characterized according to the criteria of the European CMT consortium by Southern hybridization of MspI restricted genomic DNA with probes pVAW409R1, pVAW412Hec and pEW401HE. In 11 of the 20 CMT 1 cases (55%), we observed a duplication in 17q11.2; one patient had a dinucleotide insertion in exon 6 of the PO-gene (5%). One HNPP case had a typical 17p11.2 deletion. Analysis of CA-repeats was performed with primers RM11GT and Mfd41; SSCP-analysis of the PO, PMP22 and Cx32-genes is in progress. FISH was carried out with probe pVAW409R1. 125 interphase nuclei were analyzed for each proband by counting the signals per nucleus. Normal cells show a characteristic distribution of signals: 1 signal in 5.9% of nuclei, 2 in 86.3% and 3 in 7.8%. A duplication is indicated by a shift to 3 signals in more than approximately 60% and 2 in less than 25% of the nuclei. In contrast, the 17p11.2 deletion of the HNPP patient shifts to 82.4% of nuclei with a single hybridization signal versus 14.4% with 2 signals. We detected one case with significantly abnormal distribution of interphase nuclei hybridization signals compared to cultures of normal cells and to those with 17p11.2 duplication or deletion: 3.2% nuclei revealed 1 signal, 48.0% two signals and 48.8% 3 signals, indicating a pathogenic but moderate dosis increase compared to the throughout duplicated cases. FISH with probe pVAW409R1 is a versatile tool to detect the HNPP deletion both in interphase nuclei and in metaphase chromosomes. In CMT 1 disease interphase nuclei are required for FISH analysis due to the small duplication of 1.5 Mbp. In contrast to Southern techniques, FISH is able to detect genetic mosaicism.

  10. The Evolutionary Chromosome Translocation 4;19 in Gorilla gorilla is Associated with Microduplication of the Chromosome Fragment Syntenic to Sequences Surrounding the Human Proximal CMT1A-REP

    PubMed Central

    Stankiewicz, Pawel; Park, Sung-Sup; Inoue, Ken; Lupski, James R.

    2001-01-01

    Many genomic disorders occur as a result of chromosome rearrangements involving low-copy repeats (LCRs). To better understand the molecular basis of chromosome rearrangements, including translocations, we have investigated the mechanism of evolutionary rearrangements. In contrast to several intrachromosomal rearrangements, only two evolutionary translocations have been identified by cytogenetic analyses of humans and greater apes. Human chromosome 2 arose as a result of a telomeric fusion between acrocentric chromosomes, whereas chromosomes 4 and 19 in Gorilla gorilla are the products of a reciprocal translocation between ancestral chromosomes, syntenic to human chromosomes 5 and 17, respectively. Fluorescence in situ hybridization (FISH) was used to characterize the breakpoints of the latter translocation at the molecular level. We identified three BAC clones that span translocation breakpoints. One breakpoint occurred in the region syntenic to human chromosome 5q13.3, between the HMG-CoA reductase gene (HMGCR) and RAS p21 protein activator 1 gene (RASA1). The second breakpoint was in a region syntenic to human chromosome 17p12 containing the 24 kb region-specific low-copy repeat-proximal CMT1A-REP. Moreover, we found that the t(4;19) is associated with a submicroscopic chromosome duplication involving a 19p chromosome fragment homologous to the human chromosome region surrounding the proximal CMT1A-REP. These observations further indicate that higher order genomic architecture involving low-copy repeats resulting from genomic duplication plays a significant role in karyotypic evolution. PMID:11435402

  11. Analysis of light propagation in slotted resonator based systems via coupled-mode theory.

    PubMed

    Hiremath, Kirankumar R; Niegemann, Jens; Busch, Kurt

    2011-04-25

    Optical devices with a slot configuration offer the distinct feature of strong electric field confinement in a low refractive index region and are, therefore, of considerable interest in many applications. In this work we investigate light propagation in a waveguide-resonator system where the resonators consist of slotted ring cavities. Owing to the presence of curved material interfaces and the vastly different length scales associated with the sub-wavelength sized slots and the waveguide-resonator coupling regions on the one hand, and the spatial extent of the ring on the other hand, this prototypical system provides significant challenges to both direct numerical solvers and semi-analytical approaches. We address these difficulties by modeling the slot resonators via a frequency-domain spatial Coupled-Mode Theory (CMT) approach, and compare its results with a Discontinuous Galerkin Time-Domain (DGTD) solver that is equipped with curvilinear finite elements. In particular, the CMT model is built on the underlying physical properties of the slotted resonators, and turns out to be quite efficient for analyzing the device characteristics. We also discuss the advantages and limitations of the CMT approach by comparing the results with the numerically exact solutions obtained by the DGTD solver. Besides providing considerable physical insight, the CMT model thus forms a convenient basis for the efficient analysis of more complex systems with slotted resonators such as entire arrays of waveguide-coupled resonators and systems with strongly nonlinear optical properties.

  12. Gene expression profiling studies in regenerating nerves in a mouse model for CMT1X: uninjured Cx32-knockout peripheral nerves display expression profile of injured wild type nerves.

    PubMed

    Freidin, Mona; Asche-Godin, Samantha; Abrams, Charles K

    2015-01-01

    X-linked Charcot-Marie-Tooth disease (CMT1X) is an inherited peripheral neuropathy caused by mutations in GJB1, the human gene for Connexin32 (Cx32). This present study uses Ilumina Ref8-v2 BeadArray to examine the expression profiles of injured and uninjured sciatic nerves at 5, 7, and 14 days post-crush injury (dpi) from Wild Type (WT) and Cx32-knockout (Cx32KO) mice to identify the genes and signaling pathways that are dysregulated in the absence of Schwann cell Cx32. Given the assumption that loss of Schwann cell Cx32 disrupts the regeneration and maintenance of myelinated nerve leading to a demyelinating neuropathy in CMT1X, we initially hypothesized that nerve crush injury would result in significant increases in differential gene expression in Cx32KO mice relative to WT nerves. However, microarray analysis revealed a striking collapse in the number of differentially expressed genes at 5 and 7 dpi in Cx32KO nerves relative to WT, while uninjured and 14 dpi time points showed large numbers of differentially regulated genes. Further comparisons within each genotype showed limited changes in Cx32KO gene expression following crush injury when compared to uninjured Cx32KO nerves. By contrast, WT nerves exhibited robust changes in gene expression at 5 and 7 dpi with no significant differences in gene expression by 14dpi relative to uninjured WT nerve samples. Taken together, these data suggest that the gene expression profile in uninjured Cx32KO sciatic nerve strongly resembles that of a WT nerve following injury and that loss of Schwann cell Cx32 leads to a basal state of gene expression similar to that of an injured WT nerve. These findings support a role for Cx32 in non-myelinating and regenerating populations of Schwann cells in normal axonal maintenance in re-myelination, and regeneration of peripheral nerve following injury. Disruption of Schwann cell-axonal communication in CMT1X may cause dysregulation of signaling pathways that are essential for the

  13. Near-field tsunami forecast system based on near real-time seismic moment tensor estimation in the regions of Indonesia, the Philippines, and Chile

    NASA Astrophysics Data System (ADS)

    Inazu, Daisuke; Pulido, Nelson; Fukuyama, Eiichi; Saito, Tatsuhiko; Senda, Jouji; Kumagai, Hiroyuki

    2016-05-01

    We have developed a near-field tsunami forecast system based on an automatic centroid moment tensor (CMT) estimation using regional broadband seismic observation networks in the regions of Indonesia, the Philippines, and Chile. The automatic procedure of the CMT estimation has been implemented to estimate tsunamigenic earthquakes. A tsunami propagation simulation model is used for the forecast and hindcast. A rectangular fault model based on the estimated CMT is employed to represent the initial condition of tsunami height. The forecast system considers uncertainties due to two possible fault planes and two possible scaling laws and thus shows four possible scenarios with these associated uncertainties for each estimated CMT. The system requires approximately 15 min to estimate the CMT after the occurrence of an earthquake and approximately another 15 min to make the tsunami forecast results including the maximum tsunami height and its arrival time at the epicentral region and near-field coasts available. The retrospectively forecasted tsunamis were evaluated by the deep-sea pressure and tide gauge observations, for the past eight tsunamis ( M w 7.5-8.6) that occurred throughout the regional seismic networks. The forecasts ranged from half to double the amplitudes of the deep-sea pressure observations and ranged mostly within the same order of magnitude as the maximum heights of the tide gauge observations. It was found that the forecast uncertainties increased for greater earthquakes (e.g., M w > 8) because the tsunami source was no longer approximated as a point source for such earthquakes. The forecast results for the coasts nearest to the epicenter should be carefully used because the coasts often experience the highest tsunamis with the shortest arrival time (e.g., <30 min).

  14. Conformational changes associated with L16P and T118M mutations in the membrane-embedded PMP22 protein, consequential in CMT-1A.

    PubMed

    Bello, Martiniano; Torres, Mixtli J; Méndez-Tenorio, Alfonso; Correa-Basurto, José

    2016-10-05

    Peripheral myelin protein 22 (PMP22) resides in the plasma membrane and is required for myelin formation in the peripheral nervous system. Excess PMP22 mutants accumulate in the endoplasmic reticulum (ER) resulting in the inherited neuropathies of Charcot-Marie-Tooth disease. However, there was no evidence of the structure of PMP22 or how mutations affect its folding. Therefore, in this study, we combined bioinformatics and homology modeling approaches to obtain three-dimensional native and mutated PMP22 models and its anchoring to a POPC membrane, submitted to .5-μs MD simulations, to determine how the L16P and T118M mutations affect the conformational behavior of PMP22. In addition, we investigated the ability of the native and mutated species to accumulate in the ER, via interaction with RER1, by combining protein-protein docking and MD simulations, taking the conformations that were most representative of the native and mutated PMP22 systems and RER1 conformations. Principal component analysis over MD simulations revealed that L16P and T118M mutations resulted in increased structural instability compared to the native form, which is consistent with previous experimental findings of increased structural fluctuations along a loop connecting transmembrane α-helix1 and α-helix2. Docking and MD simulations coupled with the MMGBSA approach allowed the identification that the binding interface for the PMP22-RER1 complex takes place through transmembrane α-helix1 and α-helix2, with higher effective binding free energy values between the mutated PMP22 systems and RER1 than for the native PMP22, mainly through van der Waals interactions.

  15. The evolution of ANL CMT gloveboxes

    SciTech Connect

    Malecha, R. F.; Frigo, A. A.; Preuss, D. E.

    2000-07-06

    This report summarizes the following topics: the design approach based upon user-friendly concepts; utilization of existing component designs; cost effectiveness; schedule; and adaptable to project process changes without losing overall effectiveness of user-friendly approach.

  16. CMT for transport in porous media

    SciTech Connect

    Schwartz, L.

    1997-02-01

    This session is comprised of an outline of uses for x-ray microtomography in the field of petroleum geology. Calculations, diagrams, and color photomicrographs depict the many applications of synchrotron x-ray microtomograpy in determining transport properties and fluid flow characteristics of reservoir rocks, micro-porosity in carbonates, and aspects of multi-phase transport.

  17. Systemic oxygen therapy versus oral enalapril for treatment of diabetic macular ischemia: a randomized controlled trial.

    PubMed

    Sharifipour, Farideh; Razzaghi, Mohammadreza; Ramezani, Alireza; Azarmina, Mohsen; Yaseri, Mehdi; Soheilian, Roham; Soheilian, Masoud

    2016-04-01

    The purpose of this study was to evaluate the structural and functional effects of systemic oxygen therapy and enalapril in patients with diabetic macular ischemia (DMI). This randomized clinical trial consisted of 105 eyes with DMI divided into three groups. Group I received systemic oxygen by face mask at a flow rate of 10 L/min; Group II received 5 mg enalapril daily; and Group III received placebo tablets for 3 months. Best-corrected visual acuity (BCVA), central macular thickness (CMT) measured by optical coherence tomography (OCT), extent of foveal avascular zone (FAZ) on fluorescein angiograms, and electroretinograms (ERG) were obtained at baseline and after 3 and 6 months. Overall, 102 patients completed the study. Baseline characteristics were not significantly different among groups. Significant improvement in BCVA and decrease in CMT and FAZ occurred at months 3 and 6 in oxygen group compared to deterioration in enalapril and control groups (All P values <0.001). ERG parameters were significantly better in oxygen group compared to enalapril group at months 3 and 6 and better than those in control group at month 3. Normobaric oxygen therapy for 3 months in DMI decreased CMT and FAZ and improved BCVA and ERG parameters. Enalapril did not show any favorable effect.

  18. Revisiting the thermodynamic relations in AdS /CMT models

    NASA Astrophysics Data System (ADS)

    Hyun, Seungjoon; Park, Sang-A.; Yi, Sang-Heon

    2017-03-01

    Motivated by the recent unified approach to the Smarr-like relation of anti-de Sitter (AdS) planar black holes in conjunction with the quasilocal formalism on conserved charges, we revisit the quantum statistical and thermodynamic relations of hairy AdS planar black holes. By extending the previous results, we identify the hairy contribution in the bulk and show that the holographic computation can be improved so that it is consistent with the bulk computation. We argue that the first law can be retained in its universal form and that the relation between the on-shell renormalized Euclidean action and its free energy interpretation in gravity may also be undeformed even with the hairy contribution in hairy AdS black holes.

  19. High resolution x-ray CMT: Reconstruction methods

    SciTech Connect

    Brown, J.K.

    1997-02-01

    This paper qualitatively discusses the primary characteristics of methods for reconstructing tomographic images from a set of projections. These reconstruction methods can be categorized as either {open_quotes}analytic{close_quotes} or {open_quotes}iterative{close_quotes} techniques. Analytic algorithms are derived from the formal inversion of equations describing the imaging process, while iterative algorithms incorporate a model of the imaging process and provide a mechanism to iteratively improve image estimates. Analytic reconstruction algorithms are typically computationally more efficient than iterative methods; however, analytic algorithms are available for a relatively limited set of imaging geometries and situations. Thus, the framework of iterative reconstruction methods is better suited for high accuracy, tomographic reconstruction codes.

  20. Novel and tightly regulated resorcinol and cumate-inducible expression systems for Streptomyces and other actinobacteria.

    PubMed

    Horbal, Liliya; Fedorenko, Victor; Luzhetskyy, Andriy

    2014-10-01

    Inducible expression is a versatile genetic tool for controlling gene transcription, determining gene functions and other uses. Herein, we describe our attempts to create several inducible systems based on a cumate or a resorcinol switch, a hammerhead ribozyme, the LacI repressor, and isopropyl β-d-thiogalactopyranoside (IPTG). We successfully developed a new cumate (p-isopropylbenzoic acid)-inducible gene switch in actinobacteria that is based on the CymR regulator, the operator sequence (cmt) from the Pseudomonas putida cumate degradation operon and P21 synthetic promoter. Resorcinol-inducible expression system is also functional and is composed of the RolR regulator and the PA3 promoter fused with the operator (rolO) from the Corynebacterium glutamicum resorcinol catabolic operon. Using the gusA (β-glucuronidase) gene as a reporter, we showed that the newly generated expression systems are tightly regulated and hyper-inducible. The activity of the uninduced promoters is negligible in both cases. Whereas the induction factor reaches 45 for Streptomyces albus in the case of cumate switch and 33 in the case of resorcinol toggle. The systems are also dose-dependent, which allows the modulation of gene expression even from a single promoter. In addition, the cumate system is versatile, given that it is functional in different actinomycetes. Finally, these systems are nontoxic and inexpensive, as these are characteristics of cumate and resorcinol, and they are easy to use because inducers are water-soluble and easily penetrate cells. Therefore, the P21-cmt-CymR and PA3-rolO-RolR systems are powerful tools for engineering actinobacteria.

  1. Microtubule dynamics in the peripheral nervous system: A matter of balance.

    PubMed

    Almeida-Souza, Leonardo; Timmerman, Vincent; Janssens, Sophie

    2011-11-01

    The special architecture of neurons in the peripheral nervous system, with axons extending for long distances, represents a major challenge for the intracellular transport system. Two recent studies show that mutations in the small heat shock protein HSPB1, which cause an axonal type of Charcot-Marie-Tooth (CMT) neuropathy, affect microtubule dynamics and impede axonal transport. Intriguingly, while at presymptomatic age the neurons in the mutant HSPB1 mouse show a hyperstable microtubule network, at postsymptomatic age, the microtubule network completely lost its stability as reflected by a marked decrease in tubulin acetylation levels. We here propose a model explaining the role of microtubule stabilization and tubulin acetylation in the pathogenesis of HSPB1 mutations.

  2. Stuck in traffic: an emerging theme in diseases of the nervous system.

    PubMed

    Neefjes, Jacques; van der Kant, Rik

    2014-02-01

    The past decade has seen an explosion of DNA sequencing activities and many mutations and genetic variances underlying neurological and neurodegenerative diseases have been determined. This wealth of genetic data is now placed in molecular pathways revealing the nodes that underlie the disrupted processes. Many mutations in neurological diseases affect proteins controlling endosomal/lysosomal transport. Although the age of onset of these diseases range from juvenile [i.e., Niemann-Pick type C (NPC) and Charcot-Marie-Tooth (CMT) disease] to late onset (Parkinson's and Alzheimer's disease), deregulation of endosomal transport is a common theme. This review summarizes how elucidating the genetic basis for the various neurological diseases has advanced our understanding of the endo-lysosomal system and why the various mutations all translate into similar disease phenotypes.

  3. Tunable triple Fano resonances based on multimode interference in coupled plasmonic resonator system.

    PubMed

    Li, Shilei; Zhang, Yunyun; Song, Xiaokang; Wang, Yilin; Yu, Li

    2016-07-11

    In this paper, an asymmetric plasmonic structure composed of two MIM (metal-insulator-metal) waveguides and two rectangular cavities is reported, which can support triple Fano resonances originating from three different mechanisms. And the multimode interference coupled mode theory (MICMT) including coupling phases is proposed based on single mode coupled mode theory (CMT), which is used for describing and explaining the multiple Fano resonance phenomenon in coupled plasmonic resonator systems. Just because the triple Fano resonances originate from three different mechanisms, each Fano resonance can be tuned independently or semi-independently by changing the parameters of the two rectangular cavities. Such, a narrow 'M' type of double Lorentzian-like line-shape transmission windows with the position and the full width at half maximum (FWHM) can be tuned freely is constructed by changing the parameters of the two cavities appropriately, which can find widely applications in sensors, nonlinear and slow-light devices.

  4. Cochlear implantation in a patient with deafness induced by Charcot-Marie-Tooth disease (hereditary motor and sensory neuropathies).

    PubMed

    Postelmans, J T F; Stokroos, R J

    2006-06-01

    Charcot-Marie-Tooth disease (CMT), also named hereditary motor and sensory neuropathies (HMSN), comprises a clinically and genetically heterogeneous group of disorders affecting the peripheral nervous system. Deafness induced by CMT is clinically distinct among the genetically heterogeneous group of CMT disorders. Deafness in CMT patients is associated with point mutations or deletions in the transmembrane domain in the peripheral myelin gene (PMP) 22, which are in close proximity to the extracellular component of this gene. We present a patient with deafness induced by CMT type 1A, undergoing cochlear implantation. Prior investigations showed good results due to replacing a synchronous impulse by means of cochlear implantation in patients with auditory neuropathy.

  5. Multicloud parametrization of mesoscale convective systems for the ITCZ

    NASA Astrophysics Data System (ADS)

    Khouider, B.; Moncrieff, M. W.

    2014-12-01

    Mesoscale convective systems (MCS), aligned approximately parallel to the background low-level wind shear, are ubiquitous in the Eastern Pacific intertropical convergence zone (ITCZ). They are believed to control the local Hadley circulation and have a nontrivial momentum feedback on the ambient shear. They also play a central role in the two-way interactions between convection and the synoptic and planetary scale waves. They do so by serving as both the building block for organized convection, which involves congestus cloud decks that moisten and precondition the environment for deep convection which in turn is lagged by stratiform anvils, and as a conveyer belt for convective momentum transport (CMT). Here, we propose an extension of the multicloud model of Khouider and Majda (2006) to make the stratiform anvils more sensitive to the background wind shear profile. We do so by invoking two layers of moisture in the free troposphere instead of one, in addition to the boundary layer. Linear stability, in a wind shear background consisting of both mid-level and low-level easterly jets, representing, simultaneously, the Tropical Easterly and African Easterly jets, features the usual synoptic scale instability of the multicloud model plus two new instability bands at the meso-alpha and meso-beta scales, respectively. The meso-alpha and meso-beta modes constitute a paradigm for the dynamics of shear parallel convective systems with the meso-alpha waves being the quasi-stationary systems. In this talk we will present limited domain 3D simulations, without rotation, of realistic shear parallel lines of convection with parallel stratifrom anvils moving eastward, with a steering level in the upper troposphere, as a mesoscale envelope of the individual convective cells moving inwards, with a steering level in the lower troposphere. This provides, among other things, an excellent example of nontrivial CMT effect on the background low-level wind. It results in a narrow channel

  6. Fibrosis, adipogenesis, and muscle atrophy in congenital muscular torticollis.

    PubMed

    Chen, Huan-Xiong; Tang, Sheng-Ping; Gao, Fu-Tang; Xu, Jiang-Long; Jiang, Xian-Ping; Cao, Juan; Fu, Gui-Bing; Sun, Ke; Liu, Shi-Zhe; Shi, Wei

    2014-11-01

    In the traditional view, muscle atrophy and interstitial fibrosis were regarded as the basic pathological features of congenital muscular torticollis (CMT). But in the ultrastructure study, the mesenchyme-like cells, myoblasts, myofibroblasts, and fibroblasts were found in the proliferation of interstitium of CMT. To investigate the characteristics of pathological features and the mechanisms of muscle atrophy in CMT, we retrospectively reviewed the medical records of 185 CMT patients from July 2009 to July 2011 in Shenzhen Children's Hospital in China and performed pathological studies. According to age, the 185 CMT patients were divided into 4 groups. All resected surgical specimens were processed for hematoxylin and eosin staining and Masson trichromic staining. Sudan III staining was used for frozen sections, whereas immunohistochemical staining for S-100, calpain-1, ubiquitin, and 20S proteasome was carried out on 40 CMT specimens. Eight adductor muscle specimens from 8 patients with development dysplasia of the hip were taken as control group in the immunohistochemical staining. By Masson trichromic staining, the differences in the percent area of fibrous tissue in each CMT groups were significant. In Sudan III staining and immunostaining for S-100, adipocyte hyperplasia was the pathological feature of CMT. Moreover, compared with controls, most atrophic muscle fibers in CMT specimens were found to show strong immunoreactivity for calpain-1, ubiquitin, and 20S proteasome. With increasing age, fibrosis peaked at both sides and it was low in middle age group. Adipocytes increased with age. The characteristics of pathological features in CMT are changeable with age. The calpain and the ubiquitin-proteasome system may play a role in muscle atrophy of CMT. In the CMT, adipogenesis, fibrogenesis, and myogenesis may be the results of mesenchyme-like cells in SCM (sternocleidomastoid muscle). In conclusion, the present study furthermore supports maldevelopment of the

  7. Real time earthquake information and tsunami estimation system for Indonesia, Philippines and Central-South American regions

    NASA Astrophysics Data System (ADS)

    Pulido Hernandez, N. E.; Inazu, D.; Saito, T.; Senda, J.; Fukuyama, E.; Kumagai, H.

    2015-12-01

    Southeast Asia as well as Central-South American regions are within the most active seismic regions in the world. To contribute to the understanding of source process of earthquakes the National Research Institute for Earth Science and Disaster Prevention NIED maintains the international seismic Network (ISN) since 2007. Continuous seismic waveforms from 294 broadband seismic stations in Indonesia, Philippines, and Central-South America regions are received in real time at NIED, and used for automatic location of seismic events. Using these data we perform automatic and manual estimation of moment tensor of seismic events (Mw>4.5) by using the SWIFT program developed at NIED. We simulate the propagation of local tsunamis in these regions using a tsunami simulation code and visualization system developed at NIED, combined with CMT parameters estimated by SWIFT. The goals of the system are to provide a rapid and reliable earthquake and tsunami information in particular for large seismic, and produce an appropriate database of earthquake source parameters and tsunami simulations for research. The system uses the hypocenter location and magnitude of earthquakes automatically determined at NIED by the SeisComP3 system (GFZ) from the continuous seismic waveforms in the region, to perform the automated calculation of moment tensors by SWIFT, and then carry out the automatic simulation and visualization of tsunami. The system generates maps of maximum tsunami heights within the target regions and along the coasts and display them with the fault model parameters used for tsunami simulations. Tsunami calculations are performed for all events with available automatic SWIFT/CMT solutions. Tsunami calculations are re-computed using SWIFT manual solutions for events with Mw>5.5 and centroid depths shallower than 100 km. Revised maximum tsunami heights as well as animation of tsunami propagation are also calculated and displayed for the two double couple solutions by SWIFT

  8. The role of combined SNV and CNV burden in patients with distal symmetric polyneuropathy

    PubMed Central

    Pehlivan, Davut; Beck, Christine R.; Okamoto, Yuji; Harel, Tamar; Akdemir, Zeynep H.C.; Jhangiani, Shalini N.; Withers, Marjorie A.; Goksungur, Meryem Tuba; Carvalho, Claudia M.B.; Czesnik, Dirk; Gonzaga-Jauregui, Claudia; Wiszniewski, Wojciech; Muzny, Donna M.; Gibbs, Richard A.; Rautenstrauss, Bernd; Sereda, Michael W.; Lupski, James R.

    2017-01-01

    Purpose Charcot-Marie-Tooth (CMT) disease is a heterogeneous group of genetic disorders of the peripheral nervous system. Copy-number variants (CNVs) contribute significantly to CMT, as duplication of PMP22 underlies the majority of CMT1 cases. We hypothesized that CNVs and/or single-nucleotide variants (SNVs) might exist in patients with CMT with an unknown molecular genetic etiology. Methods Two hundred patients with CMT, negative for both SNV mutations in several CMT genes and for CNVs involving PMP22, were screened for CNVs by high-resolution oligonucleotide array comparative genomic hybridization. Whole-exome sequencing was conducted on individuals with rare, potentially pathogenic CNVs. Results Putatively causative CNVs were identified in five subjects (~2.5%); four of the five map to known neuropathy genes. Breakpoint sequencing revealed Alu-Alu-mediated junctions as a predominant contributor. Exome sequencing identified MFN2 SNVs in two of the individuals. Conclusion Neuropathy-associated CNV outside of the PMP22 locus is rare in CMT. Nevertheless, there is potential clinical utility in testing for CNVs and exome sequencing in CMT cases negative for the CMT1A duplication. These findings suggest that complex phenotypes including neuropathy can potentially be caused by a combination of SNVs and CNVs affecting more than one disease-associated locus and contributing to a mutational burden. PMID:26378787

  9. Boundary-Layer Transition on Large-Scale CMT Graphite Nosetips at Reentry Conditions

    DTIC Science & Technology

    1981-01-01

    a carbon arc (or tungsten ) reference source are recorded on identical film and are processed simultaneously with the model photograph, and (3...configuration is a reverse-taper plug that is swaged in place in the model forebody. Larger nosetips, which were required for expanding the range of...using either a carbon-arc light source or a tungsten source, depending on the desired calibration range--were simultaneously developed. These

  10. Microstructure and Properties of Lap Joint Between Aluminum Alloy and Galvanized Steel by CMT

    NASA Astrophysics Data System (ADS)

    Niu, Song; Chen, Su; Dong, Honggang; Zhao, Dongsheng; Zhang, Xiaosheng; Guo, Xin; Wang, Guoqiang

    2016-05-01

    Lap joining of 1-mm-thick Novelist AC 170 PX aluminum alloy to 1.2-mm-thick ST06 Z galvanized steel sheets for automotive applications was conducted by cold metal transfer advanced welding process with ER4043 and ER4047 filler wires. Under the optimized welding parameters with ER4043 filler wire, the tensile shear strength of joint was 189 MPa, reaching 89% of the aluminum alloy base metal. Microstructure and elemental distribution were characterized by optical metalloscope and electron probe microanalysis. The lap joints with ER4043 filler wire had smaller wetting angle and longer bonded line length with better wettability than with ER4047 filler wire during welding with same parameters. The needle-like Al-Fe-Si intermetallic compounds (IMCs) were spalled into the weld and brought negative effect to the tensile strength of joints. With increasing welding current, the needle-like IMCs grew longer and spread further into the weld, which would deteriorate the tensile shear strength.

  11. Cadmium mercury telluride infrared detectors

    NASA Astrophysics Data System (ADS)

    Elliott, C. T.

    Signal Processing In The Element (SPITE) detectors used in high performance thermal imaging systems are discussed. Developments to improve spatial and temperature resolution are outlined. Focal plane arrays of electronically scanned two-dimensional arrays of CMT detectors are treated. Use of photovoltaic CMT detectors hybridized with silicon addressing circuits is reported. Research to raise the operating temperature of infrared detectors is summarized.

  12. High-performance IR thermography system based on Class II Thermal Imaging Common Modules

    NASA Astrophysics Data System (ADS)

    Bell, Ian G.

    1991-03-01

    The Class II Thermal Imaging Common Modules were originally developed for the U.K. Ministry of Defence as the basis of a number of high performance thermal imaging systems for use by the British Armed Forces. These systems are characterized by high spatial resolution, high thermal resolution and real time thermal image update rate. A TICM II thermal imaging system uses a cryogenically cooled eight element Cadmium- Mercury-Telluride (CMT) SPRITE (Signal PRocessing In The Element) detector which is mechanically scanned over the thermal scene to be viewed. The TALYTHERM system is based on a modified TICM II thermal image connected to an IBM PC-AT compatible computer having image processing hardware installed and running the T.E.M.P.S. (Thermal Emission Measurement and Processing System) software package for image processing and data analysis. The operation of a TICM II thermal imager is briefly described highlighting the use of the SPRITE detector which coupled with a serial/parallel scanning technique yields high temporal, spatial and thermal resolutions. The conversion of this military thermal image into thermography system is described, including a discussion of the modifications required to a standard imager. The technique for extracting temperature information from a real time thermal image and how this is implemented in a TALYTHERM system is described. The D.A.R.T. (Discrete Attenuation of Radiance Thermography) system which is based on an extensively modified TICM II thermal imager is also described. This system is capable of measuring temperatures up to 1000 degrees C whilst maintaining the temporal and spatial resolutions inherent in a TICM II imager. Finally applications of the TALYTHERM in areas such as NDT (Non Destructive Testing), medical research and military research are briefly described.

  13. Mitofusin 2 expression dominates over mitofusin 1 exclusively in mouse dorsal root ganglia - a possible explanation for peripheral nervous system involvement in Charcot-Marie-Tooth 2A.

    PubMed

    Kawalec, Maria; Zabłocka, Barbara; Kabzińska, Dagmara; Neska, Jacek; Beręsewicz, Małgorzata

    2014-01-01

    Mitofusin 2 (Mfn2), a protein of the mitochondrial outer membrane, is essential for mitochondrial fusion and contributes to the maintenance and operation of the mitochondrial network. Mutations in the mitofusin 2 gene cause axonal Charcot-Marie-Tooth type 2A (CMT2A), an inherited disease affecting peripheral nerve axons. The precise mechanism by which mutations in MFN2 selectively cause the degeneration of long peripheral axons is not known. There is a hypothesis suggesting the involvement of reduced expression of a homologous protein, mitofusin 1 (Mfn1), in the peripheral nervous system, and less effective compensation of defective mitofusin 2 by mitofusin 1. We therefore aimed to perform an analysis of the mitofusin 1 and mitofusin 2 mRNA and protein expression profiles in different mouse tissues, with special attention paid to dorsal root ganglia (DRGs), as parts of the peripheral nervous system. Quantitative measurement relating to mRNA revealed that expression of the Mfn2 gene dominates over Mfn1 mainly in mouse DRG, as opposed to other nervous system samples and other tissues studied. This result was further supported by Western blot evaluation. Both these sets of data confirm the hypothesis that the cellular consequences of mutations in the mitofusin 2 gene can mostly be manifested in the peripheral nervous system.

  14. Therapeutic options in Charcot-Marie-Tooth diseases.

    PubMed

    Mathis, Stéphane; Magy, Laurent; Vallat, Jean-Michel

    2015-04-01

    Charcot-Marie-Tooth (CMT) diseases represent a heterogeneous genetic disorder (more than 80 genes are implicated in these inherited neuropathies), but sharing a similar phenotype. In recent years, advances in molecular genetics and molecular biology, and also the development of various animal models of CMT, have led to a better understanding. Taken together, this knowledge represents a prerequisite for the development of future therapies in CMT, and in peripheral nervous system disorders in general. The efficacy of various substances has been shown in vitro and also in vivo (in animal models); but, no significant positive effect has yet been confirmed in humans. However, some of these trials are still in development, and we may expect positive results in the future. Although CMT is still an incurable disease, symptomatic treatments (physiotherapy, surgery, analgesic, etc.) are crucial to improve the quality of life of CMT patients.

  15. Advanced Research Deposition System (ARDS) for processing CdTe solar cells

    NASA Astrophysics Data System (ADS)

    Barricklow, Keegan Corey

    CdTe solar cells have been commercialized at the Gigawatt/year level. The development of volume manufacturing processes for next generation CdTe photovoltaics (PV) with higher efficiencies requires research systems with flexibility, scalability, repeatability and automation. The Advanced Research Deposition Systems (ARDS) developed by the Materials Engineering Laboratory (MEL) provides such a platform for the investigation of materials and manufacturing processes necessary to produce the next generation of CdTe PV. Limited by previous research systems, the ARDS was developed to provide process and hardware flexibility, accommodating advanced processing techniques, and capable of producing device quality films. The ARDS is a unique, in-line process tool with nine processing stations. The system was designed, built and assembled at the Materials Engineering Laboratory. Final assembly, startup, characterization and process development are the focus of this research. Many technical challenges encountered during the startup of the ARDS were addressed in this research. In this study, several hardware modifications needed for the reliable operation of the ARDS were designed, constructed and successfully incorporated into the ARDS. The effect of process condition on film properties for each process step was quantified. Process development to achieve 12% efficient baseline solar cell required investigation of discrete processing steps, troubleshooting process variation, and developing performance correlations. Subsequent to this research, many advances have been demonstrated with the ARDS. The ARDS consistently produces devices of 12% +/-.5% by the process of record (POR). The champion cell produced to date utilizing the ARDS has an efficiency of 16.2% on low cost commercial sodalime glass and utilizes advanced films. The ARDS has enabled investigation of advanced concepts for processing CdTe devices including, Plasma Cleaning, Plasma Enhanced Closed Space Sublimation

  16. Synchrotron micro-scale study of trace metal transport and distribution in Spartina alterniflora root system in Yangtze River intertidal zone

    DOE PAGES

    Feng, Huan; Tappero, Ryan; Zhang, Weiguo; ...

    2015-07-26

    This study is focused on micro-scale measurement of metal (Ca, Cl, Fe, K, Mn, Cu, Pb, and Zn) distributions in Spartina alterniflora root system. The root samples were collected in the Yangtze River intertidal zone in July 2013. Synchrotron X-ray fluorescence (XRF), computed microtomography (CMT), and X-ray absorption near-edge structure (XANES) techniques, which provide micro-meter scale analytical resolution, were applied to this study. Although it was found that the metals of interest were distributed in both epidermis and vascular tissue with the varying concentrations, the results showed that Fe plaque was mainly distributed in the root epidermis. Other metals (e.g.,more » Cu, Mn, Pb, and Zn) were correlated with Fe in the epidermis possibly due to scavenge by Fe plaque. Relatively high metal concentrations were observed in the root hair tip. As a result, this micro-scale investigation provides insights of understanding the metal uptake and spatial distribution as well as the function of Fe plaque governing metal transport in the root system.« less

  17. Synchrotron micro-scale study of trace metal transport and distribution in Spartina alterniflora root system in Yangtze River intertidal zone

    SciTech Connect

    Feng, Huan; Tappero, Ryan; Zhang, Weiguo; Liu, Wenliang; Yu, Lizhong; Qian, Yu; Wang, Jun; Wang, Jia -Jun; Eng, Christopher; Liu, Chang -Jun; Jones, Keith W.

    2015-07-26

    This study is focused on micro-scale measurement of metal (Ca, Cl, Fe, K, Mn, Cu, Pb, and Zn) distributions in Spartina alterniflora root system. The root samples were collected in the Yangtze River intertidal zone in July 2013. Synchrotron X-ray fluorescence (XRF), computed microtomography (CMT), and X-ray absorption near-edge structure (XANES) techniques, which provide micro-meter scale analytical resolution, were applied to this study. Although it was found that the metals of interest were distributed in both epidermis and vascular tissue with the varying concentrations, the results showed that Fe plaque was mainly distributed in the root epidermis. Other metals (e.g., Cu, Mn, Pb, and Zn) were correlated with Fe in the epidermis possibly due to scavenge by Fe plaque. Relatively high metal concentrations were observed in the root hair tip. As a result, this micro-scale investigation provides insights of understanding the metal uptake and spatial distribution as well as the function of Fe plaque governing metal transport in the root system.

  18. Synchrotron micro-scale study of trace metal transport and distribution in Spartina alterniflora root system in Yangtze River intertidal zone.

    PubMed

    Feng, Huan; Zhang, Weiguo; Liu, Wenliang; Yu, Lizhong; Qian, Yu; Wang, Jun; Wang, Jia-Jun; Eng, Christopher; Liu, Chang-Jun; Jones, Keith W; Tappero, Ryan

    2015-12-01

    This study is focused on micro-scale measurement of metal (Ca, Cl, Fe, K, Mn, Cu, Pb, and Zn) distributions in Spartina alterniflora root system. The root samples were collected in the Yangtze River intertidal zone in July 2013. Synchrotron X-ray fluorescence (XRF), computed microtomography (CMT), and X-ray absorption near-edge structure (XANES) techniques, which provide micro-meter scale analytical resolution, were applied to this study. Although it was found that the metals of interest were distributed in both epidermis and vascular tissue with the varying concentrations, the results showed that Fe plaque was mainly distributed in the root epidermis. Other metals (e.g., Cu, Mn, Pb, and Zn) were correlated with Fe in the epidermis possibly due to scavenge by Fe plaque. Relatively high metal concentrations were observed in the root hair tip. This micro-scale investigation provides insights of understanding the metal uptake and spatial distribution as well as the function of Fe plaque governing metal transport in the root system.

  19. SPES-2, AP600 intergral system test S01007 2 inch CL to core make-up tank pressure balance line break

    SciTech Connect

    Bacchiani, M.; Medich, C.; Rigamonti, M.

    1995-09-01

    The SPES-2 is a full height, full pressure experimental test facility reproducing the Westinghouse AP600 reactor with a scaling factor of 1/395. The experimental plant, designed and operated by SIET in Piacenza, consists of a full simulation of the AP600 primary core cooling system including all the passive and active safety systems. In 1992, Westinghouse, in cooperation with ENEL (Ente Nazionale per l` Energia Elettrica), ENEA (Enter per le numove Technlogie, l` Energia e l` Ambient), Siet (Societa Informazioni Esperienze Termoidraulich) and ANSALDO developed an experimental program to test the integrated behaviour of the AP600 passive safety systems. The SPES-2 test matrix, concluded in November 1994, has examined the AP600 passive safety system response for a range of small break LOCAs at different locations on the primary system and on the passive system lines; single steam generator tube ruptures with passive and active safety systems and a main steam line break transient to demonstrate the boration capability of passive safety systems for rapid cooldown. Each of the tests has provided detailed experimental results for verification of the capability of the analysis methods to predict the integrated passive safety system behaviour. Cold and hot shakedown tests have been performed on the facility to check the characteristics of the plant before starting the experimental campaign. The paper first presents a description of the SPES-2 test facility then the main results of S01007 test {open_quotes}2{close_quotes} Cold Leg (CL) to Core Make-up Tank (CMT) pressure balance line break{close_quotes} are reported and compared with predictions performed using RELAP5/mod3/80 obtained by ANSALDO through agreement with U.S.N.R.C. (U.S. Nuclear Regulatory Commission). The SPES-2 nodalization and all the calculations here presented were performed by ANSALDO and sponsored by ENEL as a part of pre-test predictions for SPES-2.

  20. Westinghouse Small Modular Reactor nuclear steam supply system design

    SciTech Connect

    Memmott, M. J.; Harkness, A. W.; Van Wyk, J.

    2012-07-01

    generator, and eight reactor coolant pumps (RCP). The containment vessel is 27.1 m (89 ft) long and 9.8 m (32 ft) in diameter, and is designed to withstand pressures up to 1.7 MPa (250 psi). It is completely submerged in a pool of water serving as a heat sink and radiation shield. Housed within the containment are four combined core makeup tanks (CMT)/passive residual heat removal (PRHR) heat exchangers, two in-containment pools (ICP), two ICP tanks and four valves which function as the automatic depressurization system (ADS). The PRHR heat exchangers are thermally connected to two different ultimate heat sink (UHS) tanks which provide transient cooling capabilities. (authors)

  1. The shifting paradigm of Charcot-Marie-Tooth disease.

    PubMed

    Echaniz-Laguna, A

    2015-01-01

    Molecular studies have created a paradigm shift in our perception of Charcot-Marie-Tooth disease (CMT). Indeed, CMT has evolved from the concept of a rather homogeneous hereditary disease exclusively involving peripheral nerves to the concept of a highly heterogeneous clinical and genetic syndrome mainly - but sometimes not exclusively - involving the peripheral nervous system. The phenotypic spectrum of CMT overlaps with other inherited neuropathies such as distal hereditary motor neuropathy (dHMN), hereditary sensory and autonomic neuropathy (HSAN), spinal muscular atrophy (SMA) subtypes, and the neuropathies of mitochondrial disorders. At a molecular level, mutations in one given gene may alternatively provoke CMT, HSAN, dHMN or SMA variants. Over the last years, there have been dramatic advances in deciphering the molecular basis for many CMT subtypes and more than 900 different mutations in more than 60 causative genes are now described. However, as 75% of CMT causative genes apparently remain unknown and as disease-specific therapies are not available, major advances are yet to come in the field of CMT.

  2. Condensation during gravity driven ECC: Experiments with PACTEL

    SciTech Connect

    Munther, R.; Kalli, H.; Kouhia, J.

    1995-09-01

    This paper provides the results of the second series of gravity driven emergency core cooling (ECC) experiments with PACTEL (Parallel Channel Test Loop). The simulated accident was a small break loss-of-coolant accident (SBLOCA) with a break in a cold leg. The ECC flow was provided from a core makeup tank (CMT) located at a higher elevation than the main part of the primary system. The CMT was pressurized with pipings from the pressurizer and a cold leg. The tests indicated that steam condensation in the CMT can prevent ECC and lead to core uncovery.

  3. Application of Automated SEM-EDS Based Mineral Identification Systems to Problems in Metamorphic Petrology

    NASA Astrophysics Data System (ADS)

    Fairhurst, Robert; Barrow, Wendy; Rollinson, Gavyn

    2010-05-01

    Automated scanning electron microscopy-energy dispersive x-ray spectrometer (SEM-EDS) based mineral identification systems such as QEMSCAN have been in development for over 20 years, primarily as a tool to understand mineral liberation and element distribution in metal mining industry. This powerful technique is now being used in non mining applications such as metamorphic petrology where accurate mineral identification and metamorphic fabrics are key to deciphering the metamorphic history of samples. The QEMSCAN was developed by CSIRO for application in the mining industry where it is used to understand mineralogy, texture, mineral associations, the presence of gangue minerals and deleterious elements that may potentially interfere with mineral processing and planning, and the overall impact of mineralogy on grinding and flotation processes. It is capable of identifying most rock-forming minerals in milliseconds from their characteristic x-ray spectra. The collected x-ray spectra are compared to entries in a database containing the species identification profiles (SIPs) and are assigned a label accordingly. QEMSCAN is capable of searching large sample areas at high resolution resulting in the accurate and precise determination of all minerals present. Reports that were originally developed for the mining geologist can be equally useful to the petrologist, e.g. phase/mineral maps, modal mineral abundances and mineral association reports. Identification of key minerals is of great importance to determining the petrologic history of a sample. These key minerals may be few in number and present as small microinclusions (less than 100 μm) making them difficult to identify, if at all, with the petrographic microscope. Therefore, imaging by electron-microprobe or scanning electron microscope are the methods traditionally used. However, because of the small field of view available on these instruments at a magnification necessary to resolve micron sized relicts and

  4. Upper crust response to geodynamic processes beneath Isparta Angle, SW Turkey: Revealed by CMT solutions of earthquakes

    NASA Astrophysics Data System (ADS)

    Över, Semir; Özden, Süha; Kamacı, Züheyr; Yılmaz, Hüseyin; Ünlügenç, Ulvi Can; Pınar, Ali

    2016-09-01

    The Isparta Angle is an important area of SW Anatolia where extensions in all directions (N-S, NE-SW, NW-SE and E-W) meet. These extensions were determined by normal faulting structures as well as by shallow earthquakes. All extensions, except the E-W one, were attributed to the deviatoric stresses in relation to slab forces and/or extrusion of Anatolia. The moment tensor inversion of 40 shallow earthquakes which occurred in the inner part of the Isparta Angle give focal mechanisms mostly indicating normal faulting. Inversion of all focal mechanisms of the earthquakes obtained from the moment tensor inversion yields normal faulting characterized by an approximately E-W (N268°E) σ3 axis. The calculated stress ratio R is 0.6944 indicating a triaxial stress state. Commonly accepted geodynamic models for the eastern Mediterranean region do not include plate boundary forces acting in the east or west direction. Our hypothesis is that the cause of the E-W extension is the combined forces of Gravitational Potential Energy and the hot asthenosphere upwelling through a tear fault in the subducted African plate between the Hellenic and Cyprus arcs beneath the Isparta Angle.

  5. Curcumin derivatives promote Schwann cell differentiation and improve neuropathy in R98C CMT1B mice.

    PubMed

    Patzkó, Agnes; Bai, Yunhong; Saporta, Mario A; Katona, István; Wu, Xingyao; Vizzuso, Domenica; Feltri, M Laura; Wang, Suola; Dillon, Lisa M; Kamholz, John; Kirschner, Daniel; Sarkar, Fazlul H; Wrabetz, Lawrence; Shy, Michael E

    2012-12-01

    Charcot-Marie-Tooth disease type 1B is caused by mutations in myelin protein zero. R98C mice, an authentic model of early onset Charcot-Marie-Tooth disease type 1B, develop neuropathy in part because the misfolded mutant myelin protein zero is retained in the endoplasmic reticulum where it activates the unfolded protein response. Because oral curcumin, a component of the spice turmeric, has been shown to relieve endoplasmic reticulum stress and decrease the activation of the unfolded protein response, we treated R98C mutant mice with daily gastric lavage of curcumin or curcumin derivatives starting at 4 days of age and analysed them for clinical disability, electrophysiological parameters and peripheral nerve morphology. Heterozygous R98C mice treated with curcumin dissolved in sesame oil or phosphatidylcholine curcumin performed as well as wild-type littermates on a rotarod test and had increased numbers of large-diameter axons in their sciatic nerves. Treatment with the latter two compounds also increased compound muscle action potential amplitudes and the innervation of neuromuscular junctions in both heterozygous and homozygous R98C animals, but it did not improve nerve conduction velocity, myelin thickness, G-ratios or myelin period. The expression of c-Jun and suppressed cAMP-inducible POU (SCIP)-transcription factors that inhibit myelination when overexpressed-was also decreased by treatment. Consistent with its role in reducing endoplasmic reticulum stress, treatment with curcumin dissolved in sesame oil or phosphatidylcholine curcumin was associated with decreased X-box binding protein (XBP1) splicing. Taken together, these data demonstrate that treatment with curcumin dissolved in sesame oil or phosphatidylcholine curcumin improves the peripheral neuropathy of R98C mice by alleviating endoplasmic reticulum stress, by reducing the activation of unfolded protein response and by promoting Schwann cell differentiation.

  6. Diagnosis of Charcot-Marie-Tooth Disease

    PubMed Central

    Banchs, Isabel; Casasnovas, Carlos; Albertí, Antonia; De Jorge, Laura; Povedano, Mónica; Montero, Jordi; Martínez-Matos, Juan Antonio; Volpini, Victor

    2009-01-01

    Charcot-Marie-Tooth (CMT) disease or hereditary motor and sensory neuropathy (HMSN) is a genetically heterogeneous group of conditions that affect the peripheral nervous system. The disease is characterized by degeneration or abnormal development of peripheral nerves and exhibits a range of patterns of genetic transmission. In the majority of cases, CMT first appears in infancy, and its manifestations include clumsiness of gait, predominantly distal muscular atrophy of the limbs, and deformity of the feet in the form of foot drop. It can be classified according to the pattern of transmission (autosomal dominant, autosomal recessive, or X linked), according to electrophysiological findings (demyelinating or axonal), or according to the causative mutant gene. The classification of CMT is complex and undergoes constant revision as new genes and mutations are discovered. In this paper, we review the most efficient diagnostic algorithms for the molecular diagnosis of CMT, which are based on clinical and electrophysiological data. PMID:19826499

  7. Plasma, cerebrospinal fluid, and brain distribution of /sup 14/C-melatonin in rat: a biochemical and autoradiographic study

    SciTech Connect

    Vitte, P.A.; Harthe, C.; Lestage, P.; Claustrat, B.; Bobillier, P.

    1988-01-01

    The distribution of 14C-Melatonin (14C-MT) after systemic injection was studied in the plasma, cerebrospinal fluid (CSF), and brain of rats. Chromatographic analysis (thin-layer chromatography and high-performance liquid chromatography) indicated that the radioactivity from biological samples taken at various times following the injection of label was mainly associated with 14C-MT. Computer analysis of plasma 14C-MT kinetics showed a three-compartment system with half-lives of 0.21 +/- 0.05, 5.97 +/- 1.11, and 47.52 +/- 8.86 min. The volume of distribution and the clearance were 1,736 +/- 349 ml.kg-1 and 25.1 +/- 1.7 ml.min-1.kg-1 respectively. The entry of 14C-MT into the CSF was rapid and reached a maximum at 5 min. The decay followed a two-compartment system with half-lives of 16.5 +/- 2.9 and 47.3 +/- 8.6 min. The CSF/plasma concentration ratio was 0.38 at the steady state (30 min). At 2 min the level of 14C-MT in the brain was 3.8 higher than in the CSF. Representative autoradiograms revealed an heterogeneous localization of 14C-MT in the grey matter. The highest regional values, as evaluated by the permeability area product technique, were found in cortex, thalamic nuclei, medial geniculate nucleus, anterior pretectal area, paraventricular nucleus of the hypothalamus, choroid plexuses, and bulb-pons. Thirty minutes later 14C-MT was still detected in most of the brain regions analyzed. These results point to a low but rapid penetration of circulating MT into the brain and the CSF. The heterogeneous distribution and the partial retention of 14C-MT in the brain are compatible with the hypothesis of a central action of this hormone mediated via binding sites.

  8. Exploratory Analysis Of The 3D Cloud Resolving Model Simulations of TOGA COARE: Preliminary Results

    NASA Astrophysics Data System (ADS)

    Mendes, S.; Bretherton, C.

    2007-12-01

    Global climate model studies suggest that cumulus momentum transport (CMT) in tropical oceanic convective cloud systems plays a significant role in the tropical mean circulation and transient variability. CMT is difficult to measure directly and can depend on the detailed structure and organization of the convection. Yet there have been comparatively few evaluations of CMT parameterizations and the assumptions underlying them using 3D cloud resolving model (CRM) simulations. We have analyzed CMT in a four month 3D 64x64x64 gridpoint CRM simulation of TOGA COARE with 1 km horizontal resolution. An additional 256x256x64 large-domain simulation was performed for a 10 day subperiod with strong convection combined with substantial mean vertical zonal wind shear, conditions favorably for strong CMT. Both simulations were identically forced with prescribed vertical motion, horizontal temperature and moisture advection, and relaxation of the domain-mean wind profile to observations on a one-hour timescale. Both were initialized with small amplitude white noise, but spun up realistic convection in less than a day. The domain-mean CMT in the small and large domain simulations for the 10-day common simulation period was compared. The two simulations showed remarkably similar CMT profiles on daily-mean timescales, suggesting that mesoscale contributions to CMT of scales greater than 64 km were small. The skill of a downgradient mixing-length parameterization CMT = Mc*L*DU/Dz was also tested. Here , Mc is convective mass flux, dU/dz is mean vertical shear, and L is a mixing length for updraft zonal velocity perturbations associated with entrainment and horizontal pressure gradient accelerations. This was done by regressing CMT at each height was regressed against Mc*DU/Dz at the same height across all 3D model snapshots over the 10 days. The correlation coefficient describes the accuracy of this downgradient parameterization, and L was calculated as the regression slope. In the

  9. Mitochondrial dynamics and inherited peripheral nerve diseases.

    PubMed

    Pareyson, Davide; Saveri, Paola; Sagnelli, Anna; Piscosquito, Giuseppe

    2015-06-02

    Peripheral nerves have peculiar energetic requirements because of considerable length of axons and therefore correct mitochondria functioning and distribution along nerves is fundamental. Mitochondrial dynamics refers to the continuous change in size, shape, and position of mitochondria within cells. Abnormalities of mitochondrial dynamics produced by mutations in proteins involved in mitochondrial fusion (mitofusin-2, MFN2), fission (ganglioside-induced differentiation-associated protein-1, GDAP1), and mitochondrial axonal transport usually present with a Charcot-Marie-Tooth disease (CMT) phenotype. MFN2 mutations cause CMT type 2A by altering mitochondrial fusion and trafficking along the axonal microtubule system. CMT2A is an axonal autosomal dominant CMT type which in most cases is characterized by early onset and rather severe course. GDAP1 mutations also alter fission, fusion and transport of mitochondria and are associated either with recessive demyelinating (CMT4A) and axonal CMT (AR-CMT2K) and, less commonly, with dominant, milder, axonal CMT (CMT2K). OPA1 (Optic Atrophy-1) is involved in fusion of mitochondrial inner membrane, and its heterozygous mutations lead to early-onset and progressive dominant optic atrophy which may be complicated by other neurological symptoms including peripheral neuropathy. Mutations in several proteins fundamental for the axonal transport or forming the axonal cytoskeleton result in peripheral neuropathy, i.e., CMT, distal hereditary motor neuropathy (dHMN) or hereditary sensory and autonomic neuropathy (HSAN), as well as in hereditary spastic paraplegia. Indeed, mitochondrial transport involves directly or indirectly components of the kinesin superfamily (KIF5A, KIF1A, KIF1B), responsible of anterograde transport, and of the dynein complex and related proteins (DYNC1H1, dynactin, dynamin-2), implicated in retrograde flow. Microtubules, neurofilaments, and chaperones such as heat shock proteins (HSPs) also have a fundamental

  10. Metabolite profile of a mouse model of Charcot–Marie–Tooth type 2D neuropathy: implications for disease mechanisms and interventions

    PubMed Central

    Bais, Preeti; Beebe, Kirk; Morelli, Kathryn H.; Currie, Meagan E.; Norberg, Sara N.; Evsikov, Alexei V.; Miers, Kathy E.; Seburn, Kevin L.; Guergueltcheva, Velina; Kremensky, Ivo; Jordanova, Albena; Bult, Carol J.

    2016-01-01

    ABSTRACT Charcot–Marie–Tooth disease encompasses a genetically heterogeneous class of heritable polyneuropathies that result in axonal degeneration in the peripheral nervous system. Charcot–Marie–Tooth type 2D neuropathy (CMT2D) is caused by dominant mutations in glycyl tRNA synthetase (GARS). Mutations in the mouse Gars gene result in a genetically and phenotypically valid animal model of CMT2D. How mutations in GARS lead to peripheral neuropathy remains controversial. To identify putative disease mechanisms, we compared metabolites isolated from the spinal cord of Gars mutant mice and their littermate controls. A profile of altered metabolites that distinguish the affected and unaffected tissue was determined. Ascorbic acid was decreased fourfold in the spinal cord of CMT2D mice, but was not altered in serum. Carnitine and its derivatives were also significantly reduced in spinal cord tissue of mutant mice, whereas glycine was elevated. Dietary supplementation with acetyl-L-carnitine improved gross motor performance of CMT2D mice, but neither acetyl-L-carnitine nor glycine supplementation altered the parameters directly assessing neuropathy. Other metabolite changes suggestive of liver and kidney dysfunction in the CMT2D mice were validated using clinical blood chemistry. These effects were not secondary to the neuromuscular phenotype, as determined by comparison with another, genetically unrelated mouse strain with similar neuromuscular dysfunction. However, these changes do not seem to be causative or consistent metabolites of CMT2D, because they were not observed in a second mouse Gars allele or in serum samples from CMT2D patients. Therefore, the metabolite ‘fingerprint’ we have identified for CMT2D improves our understanding of cellular biochemical changes associated with GARS mutations, but identification of efficacious treatment strategies and elucidation of the disease mechanism will require additional studies. PMID:27288508

  11. Muscle spindle alterations precede onset of sensorimotor deficits in Charcot-Marie-Tooth type 2E.

    PubMed

    Villalón, E; Jones, M R; Sibigtroth, C; Zino, S J; Dale, J M; Landayan, D S; Shen, H; Cornelison, D D W; Garcia, M L

    2017-02-01

    Charcot-Marie-Tooth (CMT) is the most common inherited peripheral neuropathy, affecting approximately 2.8 million people. The CMT leads to distal neuropathy that is characterized by reduced motor nerve conduction velocity, ataxia, muscle atrophy and sensory loss. We generated a mouse model of CMT type 2E (CMT2E) expressing human neurofilament light E396K (hNF-L(E396K) ), which develops decreased motor nerve conduction velocity, ataxia and muscle atrophy by 4 months of age. Symptomatic hNF-L(E396K) mice developed phenotypes that were consistent with proprioceptive sensory defects as well as reduced sensitivity to mechanical stimulation, while thermal sensitivity and auditory brainstem responses were unaltered. Progression from presymptomatic to symptomatic included a 50% loss of large diameter sensory axons within the fifth lumbar dorsal root of hNF-L(E396K) mice. Owing to proprioceptive deficits and loss of large diameter sensory axons, we analyzed muscle spindle morphology in presymptomatic and symptomatic hNF-L(E396K) and hNF-L control mice. Muscle spindle cross-sectional area and volume were reduced in all hNF-L(E396K) mice analyzed, suggesting that alterations in muscle spindle morphology occurred prior to the onset of typical CMT pathology. These data suggested that CMT2E pathology initiated in the muscle spindles altering the proprioceptive sensory system. Early sensory pathology in CMT2E could provide a unifying hypothesis for the convergence of pathology observed in CMT.

  12. Assessing the environmental performance of construction materials testing using EMS: An Australian study.

    PubMed

    Dejkovski, Nick

    2016-10-01

    This paper reports the audit findings of the waste management practices at 30 construction materials testing (CMT) laboratories (constituting 4.6% of total accredited CMT laboratories at the time of the audit) that operate in four Australian jurisdictions and assesses the organisation's Environmental Management System (EMS) for indicators of progress towards sustainable development (SD). In Australia, waste indicators are 'priority indicators' of environmental performance yet the quality and availability of waste data is poor. National construction and demolition waste (CDW) data estimates are not fully disaggregated and the contribution of CMT waste (classified as CDW) to the national total CDW landfill burden is difficult to quantify. The environmental and human impacts of anthropogenic release of hazardous substances contained in CMT waste into the ecosphere can be measured by construing waste indicators from the EMS. An analytical framework for evaluating the EMS is developed to elucidate CMT waste indicators and assess these indicators against the principle of proportionality. Assessing against this principle allows for: objective evaluations of whether the environmental measures prescribed in the EMS are 'proportionate' to the 'desired' (subjective) level of protection chosen by decision-makers; and benchmarking CMT waste indicators against aspirational CDW targets set by each Australian jurisdiction included in the audit. Construed together, the EMS derived waste indicators and benchmark data provide a composite indicator of environmental performance and progress towards SD. The key audit findings indicate: CMT laboratories have a 'poor' environmental performance (and overall progress towards SD) when EMS waste data are converted into indicator scores and assessed against the principle of proportionality; CMT waste recycling targets are lower when benchmarked against jurisdictional CDW waste recovery targets; and no significant difference in the average

  13. Trk receptor signaling and sensory neuron fate are perturbed in human neuropathy caused by Gars mutations.

    PubMed

    Sleigh, James N; Dawes, John M; West, Steven J; Wei, Na; Spaulding, Emily L; Gómez-Martín, Adriana; Zhang, Qian; Burgess, Robert W; Cader, M Zameel; Talbot, Kevin; Yang, Xiang-Lei; Bennett, David L; Schiavo, Giampietro

    2017-03-28

    Charcot-Marie-Tooth disease type 2D (CMT2D) is a peripheral nerve disorder caused by dominant, toxic, gain-of-function mutations in the widely expressed, housekeeping gene, GARS The mechanisms underlying selective nerve pathology in CMT2D remain unresolved, as does the cause of the mild-to-moderate sensory involvement that distinguishes CMT2D from the allelic disorder distal spinal muscular atrophy type V. To elucidate the mechanism responsible for the underlying afferent nerve pathology, we examined the sensory nervous system of CMT2D mice. We show that the equilibrium between functional subtypes of sensory neuron in dorsal root ganglia is distorted by Gars mutations, leading to sensory defects in peripheral tissues and correlating with overall disease severity. CMT2D mice display changes in sensory behavior concordant with the afferent imbalance, which is present at birth and nonprogressive, indicating that sensory neuron identity is prenatally perturbed and that a critical developmental insult is key to the afferent pathology. Through in vitro experiments, mutant, but not wild-type, GlyRS was shown to aberrantly interact with the Trk receptors and cause misactivation of Trk signaling, which is essential for sensory neuron differentiation and development. Together, this work suggests that both neurodevelopmental and neurodegenerative mechanisms contribute to CMT2D pathogenesis, and thus has profound implications for the timing of future therapeutic treatments.

  14. Continuously moving table time-of-flight angiography of the peripheral veins.

    PubMed

    Huff, Sandra; Honal, Matthias; Baumann, Tobias; Hennig, Jürgen; Markl, Michael; Ludwig, Ute

    2010-05-01

    Time-of-flight (TOF) MR angiography allows for noninvasive vessel imaging. To overcome the limited volumetric coverage of standard TOF techniques, the aim of this study was to investigate the combination of TOF and continuously moving table (CMT) acquisitions for peripheral vein imaging based on image subtraction. Two acquisition strategies are presented: a simple two-step method based on 2-fold CMT acquisition and an advanced one-step method requiring only one continuous scan. Image quality of both CMT TOF techniques was evaluated by semiquantitative image grading and by signal-to-noise ratio and contrast-to-noise ratio analysis for veins of the upper and lower leg in 10 healthy volunteers. Results were compared to a standard stationary two-dimensional (2D) TOF multistation acquisition. Image grading revealed good image quality for both CMT TOF methods, thereby confirming the feasibility of axial 2D CMT TOF to assess the veins of the lower extremities during a single scan. Quantitative evaluation showed no significant difference in signal-to-noise ratio and contrast-to-noise ratio compared to the stationary experiment. Additional measurements in three patients with postthrombotic changes and varicosities demonstrated the clinical applicability of the presented methods. CMT TOF provides promising results and permits the detection of various pathologic changes of the venous system.

  15. Microdisk-waveguide system for the control of the polarization state of light

    NASA Astrophysics Data System (ADS)

    Akhavan, Hooman

    In optical fibers and photonic integrated circuits (PIC's), the signal polarization can undergo random rotations. Since strong confinement (SC) microphotonic devices are polarization sensitive, a chip-scale polarization rotator is beneficial for the realization of chip-scale integrated optics. Moreover, control over polarization states of photon(s) is necessary for realization of quantum information technology in optical domain. Optical microdisks vertically-coupled to a waveguide bus due to the precise control over coupling distance in fabrication and relative ease of dense integration have become increasingly important as a building block for photonic integrated circuits. Both passive and active devices have been demonstrated including channel dropping filters, tunable resonant filters and switches, intensity modulators, phase modulators, laser sources, and WDM demultiplexers. In this research it has been proposed and experimentally observed preferential polarization mode conversion and signal amplification due to the existence of non-zero, asymmetric, and geometry dependent cross-polarization coupling coefficients between non-orthogonal modes of the waveguide and the vertically-coupled active microdisk. A theory based on coupled mode theory (CMT) is developed to realize microdisk-waveguide system as a narrowband polarization rotator. An integrated structure consisting of a polarization splitter, a microdisk as a narrowband polarization rotator and a combiner is proposed to be applicable in fiber communication and integrated optics. A broadband polarization rotator on ridge waveguides was designed. However, it is concluded that microdisk application for a polarization rotator is helpful to reduce the device size at the cost of bandwidth. Tuning of the microdisk resonance is required when the operating wavelength is changed. By incorporation of the two properties of the active microdisk as a polarization rotator and tunable phase shifter, two-microdisk device is

  16. Solar system positioning system

    NASA Technical Reports Server (NTRS)

    Penanen, Konstantin I.; Chui, Talso

    2006-01-01

    Power-rich spacecraft envisioned in Prometheus initiative open up possibilities for long-range high-rate communication. A constellation of spacecraft on orbits several A.U. from the Sun, equipped with laser transponders and precise clocks can be configured to measure their mutual distances to within few cm. High on-board power can create substantial non-inertial contribution to the spacecraft trajectory. We propose to alleviate this contribution by employing secondary ranging to a passive daughter spacecraft. Such constellation can form the basis of it navigation system capable of providing position information anywhere in the soIar system with similar accuracy. Apart from obvious Solar System exploration implications, this system can provide robust reference for GPS and its successors.

  17. Optimizing and Evaluating an Integrated SPECT-CmT System Dedicated to Improved 3-D Breast Cancer Imaging

    DTIC Science & Technology

    2009-05-01

    of the cone beam is a nterior to the ch est wall, po sitive values mean the beam penetrates the chest wall. The phantom used in the dual modality...SPECT -CmT experiments can be viewed in the manuscripts in Appendix D. Each phantom consists of 4 thin walled balloon lesions (Harvard Apparatus Inc...post-processed to mimic limited angle data sets by removing proj ections in contiguous sectors from desired positions around the phantom

  18. Optimizing and Evaluating an Integrated SPECT-CmT System Dedicated to Improved 3-D Breast Cancer Imaging

    DTIC Science & Technology

    2010-05-01

    measured using radiochromic film, calibrated using thermoluminescent detectors and an ion chamber. The dose absorbed during a scan is initially measured...Optimize tradeoffs between absorbed dose and image quality for the CmT subsystem Task 1(a) Embed thermoluminescent dosimeters or available MOSFET... thermoluminescent detectors (TLDs) were used in the experiments but, after consultation with experts in the field of radiation dosimetry, it was decided

  19. Natural System Mimics: Development and Evaluation of Formation/Decomposition Methods for Methane Hydrate Hosted in Fine Sediments

    NASA Astrophysics Data System (ADS)

    Mahajan, D.; Eaton, M. W.; Kerkar, P.; Jones, K. W.

    2007-05-01

    Marine hydrates constitute a major fraction of the total global hydrate inventory that to date need further characterization. Pristine samples recovered from several marine hydrate sites share a common feature: they invariably show high variability with respect to methane saturation. A fundamental understanding of host sediment characteristics with and without filled hydrates is sought. Previous studies (Winters et al., 2004) show discrepancies between field and laboratory data that was attributed to factors such as different methods used to reform hydrates in the laboratory. Our studies focus on hydrates in fine-grained natural sediments and discerning the effect of methods used to synthesize them under subsurface-mimic conditions. Our integrated approach involves characterization of host sediments by Computed Microtomography (CMT) at a beamline of the National synchrotron Light Source (NSLS) to establish parameters such as porosity and tortuosity on a grain scale of sediments with and without hydrates. The CMT technique is complemented by a recently constructed unit, namely Flexible Integrated Study of Hydrates (FISH) in which one of the pressure vessels is of 200 mL volume and fitted with a 12" sight glass. The vessel mimics hydrate formation under near-surface marine conditions. We have evaluated a sediment sample from the Gulf of Mexico (GOM) recovered during the DOE-JIP cruise. After fractionation, it was classified as Very Fine Silt (< 10 mm) under the Udden-Wentworth particle-size classification scheme. The CMT data collection was followed by charging the sample to the FISH unit and hydrates were formed by the Static method in which gas was periodically charged to maintain the pressure. Pressure dependency was established by conducting three runs at 900, 1200, and 1500 psi for hydrate formation. Results for our CMT and the formation/decomposition kinetics will be discussed. Keywords: Host sediments, sediment-hydrate interaction, methane hydrate, FISH unit

  20. Source mechanism of the 2014 Aegean Sea earthquake

    NASA Astrophysics Data System (ADS)

    Nakano, Masaru

    2016-04-01

    Rapid determination of centroid moment tensor (CMT) of earthquakes, namely the source centroid location, focal mechanism, and magnitude is important for early disaster responses and issuing Tsunami warnings. In order to evaluate capability of Turkey seismic network for rapid determinations of CMT, I investigate the source mechanism of the 2014 Aegean Sea earthquake (Mw 6.9). Although this event occur out of Turkey seismic network, I obtained stable CMT solution. The CMT solution of this earthquake represents a strike-slip fault, consistent with the geometry of the North Anatolian Fault (NAF), and the source-time function indicates that this event comprised several distinct subevents. Each subevent is considered to have ruptured a different fault segment. This observation indicates the existence of a mechanical barrier, namely a NAF segment boundary, at the hypocenter. I also determined CMT solutions of background seismicity. CMT solutions of background seismicity beneath the Aegean Sea represent strike-slip or normal faulting along the NAF or its branch faults. The tensional axes of these events are oriented northeast-southwest, indicating a transtensional tectonic regime. Beneath the Sea of Marmara, the CMT solutions represent mostly strike-slip faulting, consistent with the motion of the NAF, but we identified a normal fault event with a tensional axis parallel to the strike of the NAF. This mechanism indicates that a pull-apart basin, marking a segment boundary of the NAF, is developing there. Because ruptures of a fault system and large earthquake magnitudes are strongly controlled by the fault system geometry and fault length, mapping fault segments along NAF can help to improve the accuracy of scenarios developed for future disastrous earthquakes in the Marmara region.

  1. Immune System

    MedlinePlus

    ... Loss Surgery? A Week of Healthy Breakfasts Shyness Immune System KidsHealth > For Teens > Immune System A A A ... could put us out of commission. What the Immune System Does The immune (pronounced: ih-MYOON) system, which ...

  2. Data Systems vs. Information Systems

    PubMed Central

    Amatayakul, Margret K.

    1982-01-01

    This paper examines the current status of “hospital information systems” with respect to the distinction between data systems and information systems. It is proposed that the systems currently existing are incomplete data dystems resulting in ineffective information systems.

  3. Effect of Cimetidine and Phenobarbital on metabolite kinetics of Omeprazole in rats.

    PubMed

    Park, Eun-Ja; Cho, Hea-Young; Lee, Yong-Bok

    2005-10-01

    Omeprazole (OMP) is a proton pump inhibitor used as an oral treatment for acid-related gastrointestinal disorders. In the liver, it is primarily metabolized by cytochrome P-450 (CYP450) isoenzymes such as CYP2C19 and CYP3A4. 5-Hyroxyomeprazole (5-OHOMP) and omeprazole sulfone (OMP-SFN) are the two major metabolites of OMP in human. Cimetidine (CMT) inhibits the breakdown of drugs metabolized by CYP450 and reduces the clearance of coadministered drug resulted from both the CMT binding to CYP450 and the decreased hepatic blood flow due to CMT. Phenobarbital (PB) induces drug metabolism in laboratory animals and human. PB induction mainly involves mammalian CYP forms in gene families 2B and 3A. PB has been widely used as a prototype inducer for biochemical investigations of drug metabolism and the enzymes catalyzing this metabolism, as well as for genetic, pharmacological, and toxicological investigations. In order to investigate the influence of CMT and PB on the metabolite kinetics of OMP, we intravenously administered OMP (30 mg/kg) to rats intraperitoneally pretreated with normal saline (5 mL/kg), CMT (100 mg/kg) or PB (75 mg/kg) once a day for four days, and compared the pharmacokinetic parameters of OMP. The systemic clearance (CLt) of OMP was significantly (p<0.05) decreased in CMT-pretreated rats and significantly (p<0.05) increased in PB-pretreated rats. These results indicate that CMT inhibits the OMP metabolism due to both decreased hepatic blood flow and inhibited enzyme activity of CYP2C19 and 3A4 and that PB increases the OMP metabolism due to stimulation of the liver blood flow and/or bile flow, due not to induction of the enzyme activity of CYP3A4.

  4. Prototype Earthquake Early Warning System for Areas of Highest Seismic Risk in the Western U.S.

    NASA Astrophysics Data System (ADS)

    Bock, Y.; Geng, J.; Goldberg, D.; Saunders, J. K.; Haase, J. S.; Squibb, M. B.; Melgar, D.; Crowell, B. W.; Clayton, R. W.; Yu, E.; Walls, C. P.; Mann, D.; Mencin, D.; Mattioli, G. S.

    2015-12-01

    We report on a prototype earthquake early warning system for the Western U.S. based on GNSS (GPS+GLONASS) observations, and where available collocated GNSS and accelerometer data (seismogeodesy). We estimate with latency of 2-3 seconds GNSS displacement waveforms from more than 120 stations, focusing on the southern segment of the San Andreas fault, the Hayward and Rodgers Creek faults and Cascadia. The displacements are estimated using precise point positioning with ambiguity resolution (PPP-AR), which provides for efficient processing of hundreds of "clients" within the region of interest with respect to a reference frame well outside the expected zone of deformation. The GNSS displacements are useful for alleviating magnitude saturation concerns, rapid earthquake magnitude estimation using peak ground displacements, CMT solutions and finite fault slip models. However, GNSS alone is insufficient for strict earthquake early warning (i.e., P wave detection). Therefore, we employ a self-contained seismogeodetic technique, where collocations of GNSS and accelerometer instruments are available, to estimate real-time displacement and velocity waveforms using PPP-AR with accelerometers (PPP-ARA). Using the velocity waveforms we can detect the P wave arrival for earthquakes of interest (>M 5.5), estimate a hypocenter, S wave propagation, and earthquake magnitude using Pd scaling relationships within seconds. Currently we are gearing up to receive observatory-grade accelerometer data from the CISN. We have deployed 25 inexpensive MEMS accelerometers at existing GNSS stations. The SIO Geodetic Modules that control the flow of the GNSS and accelerometer data are being upgraded with in situ PPP-ARA and P wave picking. In situ processing allows us to use the data at the highest sampling rate of the GNSS receiver (10 Hz or higher), in combination with the 100 Hz accelerometer data. Adding the GLONASS data allows for increased precision in the vertical, an important factor in P

  5. Operating Systems.

    ERIC Educational Resources Information Center

    Denning, Peter J.; Brown, Robert L.

    1984-01-01

    A computer operating system spans multiple layers of complexity, from commands entered at a keyboard to the details of electronic switching. In addition, the system is organized as a hierarchy of abstractions. Various parts of such a system and system dynamics (using the Unix operating system as an example) are described. (JN)

  6. Respiratory System

    MedlinePlus

    ... this page from the NHLBI on Twitter. The Respiratory System The respiratory system is made up of organs ... vessels, and the muscles that enable breathing. The Respiratory System Figure A shows the location of the respiratory ...

  7. Lymph system

    MedlinePlus

    Lymphatic system ... Dains JE, Flynn JA, Solomon BS, Stewart RW. Lymphatic system. In: Ball JW, Dains JE, Flynn JA, Solomon ... 2015:chap 9. Hall JE. The microcirculation and lymphatic system: capillary fluid exchange, interstitial fluid, and lymph flow. ...

  8. Digestive System

    MedlinePlus

    ... Old Feeding Your 1- to 2-Year-Old Digestive System KidsHealth > For Parents > Digestive System A A A ... the body can absorb and use. About the Digestive System Almost all animals have a tube-type digestive ...

  9. A Review of X-linked Charcot-Marie-Tooth Disease.

    PubMed

    Wang, Ying; Yin, Fei

    2016-05-01

    X-linked Charcot-Marie-Tooth disease (CMTX) is the second common genetic variant of CMT. CMTX type 1 causes 90% of CMTX. The most important clinical features of CMTX are similar with other types of CMT; however, a few patients get the central nervous system involved with or without white matter lesions; males are more severely and earlier affected than females. In this review, the authors focus on the origin and classification of CMTX, the central nervous system manifestations of CMTX1, the possible mechanism by which GJB1 mutations cause CMT1X, and the emerging therapeutic strategies for CMTX. Moreover, several cases are presented to illustrate the central nervous system manifestations.

  10. Mechanical Systems

    NASA Technical Reports Server (NTRS)

    Davis, Robert E.

    2002-01-01

    The presentation provides an overview of requirement and interpretation letters, mechanical systems safety interpretation letter, design and verification provisions, and mechanical systems verification plan.

  11. Systems Thinking (and Systems Doing).

    ERIC Educational Resources Information Center

    Brethower, Dale M.; Dams, Peter-Cornelius

    1999-01-01

    Introduces human performance technology (HPT) by answering the following questions related to: what systems does; practical issues and questions to which systems thinking is relevant; research questions and answers with respect to systems thinking; how HPT practitioners can do systems thinking; systems thinking tools; what is and is not known…

  12. Identification of Genetic Causes of Inherited Peripheral Neuropathies by Targeted Gene Panel Sequencing.

    PubMed

    Nam, Soo Hyun; Hong, Young Bin; Hyun, Young Se; Nam, Da Eun; Kwak, Geon; Hwang, Sun Hee; Choi, Byung-Ok; Chung, Ki Wha

    2016-05-31

    Inherited peripheral neuropathies (IPN), which are a group of clinically and genetically heterogeneous peripheral nerve disorders including Charcot-Marie-Tooth disease (CMT), exhibit progressive degeneration of muscles in the extremities and loss of sensory function. Over 70 genes have been reported as genetic causatives and the number is still growing. We prepared a targeted gene panel for IPN diagnosis based on next generation sequencing (NGS). The gene panel was designed to detect mutations in 73 genes reported to be genetic causes of IPN or related peripheral neuropathies, and to detect duplication of the chromosome 17p12 region, the major genetic cause of CMT1A. We applied the gene panel to 115 samples from 63 non-CMT1A families, and isolated 15 pathogenic or likely-pathogenic mutations in eight genes from 25 patients (17 families). Of them, eight mutations were unreported variants. Of particular interest, this study revealed several very rare mutations in the SPTLC2, DCTN1, and MARS genes. In addition, the effectiveness of the detection of CMT1A was confirmed by comparing five 17p12-nonduplicated controls and 15 CMT1A cases. In conclusion, we developed a gene panel for one step genetic diagnosis of IPN. It seems that its time- and cost-effectiveness are superior to previous tiered-genetic diagnosis algorithms, and it could be applied as a genetic diagnostic system for inherited peripheral neuropathies.

  13. Steroid-dependent sensorineural hearing loss in a patient with Charcot-Marie-Tooth disease showing auditory neuropathy.

    PubMed

    Maeda, Yukihide; Kataoka, Yuko; Sugaya, Akiko; Kariya, Shin; Kobayashi, Katsuhiro; Nishizaki, Kazunori

    2015-06-01

    Charcot-Marie-Tooth disease (CMT) is the most common form of hereditary sensorimotor neuropathy and sometimes involves disorders of the peripheral auditory system. We present a case of steroid-dependent auditory neuropathy associated with CMT, in which the patient experienced 3 episodes of acute exacerbation of hearing loss and successful rescue of hearing by prednisolone. An 8-year-old boy was referred to the otolaryngology department at the University Hospital. He had been diagnosed with CMT type 1 (demyelinating type) at the Child Neurology Department and was suffering from mild hearing loss due to auditory neuropathy. An audiological diagnosis of auditory neuropathy was confirmed by auditory brainstem response and distortion-product otoacoustic emissions. At 9 years and 0 months old, 9 years and 2 months old, and 10 years and 0 months old, he had experienced acute exacerbations of hearing loss, each of which was successfully rescued by intravenous or oral prednisolone within 2 weeks. Steroid-responsive cases of CMT have been reported, but this is the first case report of steroid-responsive sensorineural hearing loss in CMT. The present case may have implications for the mechanisms of action of glucocorticoids in the treatment of sensorineural hearing loss.

  14. Exacerbation of Charcot-Marie-Tooth type 2E neuropathy following traumatic nerve injury.

    PubMed

    Villalón, Eric; Dale, Jeffrey M; Jones, Maria; Shen, Hailian; Garcia, Michael L

    2015-11-19

    Charcot-Marie-Tooth disease (CMT) is the most commonly inherited peripheral neuropathy. CMT disease signs include distal limb neuropathy, abnormal gait, sensory defects, and deafness. We generated a novel line of CMT2E mice expressing hNF-L(E397K), which displayed muscle atrophy of the lower limbs without denervation, proximal reduction in large caliber axons, and decreased nerve conduction velocity. In this study, we challenged wild type, hNF-L and hNF-L(E397K) mice with crush injury to the sciatic nerve. We analyzed functional recovery by measuring toe spread and analyzed gait using the Catwalk system. hNF-L(E397K) mice demonstrated reduced recovery from nerve injury consistent with increased susceptibility to neuropathy observed in CMT patients. In addition, hNF-L(E397K) developed a permanent reduction in their ability to weight bear, increased mechanical allodynia, and premature gait shift in the injured limb, which led to increasingly disrupted interlimb coordination in hNF-L(E397K). Exacerbation of neuropathy after injury and identification of gait alterations in combination with previously described pathology suggests that hNF-L(E397K) mice recapitulate many of clinical signs associated with CMT2. Therefore, hNF-L(E397K) mice provide a model for determining the efficacy of novel therapies.

  15. Identification of Genetic Causes of Inherited Peripheral Neuropathies by Targeted Gene Panel Sequencing

    PubMed Central

    Nam, Soo Hyun; Hong, Young Bin; Hyun, Young Se; Nam, Da Eun; Kwak, Geon; Hwang, Sun Hee; Choi, Byung-Ok; Chung, Ki Wha

    2016-01-01

    Inherited peripheral neuropathies (IPN), which are a group of clinically and genetically heterogeneous peripheral nerve disorders including Charcot-Marie-Tooth disease (CMT), exhibit progressive degeneration of muscles in the extremities and loss of sensory function. Over 70 genes have been reported as genetic causatives and the number is still growing. We prepared a targeted gene panel for IPN diagnosis based on next generation sequencing (NGS). The gene panel was designed to detect mutations in 73 genes reported to be genetic causes of IPN or related peripheral neuropathies, and to detect duplication of the chromosome 17p12 region, the major genetic cause of CMT1A. We applied the gene panel to 115 samples from 63 non-CMT1A families, and isolated 15 pathogenic or likely-pathogenic mutations in eight genes from 25 patients (17 families). Of them, eight mutations were unreported variants. Of particular interest, this study revealed several very rare mutations in the SPTLC2, DCTN1, and MARS genes. In addition, the effectiveness of the detection of CMT1A was confirmed by comparing five 17p12-nonduplicated controls and 15 CMT1A cases. In conclusion, we developed a gene panel for one step genetic diagnosis of IPN. It seems that its time- and cost-effectiveness are superior to previous tiered-genetic diagnosis algorithms, and it could be applied as a genetic diagnostic system for inherited peripheral neuropathies. PMID:27025386

  16. Counterpoint: long-course chemoradiation is preferable in the neoadjuvant treatment of rectal cancer.

    PubMed

    Minsky, Bruce D

    2011-07-01

    There are 2 approaches to preoperative therapy. Short-course (25 Gy in 5 fractions) radiation and long-course (50.4 Gy in 28 fractions) radiation combined with chemotherapy (CMT). Although short-course radiation therapy is used in some European countries, it is not favored in all European countries or North America. Unlike long-course CMT, it cannot be safely combined with adequate doses of systemic concurrent chemotherapy, and, as currently designed, it does not increase sphincter preservation. Long-course CMT remains the preferred regimen for cT3 and/or node-positive disease. With parallel advances in staging, surgery, systemic therapy, and molecular markers, more selective approaches are being investigated.

  17. Fluid Management System (FMS) fluid systems overview

    NASA Technical Reports Server (NTRS)

    Baird, R. S.

    1990-01-01

    Viewgraphs on fluid management system (FMS) fluid systems overview are presented. Topics addressed include: fluid management system description including system requirements (integrated nitrogen system, integrated water system, and integrated waste gas system) and physical description; and fluid management system evolution.

  18. Systems thinking.

    PubMed

    Cabrera, Derek; Colosi, Laura; Lobdell, Claire

    2008-08-01

    Evaluation is one of many fields where "systems thinking" is popular and is said to hold great promise. However, there is disagreement about what constitutes systems thinking. Its meaning is ambiguous, and systems scholars have made diverse and divergent attempts to describe it. Alternative origins include: von Bertalanffy, Aristotle, Lao Tsu or multiple aperiodic "waves." Some scholars describe it as synonymous with systems sciences (i.e., nonlinear dynamics, complexity, chaos). Others view it as taxonomy-a laundry list of systems approaches. Within so much noise, it is often difficult for evaluators to find the systems thinking signal. Recent work in systems thinking describes it as an emergent property of four simple conceptual patterns (rules). For an evaluator to become a "systems thinker", he or she need not spend years learning many methods or nonlinear sciences. Instead, with some practice, one can learn to apply these four simple rules to existing evaluation knowledge with transformative results.

  19. Telemetry Systems

    NASA Technical Reports Server (NTRS)

    1996-01-01

    Goddard Space Flight Center developed satellite telemetry processing technology to meet NASA's sophisticated processing requirements. The Microelectronic Systems Branch, a 'company' within Goddard, provided NASA with the telemetry data systems from 1985 to 1994. TSI/Telsys, Inc. was then founded to commercialize the systems and began operations on October 1, 1995. The system aids the remote sensing industry by providing affordable and quick access to data collected from space.

  20. Systems Engineering

    NASA Technical Reports Server (NTRS)

    Pellerano, Fernando

    2015-01-01

    This short course provides information on what systems engineering is and how the systems engineer guides requirements, interfaces with the discipline leads, and resolves technical issues. There are many system-wide issues that either impact or are impacted by the thermal subsystem. This course will introduce these issues and illustrate them with real life examples.

  1. Congenital muscular torticollis in older children: treatment with Z-plasty technique.

    PubMed

    Ekici, Nur Yucel; Kizilay, Ahmet; Akarcay, Mustafa; Firat, Yezdan

    2014-09-01

    Congenital muscular torticollis (CMT) is a common congenital disorder of the musculoskeletal system in neonates and infants. The aim of this study was to evaluate the results of inferior Z-plasty in older children with CMT. They had mean age of 10 years (range, 5-14 years) and were followed up for 1 to 6 years. Postoperative protocol included a neck exercise program composed of active and passive movements in all cases and immobilization with a cervical collar in only 4 patients. This study concluded that surgical management of older children with CMT using Z-lengthening gives excellent clinical and functional results. The procedure is much more effective than other techniques and relatively complication-free and safe. Postoperative cervical collar and a well-planed physiotherapy protocol go a long way toward ensuring good to excellent results. Early diagnosis and treatment are necessary for good results.

  2. Analysis of the AP600 core makeup tank experiments using the NOTRUMP code

    SciTech Connect

    Cunningham, J.C.; Haberstroh, R.C.; Hochreiter, L.E.; Jaroszewicz, J.

    1995-12-31

    The AP600 design utilizes passive methods to perform core and containment cooling functions for a postulated loss of coolant. The core makeup tank (CMT) is an important feature of the AP600 passive safety system. The NOTRUMP code has been compared to the 300-series core makeup tank experiments. It has been observed that the code will capture the correct thermal-hydraulic behavior observed in the experiments. The correlations used for wall film condensation and convective heat transfer to the heated CMT liquid appear to be appropriate for these applications. The code will predict the rapid condensation and mixing thermal-hydraulic behavior observed in the 300-series tests. The NOTRUMP predictions can be noding-dependent since the condensation is extremely dependent on the amount of cold CMT liquid that mixes with the incoming steam flow.

  3. System Effectiveness

    SciTech Connect

    Powell, Danny H; Elwood Jr, Robert H

    2011-01-01

    An effective risk assessment system is needed to address the threat posed by an active or passive insider who, acting alone or in collusion, could attempt diversion or theft of nuclear material. It is critical that a nuclear facility conduct a thorough self-assessment of the material protection, control, and accountability (MPC&A) system to evaluate system effectiveness. Self-assessment involves vulnerability analysis and performance testing of the MPC&A system. The process should lead to confirmation that mitigating features of the system effectively minimize the threat, or it could lead to the conclusion that system improvements or upgrades are necessary to achieve acceptable protection against the threat. Analysis of the MPC&A system is necessary to understand the limits and vulnerabilities of the system to internal threats. Self-assessment helps the facility be prepared to respond to internal threats and reduce the risk of theft or diversion of nuclear material. MSET is a self-assessment or inspection tool utilizing probabilistic risk assessment (PRA) methodology to calculate the system effectiveness of a nuclear facility's MPC&A system. MSET analyzes the effectiveness of an MPC&A system based on defined performance metrics for MPC&A functions based on U.S. and international best practices and regulations. A facility's MC&A system can be evaluated at a point in time and reevaluated after upgrades are implemented or after other system changes occur. The total system or specific subareas within the system can be evaluated. Areas of potential performance improvement or system upgrade can be assessed to determine where the most beneficial and cost-effective improvements should be made. Analyses of risk importance factors show that sustainability is essential for optimal performance. The analyses reveal where performance degradation has the greatest detrimental impact on total system risk and where performance improvements have the greatest reduction in system risk

  4. Cryogenic Systems

    NASA Astrophysics Data System (ADS)

    Hosoyama, Kenji

    2002-02-01

    In this lecture we discuss the principle of method of cooling to a very low temperature, i.e. cryogenic. The "gas molecular model" will be introduced to explain the mechanism cooling by the expansion engine and the Joule-Thomson expansion valve. These two expansion processes are normally used in helium refrigeration systems to cool the process gas to cryogenic temperature. The reverse Carnot cycle will be discussed in detail as an ideal refrigeration cycle. First the fundamental process of liquefaction and refrigeration cycles will be discussed, and then the practical helium refrigeration system. The process flow of the system and the key components; -compressor, expander, and heat exchanger- will be discussed. As an example of an actual refrigeration system, we will use the cryogenic system for the KEKB superconducting RF cavity. We will also discuss the liquid helium distribution system, which is very important, especially for the cryogenic systems used in accelerator applications. 1 Principles of Cooling and Fundamental Cooling Cycle 2 Expansion engine, Joule-Thomson expansion, kinetic molecular theory, and enthalpy 3 Liquefaction Systems 4 Refrigeration Systems 5 Practical helium liquefier/refrigeration system 6 Cryogenic System for TRISTAN Superconducting RF Cavity

  5. Oral high dose ascorbic acid treatment for one year in young CMT1A patients: a randomised, double-blind, placebo-controlled phase II trial

    PubMed Central

    2009-01-01

    Background High dose oral ascorbic acid substantially improved myelination and locomotor function in a Charcot-Marie-Tooth type 1A mouse model. A phase II study was warranted to investigate whether high dose ascorbic acid also has such a substantial effect on myelination in Charcot-Marie-Tooth type 1A patients and whether this treatment is safe. Methods Patients below age 25 years were randomly assigned to receive placebo or ascorbic acid (one gram twice daily) in a double-blind fashion during one year. The primary outcome measure was the change over time in motor nerve conduction velocity of the median nerve. Secondary outcome measures included changes in minimal F response latencies, compound muscle action potential amplitude, muscle strength, sensory function, Charcot-Marie-Tooth neuropathy score, and disability. Results There were no significant differences between the six placebo-treated (median age 16 years, range 13 to 24) and the five ascorbic acid-treated (19, 14 to 24) patients in change in motor nerve conduction velocity of the median nerve (mean difference ascorbic acid as opposed to placebo treatment of 1.3 m/s, confidence interval -0.3 to 3.0 m/s, P = 0.11) or in change of any of the secondary outcome measures over time. One patient in the ascorbic acid group developed a skin rash, which led to discontinuation of the study medication. Conclusion Oral high dose ascorbic acid for one year did not improve myelination of the median nerve in young Charcot-Marie-Tooth type 1A patients. Treatment was relatively safe. Trial registration Current Controlled Trials ISRCTN56968278, ClinicalTrials.gov NCT00271635. PMID:19909499

  6. Imaging diagnosis--celiacomesenteric trunk and portal vein hypoplasia in a pit bull terrier.

    PubMed

    Ricciardi, Mario; Martino, Rosmara; Assad, Eyad Abu

    2014-01-01

    The computed tomography (CT) imaging findings of a celiacomesenteric trunk (CMT) in a 1-year-old dog with primary hypoplasia of the portal vein (PHPV) are described. Computed tomography angiography revealed acquired porto-systemic shunts secondary to portal hypertension and a common origin of the celiac and cranial mesenteric arteries. The imaging findings and the association of a CMT with other vascular diseases have never been reported in dogs. The recognition of this rare arterial anomaly should prompt to investigate possible concurrent vascular diseases and may influence the planning of abdominal surgeries.

  7. Geothermal systems

    NASA Technical Reports Server (NTRS)

    Mohl, C.

    1978-01-01

    Several tasks of JPL related to geothermal energy are discussed. The major task is the procurement and test and evaluation of a helical screw drive (wellhead unit). A general review of geothermal energy systems is given. The presentation focuses attention on geothermal reservoirs in California, with graphs and charts to support the discussion. Included are discussions on cost analysis, systems maintenance, and a comparison of geothermal and conventional heating and cooling systems.

  8. CALUTRON SYSTEM

    DOEpatents

    Lawrence, E.O.

    1958-08-12

    A calutron system capable of functioning with only a portion of the separation tanks in the system operating is described. The invention is a calutron system comprssing a closed series of alternated tanks and electromagnets having a mid-yoke connecting intermediate positions of the series. dividing the series into twv-o portions, and thereby providing a closed magnetic path through either of the portions.

  9. Systemic Darwinism

    PubMed Central

    Winther, Rasmus Grønfeldt

    2008-01-01

    Darwin's 19th century evolutionary theory of descent with modification through natural selection opened up a multidimensional and integrative conceptual space for biology. We explore three dimensions of this space: explanatory pattern, levels of selection, and degree of difference among units of the same type. Each dimension is defined by a respective pair of poles: law and narrative explanation, organismic and hierarchical selection, and variational and essentialist thinking. As a consequence of conceptual debates in the 20th century biological sciences, the poles of each pair came to be seen as mutually exclusive opposites. A significant amount of 21st century research focuses on systems (e.g., genomic, cellular, organismic, and ecological/global). Systemic Darwinism is emerging in this context. It follows a “compositional paradigm” according to which complex systems and their hierarchical networks of parts are the focus of biological investigation. Through the investigation of systems, Systemic Darwinism promises to reintegrate each dimension of Darwin's original logical space. Moreover, this ideally and potentially unified theory of biological ontology coordinates and integrates a plurality of mathematical biological theories (e.g., self-organization/structure, cladistics/history, and evolutionary genetics/function). Integrative Systemic Darwinism requires communal articulation from a plurality of perspectives. Although it is more general than these, it draws on previous advances in Systems Theory, Systems Biology, and Hierarchy Theory. Systemic Darwinism would greatly further bioengineering research and would provide a significantly deeper and more critical understanding of biological reality. PMID:18697926

  10. Systemic darwinism.

    PubMed

    Winther, Rasmus Grønfeldt

    2008-08-19

    Darwin's 19th century evolutionary theory of descent with modification through natural selection opened up a multidimensional and integrative conceptual space for biology. We explore three dimensions of this space: explanatory pattern, levels of selection, and degree of difference among units of the same type. Each dimension is defined by a respective pair of poles: law and narrative explanation, organismic and hierarchical selection, and variational and essentialist thinking. As a consequence of conceptual debates in the 20th century biological sciences, the poles of each pair came to be seen as mutually exclusive opposites. A significant amount of 21st century research focuses on systems (e.g., genomic, cellular, organismic, and ecological/global). Systemic Darwinism is emerging in this context. It follows a "compositional paradigm" according to which complex systems and their hierarchical networks of parts are the focus of biological investigation. Through the investigation of systems, Systemic Darwinism promises to reintegrate each dimension of Darwin's original logical space. Moreover, this ideally and potentially unified theory of biological ontology coordinates and integrates a plurality of mathematical biological theories (e.g., self-organization/structure, cladistics/history, and evolutionary genetics/function). Integrative Systemic Darwinism requires communal articulation from a plurality of perspectives. Although it is more general than these, it draws on previous advances in Systems Theory, Systems Biology, and Hierarchy Theory. Systemic Darwinism would greatly further bioengineering research and would provide a significantly deeper and more critical understanding of biological reality.

  11. Anticipatory systems as linguistic systems

    NASA Astrophysics Data System (ADS)

    Ekdahl, Bertil

    2000-05-01

    The idea of system is well established although not well defined. What makes up a system depends on the observer. Thinking in terms of systems is only a convenient way to conceptualize organizations, natural or artificial, that show coherent properties. Among all properties, which can be ascribed to systems, one property seems to be more outstanding than others, namely that of being anticipatory. In nature, anticipatory properties are found only in living organizations. In this way it can be said to separate non-living systems from living because there is no indication that any natural phenomenon occurring in systems where there is no indication of life is anticipatory. The characteristic of living systems is that they are exposed to the evolution contrary to causal systems that do not undergo changes due to the influence of the environment. Causal systems are related to the past in such a way that subsequent situations can be calculated from knowledge of past situations. In causal systems the past is the cause of the present and there is no reference to the future as a determining agent, contrary to anticipatory systems where expectations are the cause of the present action. Since anticipatory properties are characteristic of living systems, this property, as all other properties in living systems, is a result of the evolution and can be found in plants as well as in animals. Thus, it is not only tied to consciousness but is found at a more basic level, i.e., in the interplay between genotype and phenotype. Anticipation is part of the genetic language in such a way that appropriate actions, for events in the anticipatory systems environment, are inscribed in the genes. Anticipatory behavior, as a result of the interpretation of the genetic language, has been selected by the evolution. In this paper anticipatory systems are regarded as linguistic systems and I argue that as such anticipation cannot be fragmented but must be holistically studied. This has the

  12. Analysis of the preliminary results based on the first source solutions for the 29th September 2009 Samoan tsunami: hints for a tsunami early warning system strategy

    NASA Astrophysics Data System (ADS)

    Tinti, Stefano; Armigliato, Alberto; Tonini, Roberto

    2010-05-01

    The 29 September 2009 Samoan tsunami was triggered by a strong earthquake (Mw=8.1) that occurred at 17:48 UTC offshore south of Samoa Islands. This earthquake represents an example of the so-called "outer-rise" earthquakes, that occur in the subducting plate before it enters in the subduction zone and their fault mechanism is normal instead of thrust as expected inside the subduction zone. The areas most affected were the south coasts of Western and American Samoa, where maximum peak-to-peak height of about 3.5 meters and 1.5 meters were recorded by tide-gauge stations respectively at Pago-Pago (American Samoa) and at Apia (Western Samoa). Almost 200 persons were killed and run-up heights were measured in excess of 5 meters on several locations along the coast. This "anomalous" event is considered here "a posteriori" as a good case to test (and to open a discussion on) the today strategies used to forecast tsunami characteristics in the frame of Tsunami Early Warning Systems. In this work different fault models based on the focal mechanism solution proposed by Harvard CMT and USGS immediately after the 2009 Samoan earthquake are considered and tested by comparing some recorded signals (three offshore DART buoys and the two coastal tide gauges located at Apia and Pago-Pago) to the synthetic signals resulting from the numerical simulations provided by the UBO-TSUFD code, that is developed and maintained by the Tsunami Research Team of Bologna University. The analysis found out that all the considered sources lead to some discrepancies between observed and computed signals, though some of them reproduce some of the records quite well. These results suggest some important considerations on the tsunami forecast methods as well as on the difficulty and need of issuing timely and reliable warning in case of complex hazardous situation, which is a task that may require sophisticated decision-making platforms. This work has been conducted in the frame of the European

  13. Power system

    DOEpatents

    Hickam, Christopher Dale

    2008-03-18

    A power system includes a prime mover, a transmission, and a fluid coupler having a selectively engageable lockup clutch. The fluid coupler may be drivingly connected between the prime mover and the transmission. Additionally, the power system may include a motor/generator drivingly connected to at least one of the prime mover and the transmission. The power-system may also include power-system controls configured to execute a control method. The control method may include selecting one of a plurality of modes of operation of the power system. Additionally, the control method may include controlling the operating state of the lockup clutch dependent upon the mode of operation selected. The control method may also include controlling the operating state of the motor/generator dependent upon the mode of operation selected.

  14. Crystal structure of the extracellular domain of human myelin protein zero

    SciTech Connect

    Liu, Zhigang; Wang, Yong; Yedidi, Ravikiran S.; Brunzelle, Joseph S.; Kovari, Iulia A.; Sohi, Jasloveleen; Kamholz, John; Kovari, Ladislau C.

    2012-03-27

    Charcot-Marie-Tooth disease (CMT), a hereditary motor and sensory neuropathy, is the most common genetic neuropathy with an incidence of 1 in 2600. Several forms of CMT have been identified arising from different genomic abnormalities such as CMT1 including CMT1A, CMT1B, and CMTX. CMT1 with associated peripheral nervous system (PNS) demyelination, the most frequent diagnosis, demonstrates slowed nerve conduction velocities and segmental demyelination upon nerve biopsy. One of its subtypes, CMT1A, presents a 1.5-Mb duplication in the p11-p12 region of the human chromosome 17 which encodes peripheral myelin protein 22 (PMP22). CMT1B, a less common form, arises from the mutations in the myelin protein zero (MPZ) gene on chromosome 1, region q22-q23, which encodes the major structural component of the peripheral myelin. A rare type of CMT1 has been found recently and is caused by point mutations in early growth response gene 2 (EGR2), encoding a zinc finger transcription factor in Schwann cells. In addition, CMTX, an X-linked form of CMT, arises from a mutation in the connexin-32 gene. Myelin protein zero, associated with CMT1B, is a transmembrane protein of 219 amino acid residues. Human MPZ consists of three domains: 125 residues constitute the glycosylated immunoglobulin-like extracellular domain; 27 residues span the membrane; and 67 residues comprise the highly basic intracellular domain. MPZ makes up approximately 50% of the protein content of myelin, and is expressed predominantly in Schwann cells, the myelinating cell of the PNS. Myelin protein zero, a homophilic adhesion molecule, is a member of the immunoglobulin super-family and is essential for normal myelin structure and function. In addition, MPZ knockout mice displayed abnormal myelin that severely affects the myelination pathway, and overexpression of MPZ causes congenital hypomyelination of peripheral nerves. Myelin protein zero mutations account for {approx}5% of patients with CMT. To date, over 125

  15. Phacoemulsification systems.

    PubMed

    1989-11-01

    Our objectives in conducting this evaluation were to present an overview of a basic phacoemulsification system and its components, to describe the phacoemulsification procedure within the context of the operating principles of the system's components, and to compare two manufacturers' products. Specifications for additional phacoemulsification systems are available in the November 1989 edition of ECRI's Hospital Product Comparison System. Both of the evaluated systems enable a surgeon to perform a complete cataract extraction procedure by phacoemulsification. We rated both units Acceptable. In selecting a unit, users should consider performance, safety, human factors design, and manufacturer training and support. Although list prices vary widely among available systems, cost factors should not override clinical performance and safety requirements. While we measured certain engineering parameters, such as stroke length and ultrasound (US) output forces exerted on a medium, we stress that the results of these tests do not provide enough information to predict clinical performance. Clinical performance of phacoemulsification systems can be determined only by the experience of the clinicians who use them. Clinicians should review our evaluation thoroughly before making a purchasing decision. The information we present is useful for purchasing the evaluated or other available models because our criteria will guide users in assessing all components, and our findings and discussion on some aspects are common to many available systems (e.g., type of pump, irrigation and aspiration [I/A] characteristics). The in-depth clinical and technical information will help users to better understand principles, thereby helping them to better define their needs. Although we discovered a number of problems with the evaluated models, users should not assume that similar or other problems do not exist with systems that we did not evaluate. The willingness of manufacturers to cooperate in

  16. Electronic system

    DOEpatents

    Robison, G H; Dickson, J F

    1960-11-15

    An electronic system is designed for indicating the occurrence of a plurality of electrically detectable events within predetermined time intervals. The system comprises separate input means electrically associated with the events under observation an electronic channel associated with each input means, including control means and indicating means; timing means adapted to apply a signal from the input means after a predetermined time to the control means to deactivate each of the channels; and means for resetting the system to its initial condition after the observation of each group of events. (D.L.C.)

  17. Systems and Components Fuel Delivery System, Water Delivery System, ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    Systems and Components - Fuel Delivery System, Water Delivery System, Derrick Crane System, and Crane System Details - Marshall Space Flight Center, F-1 Engine Static Test Stand, On Route 565 between Huntsville and Decatur, Huntsville, Madison County, AL

  18. Systems Vaccinology

    PubMed Central

    Pulendran, Bali; Li, Shuzhao; Nakaya, Helder I

    2010-01-01

    Vaccination is one of the greatest triumphs of modern medicine, yet we remain largely ignorant of the mechanisms by which successful vaccines stimulate protective immunity. Two recent advances are beginning to illuminate such mechanisms: realization of the pivotal role of the innate immune system in sensing microbes and stimulating adaptive immunity, and advances in systems biology. Recent studies have used systems biology approaches to obtain a global picture of the immune responses to vaccination in humans. This has enabled the identification of early innate signatures that predict the immunogenicity of vaccines, and identification of potentially novel mechanisms of immune regulation. Here we review these advances, and critically examine the potential opportunities and challenges posed by systems biology in vaccine development. PMID:21029962

  19. Respiratory system

    NASA Technical Reports Server (NTRS)

    Bartlett, R. G., Jr.

    1973-01-01

    The general anatomy and function of the human respiratory system is summarized. Breathing movements, control of breathing, lung volumes and capacities, mechanical relations, and factors relevant to respiratory support and equipment design are discussed.

  20. Bioculture System

    NASA Technical Reports Server (NTRS)

    Figliozzi, Gianine; Sato, Kevin Y.; Sun. Sidney

    2013-01-01

    The document is a 2 page fact sheet that describes the Bioculture system, how it may be used by researchers for life science research, how and when it will be installed and validated aboard the international space station.

  1. Microelectromechanical Systems

    NASA Technical Reports Server (NTRS)

    Gabriel, Kaigham J.

    1995-01-01

    Micro-electromechanical systems (MEMS) is an enabling technology that merges computation and communication with sensing and actuation to change the way people and machines interact with the physical world. MEMS is a manufacturing technology that will impact widespread applications including: miniature inertial measurement measurement units for competent munitions and personal navigation; distributed unattended sensors; mass data storage devices; miniature analytical instruments; embedded pressure sensors; non-invasive biomedical sensors; fiber-optics components and networks; distributed aerodynamic control; and on-demand structural strength. The long term goal of ARPA's MEMS program is to merge information processing with sensing and actuation to realize new systems and strategies for both perceiving and controlling systems, processes, and the environment. The MEMS program has three major thrusts: advanced devices and processes, system design, and infrastructure.

  2. SAMPLING SYSTEM

    DOEpatents

    Hannaford, B.A.; Rosenberg, R.; Segaser, C.L.; Terry, C.L.

    1961-01-17

    An apparatus is given for the batch sampling of radioactive liquids such as slurries from a system by remote control, while providing shielding for protection of operating personnel from the harmful effects of radiation.

  3. Development and Optimization of a Dedicated, Hybrid Dual-Modality SPECT-CmT System for Improved Breast Lesion Diagnosis

    DTIC Science & Technology

    2010-01-01

    illustrate the s ystem movement. FIGURE 2: Photograph of bea m stop arra y with 2mm lead b eads that w as placed in front of the collimator ( ~15cm...the front of the collimator housing, which was approximately 15cm from the x-ray focal spot, during a CmT acquisit ion such that the amount of scatter...using a cubic spline to obtain a single scatter measurement near the boundaries at both ends of 5 Case: n < m Case: n = m Interpolate scatter

  4. Effectiveness of real-time quantitative PCR compare to repeat PCR for the diagnosis of Charcot-Marie-Tooth Type 1A and hereditary neuropathy with liability to pressure palsies.

    PubMed

    Choi, Jong Rak; Lee, Woon Hyoung; Sunwoo, Il Nam; Lee, Eun Kyung; Lee, Chang Hoon; Lim, Jong Baeck

    2005-06-30

    The majority of cases of Charcot-Marie-Tooth type 1A (CMT1A) and of hereditary neuropathy with a liability to pressure palsies (HNPP) are the result of heterozygosity for the duplication or deletion of peripheral myelin protein 22 gene (PMP22) on 17p11.2. Southern blots, pulsed-field gel electrophoresis (PFGE), fluorescence in situ hybridization (FISH) and polymorphic marker analysis are currently used diagnostic methods. But they are time-consuming, labor-intensive and have some significant limitations. We describe a rapid real- time quantitative PCR method for determining gene copy number for the identification of DNA duplication or deletion occurring in CMT1A or HNPP and compare the results obtained with REP-PCR. Six patients with CMT1A and 14 patients with HNPP [confirmed by Repeat (REP)-PCR], and 16 patients with suspicious CMT1A and 13 patients with suspicious HNPP [negative REP-PCR], and 15 normal controls were studied. We performed REP-PCR, which amplified a 3.6 Kb region (including a 1.7Kb recombination hotspot), using specific CMT1A-REP and real-time quantitative PCR on the LightCycler system. Using a comparative threshold cycle (Ct) method and beta -globin as a reference gene, the gene copy number of the PMP22 gene was quantified. The PMP22 duplication ratio ranged from 1.35 to 1.74, and the PMP22 deletion ratio from 0.41 to 0.53. The PMP22 ratio in normal controls ranged from 0.81 to 1.12. All 6 patients with CMT1A and 14 patients with HNPP confirmed by REP-PCR were positive by real-time quantitative PCR. Among the 16 suspicious CMT1A and 13 suspicious HNPP with negative REP-PCR, 2 and 4 samples, respectively, were positive by real-time quantitative PCR. Real-time quantitative PCR is a more sensitive and more accurate method than REP-PCR for the detection of PMP22 duplications or deletions, and it is also faster and easier than currently available methods. Therefore, we believe that the real-time quantitative method is useful for diagnosing CMT1A and

  5. Systems Studies

    SciTech Connect

    Graham, R.L.

    1998-03-17

    The Systems Studies Activity had two objectives: (1) to investigate nontechnical barriers to the deployment of biomass production and supply systems and (2) to enhance and extend existing systems models of bioenergy supply and use. For the first objective, the Activity focused on existing bioenergy markets. Four projects were undertaken: a comparative analysis of bioenergy in Sweden and Austria; a one-day workshop on nontechnical barriers jointly supported by the Production Systems Activity; the development and testing of a framework for analyzing barriers and drivers to bioenergy markets; and surveys of wood pellet users in Sweden, Austria and the US. For the second objective, two projects were undertaken. First, the Activity worked with the Integrated BioEnergy Systems (TBS) Activity of TEA Bioenergy Task XIII to enhance the BioEnergy Assessment Model (BEAM). This model is documented in the final report of the IBS Activity. The Systems Studies Activity contributed to enhancing the feedstock portion of the model by developing a coherent set of willow, poplar, and switchgrass production modules relevant to both the US and the UK. The Activity also developed a pretreatment module for switchgrass. Second, the Activity sponsored a three-day workshop on modeling bioenergy systems with the objectives of providing an overview of the types of models used to evaluate bioenergy and promoting communication among bioenergy modelers. There were nine guest speakers addressing different types of models used to evaluate different aspects of bioenergy, ranging from technoeconomic models based on the ASPEN software to linear programming models to develop feedstock supply curves for the US. The papers from this workshop have been submitted to Biomass and Bioenergy and are under editorial review.

  6. Systemic fluoride.

    PubMed

    Sampaio, Fábio Correia; Levy, Steven Marc

    2011-01-01

    There is substantial evidence that fluoride, through different applications and formulas, works to control caries development. The first observations of fluoride's effects on dental caries were linked to fluoride naturally present in the drinking water, and then from controlled water fluoridation programs. Other systemic methods to deliver fluoride were later suggested, including dietary fluoride supplements such as salt and milk. These systemic methods are now being questioned due to the fact that many studies have indicated that fluoride's action relies mainly on its post-eruptive effect from topical contact with the tooth structure. It is known that even the methods of delivering fluoride known as 'systemic' act mainly through a topical effect when they are in contact with the teeth. The effectiveness of water fluoridation in many geographic areas is lower than in previous eras due to the widespread use of other fluoride modalities. Nevertheless, this evidence should not be interpreted as an indication that systemic methods are no longer relevant ways to deliver fluoride on an individual basis or for collective health programs. Caution must be taken to avoid excess ingestion of fluoride when prescribing dietary fluoride supplements for children in order to minimize the risk of dental fluorosis, particularly if there are other relevant sources of fluoride intake - such as drinking water, salt or milk and/or dentifrice. Safe and effective doses of fluoride can be achieved when combining topical and systemic methods.

  7. Systemic trauma.

    PubMed

    Goldsmith, Rachel E; Martin, Christina Gamache; Smith, Carly Parnitzke

    2014-01-01

    Substantial theoretical, empirical, and clinical work examines trauma as it relates to individual victims and perpetrators. As trauma professionals, it is necessary to acknowledge facets of institutions, cultures, and communities that contribute to trauma and subsequent outcomes. Systemic trauma-contextual features of environments and institutions that give rise to trauma, maintain it, and impact posttraumatic responses-provides a framework for considering the full range of traumatic phenomena. The current issue of the Journal of Trauma & Dissociation is composed of articles that incorporate systemic approaches to trauma. This perspective extends conceptualizations of trauma to consider the influence of environments such as schools and universities, churches and other religious institutions, the military, workplace settings, hospitals, jails, and prisons; agencies and systems such as police, foster care, immigration, federal assistance, disaster management, and the media; conflicts involving war, torture, terrorism, and refugees; dynamics of racism, sexism, discrimination, bullying, and homophobia; and issues pertaining to conceptualizations, measurement, methodology, teaching, and intervention. Although it may be challenging to expand psychological and psychiatric paradigms of trauma, a systemic trauma perspective is necessary on both scientific and ethical grounds. Furthermore, a systemic trauma perspective reflects current approaches in the fields of global health, nursing, social work, and human rights. Empirical investigations and intervention science informed by this paradigm have the potential to advance scientific inquiry, lower the incidence of a broader range of traumatic experiences, and help to alleviate personal and societal suffering.

  8. Turbine system

    DOEpatents

    McMahan, Kevin Weston; Dillard, Daniel Jackson

    2016-05-03

    A turbine system is disclosed. The turbine system includes a transition duct having an inlet, an outlet, and a passage extending between the inlet and the outlet and defining a longitudinal axis, a radial axis, and a tangential axis. The outlet of the transition duct is offset from the inlet along the longitudinal axis and the tangential axis. The turbine system further includes a turbine section connected to the transition duct. The turbine section includes a plurality of shroud blocks at least partially defining a hot gas path, a plurality of buckets at least partially disposed in the hot gas path, and a plurality of nozzles at least partially disposed in the hot gas path. At least one of a shroud block, a bucket, or a nozzle includes means for withstanding high temperatures.

  9. Robotic System

    NASA Technical Reports Server (NTRS)

    1984-01-01

    A complicated design project, successfully carried out by New York manufacturing consultant with help from NERAC, Inc., resulted in new type robotic system being marketed for industrial use. Consultant Robert Price, operating at E.S.I, Inc. in Albany, NY, sought help from NERAC to develop an automated tool for deburring the inside of 8 inch breech ring assemblies for howitzers produced by Watervliet Arsenal. NERAC conducted a search of the NASA data base and six others. From information supplied, Price designed a system consisting of a standard industrial robot arm, with a specially engineered six-axis deburring tool fitted to it. A microcomputer and computer program direct the tool on its path through the breech ring. E.S.I. markets the system to aerospace and metal cutting industries for deburring, drilling, routing and refining machined parts.

  10. Charcot-Marie-Tooth and Related Diseases

    MedlinePlus

    ... and scientists, artists and singers, athletes and teachers, active teens and students. I know children with CMT ... allowed many people with the disease to lead active, productive lives. What causes CMT? CMT is caused ...

  11. Microbiology System

    NASA Technical Reports Server (NTRS)

    1992-01-01

    Technology originating in a NASA-sponsored study of the measurement of microbial growth in zero gravity led to the development of Biomerieux Vitek, Inc.'s VITEK system. VITEK provides a physician with accurate diagnostic information and identifies the most effective medication. Test cards are employed to identify organisms and determine susceptibility to antibiotics. A photo-optical scanner scans the card and monitors changes in the growth of cells contained within the card. There are two configurations - VITEK and VITEK JR as well as VIDAS, a companion system that detects bacteria, viruses, etc. from patient specimens. The company was originally created by McDonnell Douglas, the NASA contractor.

  12. ELECTRONIC SYSTEM

    DOEpatents

    Robison, G.H. et al.

    1960-11-15

    An electronic system is described for indicating the occurrence of a plurality of electrically detectable events within predetermined time intervals. It is comprised of separate input means electrically associated with the events under observation: an electronic channel associated with each input means including control means and indicating means; timing means associated with each of the input means and the control means and adapted to derive a signal from the input means and apply it after a predetermined time to the control means to effect deactivation of each of the channels; and means for resetting the system to its initial condition after observation of each group of events.

  13. Computer systems

    NASA Technical Reports Server (NTRS)

    Olsen, Lola

    1992-01-01

    In addition to the discussions, Ocean Climate Data Workshop hosts gave participants an opportunity to hear about, see, and test for themselves some of the latest computer tools now available for those studying climate change and the oceans. Six speakers described computer systems and their functions. The introductory talks were followed by demonstrations to small groups of participants and some opportunities for participants to get hands-on experience. After this familiarization period, attendees were invited to return during the course of the Workshop and have one-on-one discussions and further hands-on experience with these systems. Brief summaries or abstracts of introductory presentations are addressed.

  14. Complex Systems

    PubMed Central

    Goldberger, Ary L.

    2006-01-01

    Physiologic systems in health and disease display an extraordinary range of temporal behaviors and structural patterns that defy understanding based on linear constructs, reductionist strategies, and classical homeostasis. Application of concepts and computational tools derived from the contemporary study of complex systems, including nonlinear dynamics, fractals and “chaos theory,” is having an increasing impact on biology and medicine. This presentation provides a brief overview of an emerging area of biomedical research, including recent applications to cardiopulmonary medicine and chronic obstructive lung disease. PMID:16921107

  15. International Systems.

    ERIC Educational Resources Information Center

    Saba, Farhad, Ed.

    1999-01-01

    Completes a discussion of a systems model of distance education (in articles since May 1999) focusing on the most complex level, international. Discussion includes transfer of technology from United States universities to developing nations, the free market, and the age of the global economy. Presents a list of "early indicators" of changes in…

  16. Auditory system

    NASA Technical Reports Server (NTRS)

    Ades, H. W.

    1973-01-01

    The physical correlations of hearing, i.e. the acoustic stimuli, are reported. The auditory system, consisting of external ear, middle ear, inner ear, organ of Corti, basilar membrane, hair cells, inner hair cells, outer hair cells, innervation of hair cells, and transducer mechanisms, is discussed. Both conductive and sensorineural hearing losses are also examined.

  17. Instructional Systems.

    ERIC Educational Resources Information Center

    Banathy, Bela H.

    The underlying assumption of this book is that the systems approach has a dual role in education. "As applied 'in' education, it offers a powerful methodology for decisionmaking and design development. As applied 'to' education, it may bring about a clear understanding of what education is truly about." The essential purpose of the book is to…

  18. Immune System

    EPA Science Inventory

    A properly functioning immune system is essential to good health. It defends the body against infectious agents and in some cases tumor cells. Individuals with immune deficiencies resulting from genetic defects, diseases (e.g., AIDS, leukemia), or drug therapies are more suscepti...

  19. Systems Science

    ERIC Educational Resources Information Center

    Christakis, Alexander; Hammond, Debora; Jackson, Michael; Laszlo, Alexander; Mitroff, Ian; Snowden, Dave; Troncale, Len; Carr-Chellman, Alison; Spector, J. Michael; Wilson, Brent

    2013-01-01

    Scholars representing the field of systems science were asked to identify what they considered to be the most exciting and imaginative work currently being done in their field, as well as how that work might change our understanding. The scholars included Alexander Christakis, Debora Hammond, Michael Jackson, Alexander Laszlo, Ian Mitroff, Dave…

  20. Exploring Enterprise, System of Systems, and System and Software Architectures

    DTIC Science & Technology

    2016-06-13

    2009 Carnegie Mellon University Exploring Enterprise, System of Systems, and System and Software Architectures Software Engineering Institute...TITLE AND SUBTITLE Exploring Enterprise, System of Systems, and System and Software Architectures 5a. CONTRACT NUMBER 5b. GRANT NUMBER 5c...Carnegie Mellon University, Software Engineering Institute,Pittsburgh,PA,15213 8. PERFORMING ORGANIZATION REPORT NUMBER 9. SPONSORING/MONITORING AGENCY

  1. Surveying System

    NASA Technical Reports Server (NTRS)

    1988-01-01

    Sunrise Geodetic Surveys are setting up their equipment for a town survey. Their equipment differs from conventional surveying systems that employ transit rod and chain to measure angles and distances. They are using ISTAC Inc.'s Model 2002 positioning system, which offers fast accurate surveying with exceptional signals from orbiting satellites. The special utility of the ISTAC Model 2002 is that it can provide positioning of the highest accuracy from Navstar PPS signals because it requires no knowledge of secret codes. It operates by comparing the frequency and time phase of a Navstar signal arriving at one ISTAC receiver with the reception of the same set of signals by another receiver. Data is computer processed and translated into three dimensional position data - latitude, longitude and elevation.

  2. Solar Systems

    NASA Technical Reports Server (NTRS)

    1979-01-01

    The solar collectors shown are elements of domestic solar hot water systems produced by Solar One Ltd., Virginia Beach, Virginia. Design of these systems benefited from technical expertise provided Solar One by NASA's Langley Research Center. The company obtained a NASA technical support package describing the d e sign and operation of solar heating equipment in NASA's Tech House, a demonstration project in which aerospace and commercial building technology are combined in an energy- efficient home. Solar One received further assistance through personal contact with Langley solar experts. The company reports that the technical information provided by NASA influenced Solar One's panel design, its selection of a long-life panel coating which increases solar collection efficiency, and the method adopted for protecting solar collectors from freezing conditions.

  3. Gasification system

    DOEpatents

    Haldipur, Gaurang B.; Anderson, Richard G.; Cherish, Peter

    1983-01-01

    A method and system for injecting coal and process fluids into a fluidized bed gasification reactor. Three concentric tubes extend vertically upward into the fluidized bed. Coal particulates in a transport gas are injected through an inner tube, and an oxygen rich mixture of oxygen and steam are injected through an inner annulus about the inner tube. A gaseous medium relatively lean in oxygen content, such as steam, is injected through an annulus surrounding the inner annulus.

  4. Gasification system

    DOEpatents

    Haldipur, Gaurang B.; Anderson, Richard G.; Cherish, Peter

    1985-01-01

    A method and system for injecting coal and process fluids into a fluidized bed gasification reactor. Three concentric tubes extend vertically upward into the fluidized bed. Coal particulates in a transport gas are injected through an inner tube, and an oxygen rich mixture of oxygen and steam are injected through an inner annulus about the inner tube. A gaseous medium relatively lean in oxygen content, such as steam, is injected through an annulus surrounding the inner annulus.

  5. Imaging System

    NASA Technical Reports Server (NTRS)

    1995-01-01

    The 1100C Virtual Window is based on technology developed under NASA Small Business Innovation (SBIR) contracts to Ames Research Center. For example, under one contract Dimension Technologies, Inc. developed a large autostereoscopic display for scientific visualization applications. The Virtual Window employs an innovative illumination system to deliver the depth and color of true 3D imaging. Its applications include surgery and Magnetic Resonance Imaging scans, viewing for teleoperated robots, training, and in aviation cockpit displays.

  6. Security system

    DOEpatents

    Baumann, Mark J.; Kuca, Michal; Aragon, Mona L.

    2016-02-02

    A security system includes a structure having a structural surface. The structure is sized to contain an asset therein and configured to provide a forceful breaching delay. The structure has an opening formed therein to permit predetermined access to the asset contained within the structure. The structure includes intrusion detection features within or associated with the structure that are activated in response to at least a partial breach of the structure.

  7. Tychonic System

    NASA Astrophysics Data System (ADS)

    Murdin, P.

    2000-11-01

    The world system proposed in 1583 by the Danish astronomer Tycho Brahe (1546-1601). Unable to accept the Copernican doctrine that the Earth moves around the Sun, he put forward the view, later disproved by Kepler (1571-1630), that the planets move around the Sun, but the Sun and Moon move around the Earth. The theory explained the observed variations of the phases of Venus, for which the Ptolemai...

  8. Systemic Assurance

    DTIC Science & Technology

    2015-07-31

    to, warranty of fitness for purpose or merchantability, exclusivity, or results obtained from use of the material. Stevens Institute of Technology...customer organizations judge them fit for use in the mission environment. These assurance judgments must be based on evidence that a system manifests...which it is achieved. Assurance is a human judgment of fitness for use. This judgment is thus contextualized by the mission definition, the features

  9. Copernican System

    NASA Astrophysics Data System (ADS)

    Murdin, P.

    2000-11-01

    The heliocentric (i.e. `Sun-centered') theory proposed by the Polish astronomer Nicolaus Copernicus (1473-1543), and published by him in 1543 in his book, De Revolutionibus Orbium Coelestium. In this system Copernicus placed the Sun at the center of the universe and regarded the Earth and the planets as moving around it in circular orbits. Because of his retention of the notion of circular motion...

  10. CONTROL SYSTEM

    DOEpatents

    Shannon, R.H.; Williamson, H.E.

    1962-10-30

    A boiling water type nuclear reactor power system having improved means of control is described. These means include provisions for either heating the coolant-moderator prior to entry into the reactor or shunting the coolantmoderator around the heating means in response to the demand from the heat engine. These provisions are in addition to means for withdrawing the control rods from the reactor. (AEC)

  11. Bioregenerative system

    NASA Technical Reports Server (NTRS)

    1987-01-01

    The design course is an eight semester credit multi-disciplinary engineering design course taught primarily to Engineering Science, Aerospace, Electrical and Mechanical Engineering seniors. This year the course project involved the design of the three interrelated loops: atmospheric, liquid nutrient and solid waste management, associated with growing higher plants to support man during long-term space missions. The project is complementary to the NASA Kennedy Space Center Controlled Environmental Life Support System (CELSS) project. The first semester the class worked on a preliminary design for a complete system. This effort included means for monitoring and control of composition, temperature, flow rate, etc., for the atmosphere and liquid nutrient solution; disease and contaminant monitoring and control; plant mechanical support, propagation and harvesting; solid and liquid waste recycling; and system maintenance and refurbishing. The project has significant biological, mechanical, electrical and Al/Robotics aspects. The second semester a small number of subsystems or components, identified as important and interesting during the first semester, were selected for detail design, fabrication, and testing. The class was supported by close cooperation with The Kennedy Space Center and by two teaching assistants. The availability of a dedicated, well equipped project room greatly enhanced the communication and team spirit of the class.

  12. Braking system

    DOEpatents

    Norgren, D.U.

    1982-09-23

    A balanced braking system comprising a plurality of braking assemblies located about a member to be braked. Each of the braking assemblies consists of a spring biased piston of a first material fitted into a body of a different material which has a greater contraction upon cooling than the piston material. The piston is provided with a recessed head portion over which is positioned a diaphragm and forming a space therebetween to which is connected a pressurized fluid supply. The diaphragm is controlled by the fluid in the space to contact or withdraw from the member to be braked. A cooling means causes the body within which the piston is fitted to contract more than the piston, producing a tight shrink fit therebetween. The braking system is particularly applicable for selectively braking an arbor of an electron microscope which immobilizes, for example, a vertically adjustable low temperature specimen holder during observation. The system provides balanced braking forces which can be easily removed and re-established with minimal disturbance to arbor location.

  13. Balance System

    NASA Technical Reports Server (NTRS)

    1988-01-01

    TherEx Inc.'s AT-1 Computerized Ataxiameter precisely evaluates posture and balance disturbances that commonly accompany neurological and musculoskeletal disorders. Complete system includes two-strain gauged footplates, signal conditioning circuitry, a computer monitor, printer and a stand-alone tiltable balance platform. AT-1 serves as assessment tool, treatment monitor, and rehabilitation training device. It allows clinician to document quantitatively the outcome of treatment and analyze data over time to develop outcome standards for several classifications of patients. It can evaluate specifically the effects of surgery, drug treatment, physical therapy or prosthetic devices.

  14. Sterilization System

    NASA Technical Reports Server (NTRS)

    1990-01-01

    Cox Sterile Products, Inc.'s Rapid Heat Transfer Sterilizer employs a heat exchange process that induces rapid air movement; the air becomes the heat transfer medium, maintaining a uniform temperature of 375 degrees Fahrenheit. It features pushbutton controls for three timing cycles for different instrument loads, a six-minute cycle for standard unpackaged instruments, eight minutes for certain specialized dental/medical instruments and 12 minutes for packaged instruments which can then be stored in a drawer in sterile condition. System will stay at 375 degrees all day. Continuous operation is not expensive because of the sterilizer's very low power requirements.

  15. Purification system

    NASA Technical Reports Server (NTRS)

    Flanagan, David T. (Inventor); Gibbons, Randall E. (Inventor)

    1992-01-01

    A system for prolonging the life of a granulated activated charcoal (GAC) water treatment device is disclosed in which an ultraviolet light transparent material is used to constrain water to flow over carbon surfaces. It is configured to receive maximum flux from a UV radiation source for the purpose of preventing microbial proliferation on the carbon surfaces; oxidizing organic contaminants adsorbed from the water onto the carbon surfaces and from biodegradation of adsorbed microbial forms; disinfecting water; and oxidizing organic contaminants in the water.

  16. Relaxation System

    NASA Technical Reports Server (NTRS)

    1987-01-01

    Environ Corporation's relaxation system is built around a body lounge, a kind of super easy chair that incorporates sensory devices. Computer controlled enclosure provides filtered ionized air to create a feeling of invigoration, enhanced by mood changing aromas. Occupant is also surrounded by multidimensional audio and the lighting is programmed to change colors, patterns, and intensity periodically. These and other sensory stimulators are designed to provide an environment in which the learning process is stimulated, because research has proven that while an individual is in a deep state of relaxation, the mind is more receptive to new information.

  17. Systems Pharmacology

    PubMed Central

    Boran, Aislyn D. W.; Iyengar, Ravi

    2011-01-01

    We examine how physiology and pathophysiology are studied from a systems perspective, using high-throughput experiments and computational analysis of regulatory networks. We describe the integration of these analyses with pharmacology, which leads to new understanding of drug action and enables drug discovery for complex diseases. Network studies of drug-target relationships can serve as an indication on the general trends in the approved drugs and the drug-discovery progress. There is a growing number of targeted therapies approved and in the pipeline, which meets a new set of problems with efficacy and adverse effects. The pitfalls of these mechanistically based drugs are described, along with how a systems view of drug action is increasingly important to uncover intricate signaling mechanisms that play an important part in drug action, resistance mechanisms, and off-target effects. Computational methodologies enable the classification of drugs according to their structures and to which proteins they bind. Recent studies have combined the structural analyses with analysis of regulatory networks to make predictions about the therapeutic effects of drugs for complex diseases and possible off-target effects. PMID:20687178

  18. Transfer system

    DOEpatents

    Kurosawa, Kanji; Koga, Bunichiro; Ito, Hideki; Kiriyama, Shigeru; Higuchi, Shizuo

    2003-05-20

    A transport system includes a traveling rail (1) which constitutes a transport route and a transport body (3) which is capable of traveling on the traveling rail in the longitudinal direction of the traveling rail. Flexible drive tubes (5) are arranged on the traveling rail in the longitudinal direction of the traveling rail. The transport body includes a traveling wheel (4) which is capable of rolling on the traveling rail and drive wheels (2) which are capable of rolling on the drive tubes upon receiving the rotational drive power generated by pressure of a pressure medium supplied to the drive tubes while depressing the drive tubes. The traveling rail includes a plurality of transport sections and the transport body is capable of receiving a rotational drive force from the drive tubes at every transport sections. If necessary, a transport route changeover switch which changes over the transport route can be provided between the transport sections.

  19. Power systems

    NASA Astrophysics Data System (ADS)

    Kaplan, G.

    1982-01-01

    Significant events in current, prototype, and experimental utility power generating systems in 1981 are reviewed. The acceleration of licensing and the renewal of plans for reprocessing of fuel for nuclear power plants are discussed, including the rise of French reactor-produced electricity to over 40% of the country's electrical output. A 4.5 MW fuel cell neared completion in New York City, while three 2.5 MW NASA-designed windpowered generators began producing power in the state of Washington. Static bar compensators, nonflammable-liquid cooled power transformers, and ZnO surge arrestors were used by utilities for the first time, and the integration of a coal gasifier-combined cycle power plant approached the planning phase. An MHD generator was run for 1000 hours and produced 50-60 kWe, while a 20 MVA superconducting generator was readied for testing.

  20. Pumping system

    SciTech Connect

    Kime, J.A.

    1987-05-19

    This patent describes a gas-oil production system for pumping formation fluid in a well through a tubing string within which a down hole pump connects to a hydraulic stroking device through a rod string providing the pump including a plunger reciprocally driven by the hydraulic stroking device toward an upper terminal position during a plunger upstroke. The rod string normally supports the weight of a column of fluid and toward a lower terminal position at the end of a plunger downstroke during which the weight of the column fluid is normally transferred to the tubing string through fluid within the pump. The method for detecting when the well is pumped off comprises: supplying working fluid to the hydraulic stroking device to raise the hydraulic stroking device and thereby move the plunger from the lower terminal position to the upper terminal position; and removing the working fluid at a controlled rate from the hydraulic stroking device.

  1. Systems toxicology.

    PubMed

    Hartung, Thomas; van Vliet, Erwin; Jaworska, Joanna; Bonilla, Leo; Skinner, Nigel; Thomas, Russell

    2012-01-01

    The need for a more mechanistic understanding of the ways in which chemicals modulate biological pathways is urgent if we are to identify and better assess safety issues relating to a wide range of substances developed by the pharmaceutical, chemical, agri-bio, and cosmetic industries. Omics technologies provide a valuable opportunity to refine existing methods and provide information for so-called integrated testing strategies via the creation of signatures of toxicity. By mapping these signatures to underlying pathways of toxicity, some of which have been identified by toxicologists over the last few decades, and bringing them together with pathway information determined from biochemistry and molecular biology, a "systems toxicology" approach will enable virtual experiments to be conducted that can improve the prediction of hazard and the assessment of compound toxicity.

  2. DGAT2 Mutation in a Family with Autosomal-Dominant Early-Onset Axonal Charcot-Marie-Tooth Disease.

    PubMed

    Hong, Young Bin; Kang, Junghee; Kim, Ji Hyun; Lee, Jinho; Kwak, Geon; Hyun, Young Se; Nam, Soo Hyun; Hong, Hyun Dae; Choi, Yu-Ri; Jung, Sung-Chul; Koo, Heasoo; Lee, Ji Eun; Choi, Byung-Ok; Chung, Ki Wha

    2016-05-01

    Charcot-Marie-Tooth disease (CMT) is the most common inherited peripheral neuropathy and is a genetically and clinically heterogeneous disorder. We examined a Korean family in which two individuals had an autosomal-dominant axonal CMT with early-onset, sensory ataxia, tremor, and slow disease progression. Pedigree analysis and exome sequencing identified a de novo missense mutation (p.Y223H) in the diacylglycerol O-acyltransferase 2 (DGAT2) gene. DGAT2 encodes an endoplasmic reticulum-mitochondrial-associated membrane protein, acyl-CoA:diacylglycerol acyltransferase, which catalyzes the final step of the triglyceride (TG) biosynthesis pathway. The patient showed consistently decreased serum TG levels, and overexpression of the mutant DGAT2 significantly inhibited the proliferation of mouse motor neuron cells. Moreover, the variant form of human DGAT2 inhibited the axonal branching in the peripheral nervous system of zebrafish. We suggest that mutation of DGAT2 is the novel underlying cause of an autosomal-dominant axonal CMT2 neuropathy. This study will help provide a better understanding of the pathophysiology of axonal CMT and contribute to the molecular diagnostics of peripheral neuropathies.

  3. Bicyclic-Capped Histone Deacetylase 6 Inhibitors with Improved Activity in a Model of Axonal Charcot-Marie-Tooth Disease.

    PubMed

    Shen, Sida; Benoy, Veronick; Bergman, Joel A; Kalin, Jay H; Frojuello, Mariana; Vistoli, Giulio; Haeck, Wanda; Van Den Bosch, Ludo; Kozikowski, Alan P

    2016-02-17

    Charcot-Marie-Tooth (CMT) disease is a disorder of the peripheral nervous system where progressive degeneration of motor and sensory nerves leads to motor problems and sensory loss and for which no pharmacological treatment is available. Recently, it has been shown in a model for the axonal form of CMT that histone deacetylase 6 (HDAC6) can serve as a target for the development of a pharmacological therapy. Therefore, we aimed at developing new selective and activity-specific HDAC6 inhibitors with improved biochemical properties. By utilizing a bicyclic cap as the structural scaffold from which to build upon, we developed several analogues that showed improved potency compared to tubastatin A while maintaining excellent selectivity compared to HDAC1. Further screening in N2a cells examining both the acetylation of α-tubulin and histones narrowed down the library of compounds to three potent and selective HDAC6 inhibitors. In mutant HSPB1-expressing DRG neurons, serving as an in vitro model for CMT2, these inhibitors were able to restore the mitochondrial axonal transport deficits. Combining structure-based development of HDAC6 inhibitors, screening in N2a cells and in a neuronal model for CMT2F, and preliminary ADMET and pharmacokinetic profiles, resulted in the selection of compound 23d that possesses improved biochemical, functional, and druglike properties compared to tubastatin A.

  4. Charcot–Marie–Tooth disease and intracellular traffic

    PubMed Central

    Bucci, Cecilia; Bakke, Oddmund; Progida, Cinzia

    2012-01-01

    Mutations of genes whose primary function is the regulation of membrane traffic are increasingly being identified as the underlying causes of various important human disorders. Intriguingly, mutations in ubiquitously expressed membrane traffic genes often lead to cell type- or organ-specific disorders. This is particularly true for neuronal diseases, identifying the nervous system as the most sensitive tissue to alterations of membrane traffic. Charcot–Marie–Tooth (CMT) disease is one of the most common inherited peripheral neuropathies. It is also known as hereditary motor and sensory neuropathy (HMSN), which comprises a group of disorders specifically affecting peripheral nerves. This peripheral neuropathy, highly heterogeneous both clinically and genetically, is characterized by a slowly progressive degeneration of the muscle of the foot, lower leg, hand and forearm, accompanied by sensory loss in the toes, fingers and limbs. More than 30 genes have been identified as targets of mutations that cause CMT neuropathy. A number of these genes encode proteins directly or indirectly involved in the regulation of intracellular traffic. Indeed, the list of genes linked to CMT disease includes genes important for vesicle formation, phosphoinositide metabolism, lysosomal degradation, mitochondrial fission and fusion, and also genes encoding endosomal and cytoskeletal proteins. This review focuses on the link between intracellular transport and CMT disease, highlighting the molecular mechanisms that underlie the different forms of this peripheral neuropathy and discussing the pathophysiological impact of membrane transport genetic defects as well as possible future ways to counteract these defects. PMID:22465036

  5. A clinical study to evaluate the efficacy of intravitreal Anti-VEGF therapy in treating macular edema due to retinal venous occlusions

    PubMed Central

    Kumar, Poninder; Banarji, Ajay; Patyal, Sagarika; Gurunadh, V.S.; Ahluwalia, T.S.; Oli, Avadesh; Moulick, P.S.; Makker, Anuradha

    2013-01-01

    Background A non-randomized, interventional study was carried out various types of retinal venous occlusions with significant macular edema who required an Anti-VEGF injection. Method One hundred and one consecutive patients diagnosed as a case of CRVO/HCRVO/BRVO were enrolled in the study provided they had significant macular edema. Atleast three intra-vitreal injections of Anti-VEGFs were given and both the pre and post injections BCVA and CMT on OCT were observed and analyzed. Results 87 patients (86.14%) showed a significant improvement of vision of atleast two lines on the Snellen's and mean BCVA improved from log MAR +1.084 to log MAR +0.455. CMT on OCT showed reduction in thickness after Anti-VEGF therapy in 99 patients out of 101 and mean CMT decreased from 586.30 μ at baseline to 329.50 μ. Both of these findings were statistically very significant. Conclusions Anti-VEGF therapy had a marked improvement in BCVA along with a dramatic reduction in CMT in the vast majority of RVOs patients with no serious ocular or systemic side effects. PMID:24600120

  6. Remodelling of motor nerve terminals in demyelinating axons of periaxin null mutant mice

    PubMed Central

    Court, Felipe A; Brophy, Peter J; Ribchester, Richard R

    2015-01-01

    Myelin formation around axons increases nerve conduction velocity and regulates phenotypic characteristics of the myelinated axon. In the peripheral nervous system, demyelinating forms of hereditary Charcot-Marie-Tooth (CMT) diseases, due to Schwann-cell intrinsic molecular defects, leads to reduced nerve conduction velocity and changes in the axonal phenotype. Several mouse models of CMT diseases have been generated, allowing the study of consequences of demyelination in peripheral nerve fibres. Nevertheless, the effect of demyelination at the level of the neuromuscular synapse has been largely overlooked. Here we show that in the periaxin knock-out mice, a model of CMT condition, neuromuscular junctions develop profound morphological changes in pre-terminal region of motoraxons. These changes include extensive preterminal branches which originate in demyelinated regions of the nerve fibre and axonal swellings associated with residually-myelinated regions of the fibre. Using intracellular recording from muscle fibres we detected asynchronous failure of action potential transmission at high but not low stimulation frequencies, a phenomenon consistent with branch point failure. Taken together, our morphological and electrophysiological findings suggest that preterminal branching due to segmental demyelination near the neuromuscular synapse in periaxin KO mice may underlie phenotypic disabilities present in this mouse model of CMT disease. These results opens a new avenue of research in order to understand the cellular changes responsible for clinical disabilities in demyelinating conditions. PMID:18205176

  7. Evaluation of synchrotron X-ray computerized microtomography for the visualization of transport processes in low-porosity materials.

    PubMed

    Altman, Susan J; Peplinski, William J; Rivers, Mark L

    2005-07-01

    Synchrotron-source X-ray computerized microtomography (CMT) is evaluated as a method to visualize transport processes. We conclude that CMT is adequate for visualization of transport experiments if the right conditions exist. Namely, 1) not much more than one-order-of-magnitude range in concentration data is needed for the study, 2) the pore space in the samples are greater than approximately 2--50 mum, depending on the sample size and system setup; 3) the sample is fine-grained enough so that a representative elemental volume (REV) can be contained by a 2--10 mm diameter sample, and 4) the transport process is slow enough that significant changes do not occur within the 25--50 min (and possibly less in the future) needed to collect data for one three-dimensional image. Absorption edge difference imaging (AEDI) in association with CMT is introduced as a method to enhance pore-space visualization. We successfully imaged the pore space in a low-porosity granodiorite, diorite and fine-grained granite cores and a higher-porosity soil aggregate sample. We found that the pore space important to transport in the core samples was smaller than what we were able to visualize with CMT. We also made rudimentary associations of minerals with pore-space location.

  8. Separation system

    DOEpatents

    Rubin, Leslie S.

    1986-01-01

    A separation system for dewatering radioactive waste materials includes a disposal container, drive structure for receiving the container, and means for releasably attaching the container to the drive structure. Separation structure disposed in the container adjacent the inner surface of the side wall structure retains solids while allowing passage of liquids. Inlet port structure in the container top wall is normally closed by first valve structure that is centrifugally actuated to open the inlet port and discharge port structure at the container periphery receives liquid that passes through the separation structure and is normally closed by second valve structure that is centrifugally actuated to open the discharge ports. The container also includes coupling structure for releasable engagement with the centrifugal drive structure. Centrifugal force produced when the container is driven in rotation by the drive structure opens the valve structures, and radioactive waste material introduced into the container through the open inlet port is dewatered, and the waste is compacted. The ports are automatically closed by the valves when the container drum is not subjected to centrifugal force such that containment effectiveness is enhanced and exposure of personnel to radioactive materials is minimized.

  9. Incinerator system

    SciTech Connect

    Rathmell, R.K.

    1986-10-07

    An incineration system is described which consists of: combustion chamber structure having an inlet, an outlet, and burner structure in the combustion chamber, heat exchanger structure defining a chamber, divider structure between the heat exchanger chamber and the combustion chamber, an array of tubes extending through the heat exchanger chamber to the inlet of the combustion chamber at the divider structure. The heat exchanger chamber has an inlet coupled to the outlet of the combustion chamber for flow of the combustion products discharged from the combustion chamber through the heat exchanger chamber over the tubes in heat exchange relation, and an outlet for discharge of products from the heat exchanger chamber, aspirator sleeve structure secured to the divider structure between the heat exchanger chamber and the combustion chamber. Each aspirator sleeve receives the outlet end of a heat exchanger tube in slip fit relation so that the heat exchanger tubes are free to thermally expand longitudinally within the aspirator sleeves, and means for flowing vapor through the heat exchanger tubes into the combustion chamber at sufficiently high velocity to produce a reduced pressure effect in the aspirator sleeves in the heat exchanger chamber to draw a minor fraction of combustion products through the aspirator sleeves into the combustion chamber for reincineration.

  10. Systemic vasculitis.

    PubMed

    Sharma, Poonam; Sharma, Sanjeev; Baltaro, Richard; Hurley, John

    2011-03-01

    The systemic vasculitides are characterized by inflammation of blood vessel walls. Vessels of any type, in any organ can be affected, resulting in a broad spectrum of signs and symptoms. The heterogenous nature of vasculitides presents a diagnostic challenge. The American College of Rheumatology classification criteria and the Chapel Hill Consensus Conference nomenclature are the most widely used to distinguish different forms of vasculitis. The Chapel Hill Consensus Conference nomenclature defines 10 primary vasculitides based on vessel size (large, medium, and small). The diagnosis relies on the recognition of a compatible clinical presentation supported by specific laboratory or imaging tests and confirmatory histology. Antineutrophilic cytoplasmic antibody testing has been of particular benefit in defining a subgroup of small vessel vasculitides. Treatment is based on clinical presentation and the pattern of organ involvement. Glucocorticoids are the primary treatment for many forms of vasculitis. Additional immunosuppressive agents, including methotrexate and cyclophosphamide, are sometimes required. Newer approaches, such as the use of anti-tumor necrosis factor or B cell therapies, are being tried in resistant cases. Patients can experience considerable treatment-related toxicity, especially infection from immunosuppressive therapy and adverse effects from steroids (e.g., osteoporosis, diabetes mellitus, cataract). Vitamin D and calcium prophylaxis are recommended in patients on long-term steroid therapy.

  11. Endocrine System (For Teens)

    MedlinePlus

    ... Loss Surgery? A Week of Healthy Breakfasts Shyness Endocrine System KidsHealth > For Teens > Endocrine System A A A ... is called the endocrine system . What Is the Endocrine System? Although we rarely think about the endocrine system, ...

  12. New Systems Produced by Systemic Change

    ERIC Educational Resources Information Center

    Battino, Wendy; Clem, Jo; Caine, Renate N.; Reigeluth, Charles M.; Chapman, Carrie; Flinders, David J.; Malopinsky, Larissa V.

    2006-01-01

    This article presents new systems produced by systemic change. First is Systemic Changes in the Chugach School District by Wendy Battino and Jo Clem. Second is Systemic Changes in Public Schools through Brain-Based Learning by Renate N. Caine. Third is A Vision of an Information-Age Educational System by Charles M. Reigeluth. Fourth is Systemic…

  13. Airborne Global Positioning System Antenna System

    DTIC Science & Technology

    2004-10-14

    GLOBAL POSITIONING SYSTEM ANTENNA SYSTEM DISTRIBUTION: SMC/ GP (3 cys); AFFSA...standard that airborne Global Positioning System ( GPS ) antenna system must meet to be identified with the applicable MSO marking. The similarity of...UNCLASSIFIED DOCUMENT NO. DATE NO. MSO-C144 14 Oct 04 Initial Release REV: REV: SHEET 1 OF 16 TITLE: AIRBORNE GLOBAL POSITIONING SYSTEM

  14. Efficacy of focal mechanic vibration treatment on balance in Charcot-Marie-Tooth 1A disease: a pilot study.

    PubMed

    Pazzaglia, Costanza; Camerota, F; Germanotta, M; Di Sipio, E; Celletti, C; Padua, L

    2016-07-01

    Patients affected by Charcot-Marie-Tooth (CMT) disease experience an impaired balance. Although the causes of the postural instability are not fully understood, somatosensory system seems to play a key role. Mechanical vibration seems to act on the somatosensory system and to improve its function. The aim of our study was to evaluate the effects of focal mechanical vibration (fMV) on the balance of CMT 1A patients. We enrolled 14 genetically confirmed CMT 1A patients (8 female and 6 male, mean age 492 years, range 32-74, mean duration of disease: 13 years, range 1-30). Patients underwent a 3-day fMV treatment on quadriceps and triceps surae and were evaluated before the treatment as well as 1 week and 1 month after the end of the treatment. The primary outcome measure was the Berg Balance Scale (BBS) and the secondary were the Dynamic Gait Index (DGI), the 6 Min Walking Test (6MWT), the muscular strength of lower limbs, the Quality of Life (QoL) questionnaire and the stabilometric variables. The statistical analysis showed a significant modification of the BBS due to the effect of treatment (p < 0.05). A significant modification was also found in the DGI (p < 0.05). Concerning the stabilometric variables we found significant changes only for the eyes closed condition; in particular, a significant decrease was found in VelocityML (p < 0.05) and Sway path length (p < 0.05). The fMV treatment applied on lower limbs of CMT 1A patients determined an improvement of balance as detected by the BBS. The concurrent improvement of stabilometric variables in the eyes closed condition only suggests that fMV acts mostly on somatosensory afferences. Further studies are needed to confirm these data on a larger sample of CMT patients.

  15. Development of Cd1-xMgxTe thin films for application as an electron reflector in CdS/CdTe solar cells

    NASA Astrophysics Data System (ADS)

    Kobyakov, Pavel S.

    Efficiencies of CdS/CdTe photovoltaic cells significantly lag behind their theoretical limit, primarily because open-circuit voltage ( VOC) of record efficiency cells (872 mV) is well below what is expected for the CdTe band gap (1.5 eV). A substantial V OC improvement can be achieved through addition of an electron reflector (ER) layer to CdTe devices. The ER layer forms a conduction-band barrier that reflects minority-charge carriers (i.e. electrons in p-type CdTe) away from the back surface. Similar to back-surface fields in c-Si, III-V, and CIGS solar cells, the ER strategy is expected to reduce back-surface recombination and is estimated to increase CdTe VOC by about 200 mV based on numerical simulation. The presented research investigates the addition of a thin layer of wider band gap Cd1-xMgxTe (CMT) to achieve a CdTe ER structure. First, a novel co-sublimation process was developed for deposition of Cd 1-xMgxTe thin films that demonstrates excellent experimental capabilities, commercial viability, and improved alloy control over other techniques. Next, the effects of processing on material properties of CMT deposition onto CdS/CdTe structures were investigated. It was discovered that substrate temperature during CMT deposition is a critical parameter for achieving uniform CMT film coverage on polycrystalline CdTe. Furthermore, CMT film growth was found to be epitaxial on CdTe where the CMT films retain the same microstructural features as the underlying CdTe grains. Despite film uniformity, significant Mg loss from the CMT film, oxide formation, and a reduction of the optical band gap was found after CdCl2-based passivation treatments. Preliminary process optimization found that band gap degradation can be minimized by utilizing MgCl2 in addition to CdCl2 as a treatment source material. Finally, development of CdS/CdTe/Cd1-xMgxTe electron reflector devices demonstrated a barrier behavior at high voltage bias and improved voltage when CdTe thickness is held

  16. RADAR WARNING SYSTEM,

    DTIC Science & Technology

    RADAR TRACKING, *AIRCRAFT DEFENSE SYSTEMS, RADAR EQUIPMENT, AIR TO AIR, SEARCH RADAR, GUIDED MISSILES, HIGH SPEED BOMBING, EARLY WARNING SYSTEMS, FIRE CONTROL SYSTEM COMPONENTS, AIRCRAFT, TIME, CHINA.

  17. System design description cone penetrometer system

    SciTech Connect

    Seda, R.Y., Westinghouse Hanford

    1996-08-12

    The system design description documents in detail the design of the cone penetrometer system. The systems includes the cone penetrometer physical package, raman spectroscopy package and moisture sensor package. Information pertinent to the system design, development, fabrication and testing is provided.

  18. Networked control of microgrid system of systems

    NASA Astrophysics Data System (ADS)

    Mahmoud, Magdi S.; Rahman, Mohamed Saif Ur; AL-Sunni, Fouad M.

    2016-08-01

    The microgrid has made its mark in distributed generation and has attracted widespread research. However, microgrid is a complex system which needs to be viewed from an intelligent system of systems perspective. In this paper, a network control system of systems is designed for the islanded microgrid system consisting of three distributed generation units as three subsystems supplying a load. The controller stabilises the microgrid system in the presence of communication infractions such as packet dropouts and delays. Simulation results are included to elucidate the effectiveness of the proposed control strategy.

  19. From systems biology to systems biomedicine.

    PubMed

    Antony, Paul M A; Balling, Rudi; Vlassis, Nikos

    2012-08-01

    Systems Biology is about combining theory, technology, and targeted experiments in a way that drives not only data accumulation but knowledge as well. The challenge in Systems Biomedicine is to furthermore translate mechanistic insights in biological systems to clinical application, with the central aim of improving patients' quality of life. The challenge is to find theoretically well-chosen models for the contextually correct and intelligible representation of multi-scale biological systems. In this review, we discuss the current state of Systems Biology, highlight the emergence of Systems Biomedicine, and highlight some of the topics and views that we think are important for the efficient application of Systems Theory in Biomedicine.

  20. Global Positioning System Shipborne Reference System

    DTIC Science & Technology

    1997-09-30

    Office of Naval Research Space and Remote Sensing 1997 Annual Report 1 GLOBAL POSITIONING SYSTEM SHIPBORNE REFERENCE SYSTEM James R. Clynch...N00014-97-WR30044 LONG-TERM GOAL The long term goal is to improve the navigation capability of naval vessels using the Global Positioning System ...COVERED 00-00-1997 to 00-00-1997 4. TITLE AND SUBTITLE Global Positioning System Shipborne Reference System 5a. CONTRACT NUMBER 5b. GRANT

  1. Mechanical systems: A compilation

    NASA Technical Reports Server (NTRS)

    1975-01-01

    A compilation of several mechanized systems is presented. The articles are contained in three sections: robotics, industrial mechanical systems, including several on linear and rotary systems and lastly mechanical control systems, such as brakes and clutches.

  2. Endocrine System (For Parents)

    MedlinePlus

    ... Old Feeding Your 1- to 2-Year-Old Endocrine System KidsHealth > For Parents > Endocrine System A A A ... to help the body function properly. About the Endocrine System The foundations of the endocrine system are the ...

  3. Immune System (For Parents)

    MedlinePlus

    ... Old Feeding Your 1- to 2-Year-Old Immune System KidsHealth > For Parents > Immune System A A A ... can lead to illness and infection. About the Immune System The immune system is the body's defense against ...

  4. Quantify information system benefits

    SciTech Connect

    Koppel, L.B.

    1995-06-01

    What are information systems and how do they relate to control systems? How do information systems produce benefits in hydrocarbon processing? What are some examples of benefit-generating information system applications? Information System Benefits (ISBEN) is a structured methodology for estimating information system benefits in hydrocarbon processing. The paper discusses information and control systems, information system benefits and applications, objectives, strategies and measures of ISBEN, ISBEN business drivers, ISBEN database, ISBEN methodology, and implementation.

  5. Data Systems Task Analysis.

    DTIC Science & Technology

    1979-08-01

    A08873 MARINE CORPS WASHINGTON DC F/B 5/9 DATA SYSTEMS TASK ANALYSIS. (U) "CLASSIFIEO. -%mm . LEVELIs DATA SYSTEMS O0 TASK ANALYSIS DCI) OO JF AUG 28...TECI-NICIAN jl. CCMPUTER SYSTEMS EVALLATOR ,13. LCMPUTER SYSTEMS MANAGER 314. LCMPUTER SYSTEMS MCNITOR A5o LCMPUTER TERMINAL OPERATOR j16. LCNFIGURATION...OPERATOR )27. DATA PROCESSING NCO )28. DATA PROCESSINIG TECHNICIAN 329e DATA SYSTEMS LIBRARIAN )35* DATA SYSTEMS OPERATICNS Cl-IEF 331. DATA SYSTEMS

  6. Practical systems thinking

    NASA Astrophysics Data System (ADS)

    Konkarikoski, K.; Ritala, R.; Ihalainen, H.

    2010-07-01

    System is a dynamic and complex whole, interacting as a structured functional unit. Systems thinking provides tools for understanding a such system structure and its dynamic behavior. Practical systems thinking course teaches first year bachelor students basics about systems and how open problem can be formulated to system task.

  7. System of systems modeling and analysis.

    SciTech Connect

    Campbell, James E.; Anderson, Dennis James; Longsine, Dennis E.; Shirah, Donald N.

    2005-01-01

    This report documents the results of an LDRD program entitled 'System of Systems Modeling and Analysis' that was conducted during FY 2003 and FY 2004. Systems that themselves consist of multiple systems (referred to here as System of Systems or SoS) introduce a level of complexity to systems performance analysis and optimization that is not readily addressable by existing capabilities. The objective of the 'System of Systems Modeling and Analysis' project was to develop an integrated modeling and simulation environment that addresses the complex SoS modeling and analysis needs. The approach to meeting this objective involved two key efforts. First, a static analysis approach, called state modeling, has been developed that is useful for analyzing the average performance of systems over defined use conditions. The state modeling capability supports analysis and optimization of multiple systems and multiple performance measures or measures of effectiveness. The second effort involves time simulation which represents every system in the simulation using an encapsulated state model (State Model Object or SMO). The time simulation can analyze any number of systems including cross-platform dependencies and a detailed treatment of the logistics required to support the systems in a defined mission.

  8. System Software Framework for System of Systems Avionics

    NASA Technical Reports Server (NTRS)

    Ferguson, Roscoe C.; Peterson, Benjamin L; Thompson, Hiram C.

    2005-01-01

    Project Constellation implements NASA's vision for space exploration to expand human presence in our solar system. The engineering focus of this project is developing a system of systems architecture. This architecture allows for the incremental development of the overall program. Systems can be built and connected in a "Lego style" manner to generate configurations supporting various mission objectives. The development of the avionics or control systems of such a massive project will result in concurrent engineering. Also, each system will have software and the need to communicate with other (possibly heterogeneous) systems. Fortunately, this design problem has already been solved during the creation and evolution of systems such as the Internet and the Department of Defense's successful effort to standardize distributed simulation (now IEEE 1516). The solution relies on the use of a standard layered software framework and a communication protocol. A standard framework and communication protocol is suggested for the development and maintenance of Project Constellation systems. The ARINC 653 standard is a great start for such a common software framework. This paper proposes a common system software framework that uses the Real Time Publish/Subscribe protocol for framework-to-framework communication to extend ARINC 653. It is highly recommended that such a framework be established before development. This is important for the success of concurrent engineering. The framework provides an infrastructure for general system services and is designed for flexibility to support a spiral development effort.

  9. Intelligent systems technology infrastructure for integrated systems

    NASA Technical Reports Server (NTRS)

    Lum, Henry, Jr.

    1991-01-01

    Significant advances have occurred during the last decade in intelligent systems technologies (a.k.a. knowledge-based systems, KBS) including research, feasibility demonstrations, and technology implementations in operational environments. Evaluation and simulation data obtained to date in real-time operational environments suggest that cost-effective utilization of intelligent systems technologies can be realized for Automated Rendezvous and Capture applications. The successful implementation of these technologies involve a complex system infrastructure integrating the requirements of transportation, vehicle checkout and health management, and communication systems without compromise to systems reliability and performance. The resources that must be invoked to accomplish these tasks include remote ground operations and control, built-in system fault management and control, and intelligent robotics. To ensure long-term evolution and integration of new validated technologies over the lifetime of the vehicle, system interfaces must also be addressed and integrated into the overall system interface requirements. An approach for defining and evaluating the system infrastructures including the testbed currently being used to support the on-going evaluations for the evolutionary Space Station Freedom Data Management System is presented and discussed. Intelligent system technologies discussed include artificial intelligence (real-time replanning and scheduling), high performance computational elements (parallel processors, photonic processors, and neural networks), real-time fault management and control, and system software development tools for rapid prototyping capabilities.

  10. [X-33 Systems

    NASA Technical Reports Server (NTRS)

    1999-01-01

    Lockheed Martin Skunk Works has compiled an Annual Performance Report of the X-33/RLV Program. This report consists of individual reports from all industry team members, as well as NASA team centers. This portion of the report is comprised of a status report of Allied-Signal Aerospace's contribution to the program. The following is a summary of the work reviewed under their portion of the agreement: (1) Communication Systems; (2) Environmental Control Systems- Active Thermal Control System (ATCS), Purge and Vent System, Hydrogen Detection System (HDS), Avionics Bay Inerting System (ABIS), and Flush Air Data System (FADS); (2) Landing Systems; (3) Power Management and Generation Systems; (4) Flight Control Actuation System (FCAS)- Electric Power Control & Distribution System (EPCDS), and Battery Power System (BPS); and (5) Vehicle Management Systems (VMS)- VMS Hardware, VMS Software Development Activities, and System Integration Laboratory (SIL).

  11. Systems engineering and analysis

    SciTech Connect

    Blanchard, B.S.; Fabrycky, W.J.

    1981-01-01

    An introduction to systems is provided and tools for systems analysis are considered, taking into account system definitions and concepts, approaches for bringing systems into being, models in systems analysis, economic analysis techniques, mathematical modeling and optimization, probability and statistics, queuing theory and analysis, and control concepts and techniques. The system design process is discussed along with the design for operational feasibility, systems engineering management, and system design case studies. Attention is given to conceptual design, preliminary system design, detail design and development, system test and evaluation, design for reliability, design for maintainability, design for supportability, design for economic feasibility, communication system design, finite population system design, energy storage system design, and procurement-inventory system design.

  12. Language as a System of Systems

    ERIC Educational Resources Information Center

    Mulder, J. W. F.; Hervey, S. G. J.

    1975-01-01

    Based on Mulder's previous classification of all semiotic systems designed to describe the system of discrete features in human languages, this article explores a further subclassification of the genus language into species. (CLK)

  13. Freedom System Text and Graphics System (TAGS)

    NASA Technical Reports Server (NTRS)

    1989-01-01

    The Text and Graphics System (TAGS) is a high-resolution facsimile system that scans text or graphics material and converts the analog SCAN data into serial digital data. This video shows the TAGS in operation.

  14. LCLS XTOD Attenuator System System Concept Report

    SciTech Connect

    Kishiyama, K; Roeben, M; Trent, J; Ryutov, D; Shen, S

    2006-04-12

    The attenuator system for the Linac Coherent Light Source (LCLS) X-ray Transport, Optics and Diagnostics (XTOD) system has been configured and analyzed by the Lawrence Livermore National Laboratory's New Technologies Engineering Division (NTED) as requested by the SLAC/LCLS program. The system layout, performance analyses and selection of the vacuum components are presented in this System Conceptual Review (SCR) report. Also included are the plans for prototype, procurement, mechanical integration, and the cost estimates.

  15. Systems Theory, Systems Technology, and Curriculum Design.

    ERIC Educational Resources Information Center

    Pratt, David

    1978-01-01

    John Goodlad (1958) stated that "Nowhere in education is there greater need for a conceptual system to guide decision-making than the field of curriculum." This research attempts to explore ways in which systems thinking can provide a conceptual system, to illuminate the study of curriculum and guide the design of curricula. (Author/RK)

  16. Intelligent tutoring systems for systems engineering methodologies

    NASA Technical Reports Server (NTRS)

    Meyer, Richard J.; Toland, Joel; Decker, Louis

    1991-01-01

    The general goal is to provide the technology required to build systems that can provide intelligent tutoring in IDEF (Integrated Computer Aided Manufacturing Definition Method) modeling. The following subject areas are covered: intelligent tutoring systems for systems analysis methodologies; IDEF tutor architecture and components; developing cognitive skills for IDEF modeling; experimental software; and PC based prototype.

  17. Systems Intelligence Inventory

    ERIC Educational Resources Information Center

    Törmänen, Juha; Hämäläinen, Raimo P.; Saarinen, Esa

    2016-01-01

    Purpose: Systems intelligence (SI) (Saarinen and Hämäläinen, 2004) is a construct defined as a person's ability to act intelligently within complex systems involving interaction and feedback. SI relates to our ability to act in systems and reason about systems to adaptively carry out productive actions within and with respect to systems such as…

  18. Collaborative Systems Testing

    ERIC Educational Resources Information Center

    Pocatilu, Paul; Ciurea, Cristian

    2009-01-01

    Collaborative systems are widely used today in various activity fields. Their complexity is high and the development involves numerous resources and costs. Testing collaborative systems has a very important role for the systems' success. In this paper we present taxonomy of collaborative systems. The collaborative systems are classified in many…

  19. Systems Engineering Measurement Primer

    DTIC Science & Technology

    1998-03-01

    Systems Engineering Measurement Primer A Basic Introduction to Systems Engineering Measurement Concepts and Use Version 1.0 March 1998 This document...Federal Systems Garry Roedler Lockheed Martin Management & Data Systems Cathy Tilton The National Registry, Inc. E. Richard Widmann Raytheon Systems...IV 1. INTRODUCTION

  20. Intelligent test integration system

    NASA Technical Reports Server (NTRS)

    Sztipanovits, J.; Padalkar, S.; Rodriguez-Moscoso, J.; Kawamura, K.; Purves, B.; Williams, R.; Biglari, H.

    1988-01-01

    A new test technology is described which was developed for space system integration. The ultimate purpose of the system is to support the automatic generation of test systems in real time, distributed computing environments. The Intelligent Test Integration System (ITIS) is a knowledge based layer above the traditional test system components which can generate complex test configurations from the specification of test scenarios.

  1. Systems Measures of Water Distribution System Resilience

    SciTech Connect

    Klise, Katherine A.; Murray, Regan; Walker, La Tonya Nicole

    2015-01-01

    Resilience is a concept that is being used increasingly to refer to the capacity of infrastructure systems to be prepared for and able to respond effectively and rapidly to hazardous events. In Section 2 of this report, drinking water hazards, resilience literature, and available resilience tools are presented. Broader definitions, attributes and methods for measuring resilience are presented in Section 3. In Section 4, quantitative systems performance measures for water distribution systems are presented. Finally, in Section 5, the performance measures and their relevance to measuring the resilience of water systems to hazards is discussed along with needed improvements to water distribution system modeling tools.

  2. DDL system: Design systhesis of digital systems

    NASA Technical Reports Server (NTRS)

    Shiva, S. G.

    1983-01-01

    Digital Systems Design Language was integrated into the CADAT system environment of NASA-MSFC. The major technical aspects of this integration are summarized. Automatic hardware synthesis is now possible starting with a high level description of the system to be synthesized. The DDL system provides a high level design verification capability, thereby minimizing design changes in the later stages of the design cycle. An overview of the DDL system covering the translation, simulation and synthesis capabilities is provided. Two companion documents (the user's and programmer's manuals) are to be consulted for detailed discussions.

  3. Microwave landing system autoland system analysis

    NASA Technical Reports Server (NTRS)

    Feather, J. B.; Craven, B. K.

    1991-01-01

    The objective was to investigate the ability of present day aircraft equipped with automatic flight control systems to fly advanced Microwave Landing Systems (MLS) approaches. The tactical approach used to achieve this objective included reviewing the design and autoland operation of the MD-80 aircraft, simulating the MLS approaches using a batch computer program, and assessing the performance of the autoland system from computer generated data. The results showed changes were required to present Instrument Landing System (ILS) procedures to accommodate the new MLS curved paths. It was also shown that in some cases, changes to the digital flight guidance systems would be required so that an autoland could be performed.

  4. Lighting system with thermal management system

    DOEpatents

    Arik, Mehmet; Weaver, Stanton Earl; Stecher, Thomas Elliot; Seeley, Charles Erklin; Kuenzler, Glenn Howard; Wolfe, Jr., Charles Franklin; Utturkar, Yogen Vishwas; Sharma, Rajdeep; Prabhakaran, Satish; Icoz, Tunc

    2015-08-25

    Lighting systems having unique configurations are provided. For instance, the lighting system may include a light source, a thermal management system and driver electronics, each contained within a housing structure. The light source is configured to provide illumination visible through an opening in the housing structure. The thermal management system is configured to provide an air flow, such as a unidirectional air flow, through the housing structure in order to cool the light source. The driver electronics are configured to provide power to each of the light source and the thermal management system.

  5. Lighting system with thermal management system

    DOEpatents

    Arik, Mehmet; Weaver, Stanton Earl; Stecher, Thomas Elliot; Seeley, Charles Erklin; Kuenzler, Glenn Howard; Wolfe, Jr., Charles Franklin; Utturkar, Yogen Vishwas; Sharma, Rajdeep; Prabhakaran, Satish; Icoz, Tunc

    2015-02-24

    Lighting systems having unique configurations are provided. For instance, the lighting system may include a light source, a thermal management system and driver electronics, each contained within a housing structure. The light source is configured to provide illumination visible through an opening in the housing structure. The thermal management system is configured to provide an air flow, such as a unidirectional air flow, through the housing structure in order to cool the light source. The driver electronics are configured to provide power to each of the light source and the thermal management system.

  6. Lighting system with thermal management system

    DOEpatents

    Arik, Mehmet; Weaver, Stanton Earl; Stecher, Thomas Elliot; Seeley, Charles Erklin; Kuenzler, Glenn Howard; Wolfe, Jr, Charles Franklin; Utturkar, Yogen Vishwas; Sharma, Rajdeep; Prabhakaran, Satish; Icoz, Tunc

    2016-10-11

    Lighting systems having unique configurations are provided. For instance, the lighting system may include a light source, a thermal management system and driver electronics, each contained within a housing structure. The light source is configured to provide illumination visible through an opening in the housing structure. The thermal management system is configured to provide an air flow, such as a unidirectional air flow, through the housing structure in order to cool the light source. The driver electronics are configured to provide power to each of the light source and the thermal management system.

  7. Lighting system with thermal management system

    DOEpatents

    Arik, Mehmet; Weaver, Stanton; Stecher, Thomas; Seeley, Charles; Kuenzler, Glenn; Wolfe, Jr., Charles; Utturkar, Yogen; Sharma, Rajdeep; Prabhakaran, Satish; Icoz, Tunc

    2013-05-07

    Lighting systems having unique configurations are provided. For instance, the lighting system may include a light source, a thermal management system and driver electronics, each contained within a housing structure. The light source is configured to provide illumination visible through an opening in the housing structure. The thermal management system is configured to provide an air flow, such as a unidirectional air flow, through the housing structure in order to cool the light source. The driver electronics are configured to provide power to each of the light source and the thermal management system.

  8. The LSST: A System of Systems

    NASA Astrophysics Data System (ADS)

    Claver, Chuck F.; Debois-Felsmann, G. P.; Delgado, F.; Hascall, P.; Marshall, S.; Nordby, M.; Schumacher, G.; Sebag, J.; LSST Collaboration

    2011-01-01

    The Large Synoptic Survey Telescope (LSST) is a complete observing system that acquires and archives images, processes and analyzes them, and publishes reduced images and catalogs of sources and objects. The LSST will operate over a ten year period producing a survey of 20,000 square degrees over the entire [Southern] sky in 6 filters (ugrizy) with each field having been visited several hundred times enabling a wide spectrum of science from fast transients to exploration of dark matter and dark energy. The LSST itself is a complex system of systems consisting of the 8.4m 3-mirror telescope, a 3.2 billion pixel camera, and a peta-scale data management system. The LSST project uses a Model Based Systems Engineering (MBSE) methodology to ensure an integrated approach to system design and rigorous definition of system interfaces and specifications. The MBSE methodology is applied through modeling of the LSST's systems with the System Modeling Language (SysML). The SysML modeling recursively establishes the threefold relationship between requirements, logical & physical functional decomposition and definition, and system and component behavior at successively deeper level of abstraction and detail. The LSST modeling includes the analysis and documenting the flow of command and control information and data between the suite of systems in the LSST observatory that are needed to carry out the activities of the survey. The MBSE approach is applied throughout all stages of the project from design, to validation and verification, though to commissioning.

  9. Systems Architecture for a Nationwide Healthcare System.

    PubMed

    Abin, Jorge; Nemeth, Horacio; Friedmann, Ignacio

    2015-01-01

    From a national level to give Internet technology support, the Nationwide Integrated Healthcare System in Uruguay requires a model of Information Systems Architecture. This system has multiple healthcare providers (public and private), and a strong component of supplementary services. Thus, the data processing system should have an architecture that considers this fact, while integrating the central services provided by the Ministry of Public Health. The national electronic health record, as well as other related data processing systems, should be based on this architecture. The architecture model described here conceptualizes a federated framework of electronic health record systems, according to the IHE affinity model, HL7 standards, local standards on interoperability and security, as well as technical advice provided by AGESIC. It is the outcome of the research done by AGESIC and Systems Integration Laboratory (LINS) on the development and use of the e-Government Platform since 2008, as well as the research done by the team Salud.uy since 2013.

  10. Biohazard Detoxification Method Utilizing Magnetic Particles

    DTIC Science & Technology

    2007-05-01

    developing a detoxification system for the human blood that is based on magnetic nanoparticles . A key component of the proposed system is a portable...Targeting of Functionalized Nanoparticles ," Argonne National Laboratory Report ANL-CMT-04/02, September 2004. 7. M. D. Kaminski and A. J. Rosengart...Finck, S. Guy, and A.J. Rosengart, "In Vitro Studies of Functionalized Magnetic Nanoparticles for Selective Removal of a Simulant Biotoxin," J. Magn. Magn

  11. TWRSview system requirements specification

    SciTech Connect

    Caldwell, J.A.; Lee, A.K.

    1995-12-01

    This document provides the system requirements specification for the TWRSview software system. The TWRSview software system is being developed to integrate electronic data supporting the development of the TWRS technical baseline

  12. Multivariable Control Systems

    DTIC Science & Technology

    1968-01-01

    one). Examples abound of systems with numerous controlled variables, and the modern tendency is toward ever greater utilization of systems and plants of this kind. We call them multivariable control systems (MCS).

  13. Autonomic Nervous System Disorders

    MedlinePlus

    Your autonomic nervous system is the part of your nervous system that controls involuntary actions, such as the beating of your heart ... breathing and swallowing Erectile dysfunction in men Autonomic nervous system disorders can occur alone or as the result ...

  14. Manned systems technology discipline

    NASA Technical Reports Server (NTRS)

    Bretoi, Remus

    1990-01-01

    Viewgraphs on manned systems technology discipline for Space Station Freedom are presented. Topics covered include: crew-systems interfaces and interactions; crew training; on-board systems maintenance and support; habitability and environment; and computational human factors.

  15. Air cushion landing system

    NASA Technical Reports Server (NTRS)

    Boghami, K. M.; Captain, K. M.; Fish, R. B.

    1978-01-01

    Static and dynamic performance of air cushion landing system is simulated in computer program that treats four primary ACLS subsystems: fan, feeding system, trunk, and cushion. Configuration of systems is sufficiently general to represent variety of practical designs.

  16. Wind energy systems

    NASA Technical Reports Server (NTRS)

    Stewart, H. J.

    1978-01-01

    A discussion on wind energy systems involved with the DOE wind energy program is presented. Some of the problems associated with wind energy systems are discussed. The cost, efficiency, and structural design of wind energy systems are analyzed.

  17. Lungs and Respiratory System

    MedlinePlus

    ... Your 1- to 2-Year-Old Lungs and Respiratory System KidsHealth > For Parents > Lungs and Respiratory System Print ... ll have taken at least 600 million breaths. Respiratory System Basics All of this breathing couldn't happen ...

  18. The Trinity System

    SciTech Connect

    Archer, Billy Joe; Vigil, Benny Manuel

    2015-01-13

    This paper describes the Trinity system, the first ASC Advanced Technology System (ATS-1). We describe the Trinity procurement timeline, the ASC computing strategy, the Trinity specific mission needs, and the Trinity system specifications.

  19. Lungs and Respiratory System

    MedlinePlus

    ... Your 1- to 2-Year-Old Lungs and Respiratory System KidsHealth > For Parents > Lungs and Respiratory System A ... ll have taken at least 600 million breaths. Respiratory System Basics All of this breathing couldn't happen ...

  20. Evaluating a Portfolio System.

    ERIC Educational Resources Information Center

    Smit, David W.

    1990-01-01

    Contributes to the knowledge of portfolio systems for writing evaluation by sharing evaluative procedures and their results. Reports on the results of a survey of students evaluated using a portfolio system. Finds that students preferred the portfolio system. (RS)

  1. Antiskid braking system

    NASA Technical Reports Server (NTRS)

    Pazdera, J. S.

    1974-01-01

    Published report describes analytical development and simulation of braking system. System prevents wheels from skidding when brakes are applied, significantly reducing stopping distance. Report also presents computer simulation study on system as applied to aircraft.

  2. Digestive System (For Teens)

    MedlinePlus

    ... Loss Surgery? A Week of Healthy Breakfasts Shyness Digestive System KidsHealth > For Teens > Digestive System A A A ... out of the body as feces. About the Digestive System Every morsel of food we eat has to ...

  3. Immune System Quiz

    MedlinePlus

    ... Room? What Happens in the Operating Room? Quiz: Immune System KidsHealth > For Kids > Quiz: Immune System A A A How much do you know about your immune system? Find out by taking this quiz! About KidsHealth ...

  4. Systems interface biology

    PubMed Central

    Doyle, Francis J; Stelling, Jörg

    2006-01-01

    The field of systems biology has attracted the attention of biologists, engineers, mathematicians, physicists, chemists and others in an endeavour to create systems-level understanding of complex biological networks. In particular, systems engineering methods are finding unique opportunities in characterizing the rich behaviour exhibited by biological systems. In the same manner, these new classes of biological problems are motivating novel developments in theoretical systems approaches. Hence, the interface between systems and biology is of mutual benefit to both disciplines. PMID:16971329

  5. System status display information

    NASA Technical Reports Server (NTRS)

    Summers, L. G.; Erickson, J. B.

    1984-01-01

    The system Status Display is an electronic display system which provides the flight crew with enhanced capabilities for monitoring and managing aircraft systems. Guidelines for the design of the electronic system displays were established. The technical approach involved the application of a system engineering approach to the design of candidate displays and the evaluation of a Hernative concepts by part-task simulation. The system engineering and selection of candidate displays are covered.

  6. Propulsion Systems Panel deliberations

    NASA Technical Reports Server (NTRS)

    Bianca, Carmelo J.; Miner, Robert; Johnston, Lawrence M.; Bruce, R.; Dennies, Daniel P.; Dickenson, W.; Dreshfield, Robert; Karakulko, Walt; Mcgaw, Mike; Munafo, Paul M.

    1993-01-01

    The Propulsion Systems Panel was established because of the specialized nature of many of the materials and structures technology issues related to propulsion systems. This panel was co-chaired by Carmelo Bianca, MSFC, and Bob Miner, LeRC. Because of the diverse range of missions anticipated for the Space Transportation program, three distinct propulsion system types were identified in the workshop planning process: liquid propulsion systems, solid propulsion systems and nuclear electric/nuclear thermal propulsion systems.

  7. Systems interface biology.

    PubMed

    Doyle, Francis J; Stelling, Jörg

    2006-10-22

    The field of systems biology has attracted the attention of biologists, engineers, mathematicians, physicists, chemists and others in an endeavour to create systems-level understanding of complex biological networks. In particular, systems engineering methods are finding unique opportunities in characterizing the rich behaviour exhibited by biological systems. In the same manner, these new classes of biological problems are motivating novel developments in theoretical systems approaches. Hence, the interface between systems and biology is of mutual benefit to both disciplines.

  8. The LSST: A System of Systems

    NASA Astrophysics Data System (ADS)

    Claver, Chuck F.; Dubois-Felsmann, G. P.; Delgado, F.; Hascall, P.; Horn, D.; Marshall, S.; Nordby, M.; Schalk, T. L.; Schumacher, G.; Sebag, J.; LSST Project Team

    2010-01-01

    The LSST is a complete observing system that acquires and archives images, processes and analyzes them, and publishes reduced images and catalogs of sources and objects. The LSST will operate over a ten year period producing a survey of 20,000 square degrees over the entire southern sky in 6 filters (ugrizy) with each field having been visited several hundred times enabling a wide spectrum of science from fast transients to exploration of dark matter and dark energy. The LSST itself is a complex system of systems consisting of the 8.4m three mirror telescope, a 3.2 billion pixel camera, and a peta-scale data management system. The LSST project uses a Model Based Systems Engineering (MBSE) methodology to ensure an integrated approach to system design and rigorous definition of system interfaces and specifications. The MBSE methodology is applied through modeling of the LSST's systems with the System Modeling Language (SysML). The SysML modeling recursively establishes the threefold relationship between requirements, logical & physical functional decomposition and definition, and system and component behavior at successively deeper levels of abstraction and detail. The MBSE approach is applied throughout all stages of the project from design, to validation and verification, though to commissioning.

  9. Lightside Atmospheric Revitalization System

    NASA Technical Reports Server (NTRS)

    Colling, A. K.; Cushman, R. J.; Hultman, M. M.; Nason, J. R.

    1980-01-01

    The system was studied as a replacement to the present baseline LiOH system for extended duration shuttle missions. The system consists of three subsystems: a solid amine water desorbed regenerable carbon dioxide removal system, a water vapor electrolysis oxygen generating system, and a Sabatier reactor carbon dioxide reduction system. The system is designed for use on a solar powered shuttle vehicle. The majority of the system's power requirements are utilized on the Sun side of each orbit, when solar power is available.

  10. Control system design method

    DOEpatents

    Wilson, David G [Tijeras, NM; Robinett, III, Rush D.

    2012-02-21

    A control system design method and concomitant control system comprising representing a physical apparatus to be controlled as a Hamiltonian system, determining elements of the Hamiltonian system representation which are power generators, power dissipators, and power storage devices, analyzing stability and performance of the Hamiltonian system based on the results of the determining step and determining necessary and sufficient conditions for stability of the Hamiltonian system, creating a stable control system based on the results of the analyzing step, and employing the resulting control system to control the physical apparatus.

  11. Umbra's system representation.

    SciTech Connect

    McDonald, Michael James

    2005-07-01

    This document describes the Umbra System representation. Umbra System representation, initially developed in the spring of 2003, is implemented in Incr/Tcl using concepts borrowed from Carnegie Mellon University's Architecture Description Language (ADL) called Acme. In the spring of 2004 through January 2005, System was converted to Umbra 4, extended slightly, and adopted as the underlying software system for a variety of Umbra applications that support Complex Systems Engineering (CSE) and Complex Adaptive Systems Engineering (CASE). System is now a standard part Of Umbra 4. While Umbra 4 also includes an XML parser for System, the XML parser and Schema are not described in this document.

  12. Integrated Systems Health Management for Intelligent Systems

    NASA Technical Reports Server (NTRS)

    Figueroa, Fernando; Melcher, Kevin

    2011-01-01

    The implementation of an integrated system health management (ISHM) capability is fundamentally linked to the management of data, information, and knowledge (DIaK) with the purposeful objective of determining the health of a system. It is akin to having a team of experts who are all individually and collectively observing and analyzing a complex system, and communicating effectively with each other in order to arrive at an accurate and reliable assessment of its health. In this paper, concepts, procedures, and approaches are presented as a foundation for implementing an intelligent systems ]relevant ISHM capability. The capability stresses integration of DIaK from all elements of a system. Both ground-based (remote) and on-board ISHM capabilities are compared and contrasted. The information presented is the result of many years of research, development, and maturation of technologies, and of prototype implementations in operational systems.

  13. Epilogue: Systems Approaches and Systems Practice

    NASA Astrophysics Data System (ADS)

    Reynolds, Martin; Holwell, Sue

    Each of the five systems approaches discussed in this volume: system dynamics (SD), the viable systems model (VSM), strategic options development and analysis (SODA), soft systems methodology (SSM) and critical systems heuristics (CSH) has a pedigree. Not in the sense of the sometimes absurd spectacle of animals paraded at dog shows. Rather, their pedigree derives from their systems foundations, their capacity to evolve and their flexibility in use. None of the five approaches has developed out of use in restricted and controlled contexts of either low or high levels of complicatedness. Neither has any one of them evolved as a consequence of being applied only to situations with either presumed stakeholder agreement on purpose, or courteous disagreement amongst stakeholders, or stakeholder coercion. The compilation is not a celebration of abstract ‘methodologies', but of theoretically robust approaches that have a genuine pedigree in practice.

  14. Systems engineering for very large systems

    NASA Technical Reports Server (NTRS)

    Lewkowicz, Paul E.

    1993-01-01

    Very large integrated systems have always posed special problems for engineers. Whether they are power generation systems, computer networks or space vehicles, whenever there are multiple interfaces, complex technologies or just demanding customers, the challenges are unique. 'Systems engineering' has evolved as a discipline in order to meet these challenges by providing a structured, top-down design and development methodology for the engineer. This paper attempts to define the general class of problems requiring the complete systems engineering treatment and to show how systems engineering can be utilized to improve customer satisfaction and profit ability. Specifically, this work will focus on a design methodology for the largest of systems, not necessarily in terms of physical size, but in terms of complexity and interconnectivity.

  15. Expert system requirements for power system restoration

    SciTech Connect

    Adibi, M.M. ); Kafka, R.J. ); Milanicz, D.P. )

    1994-08-01

    This paper is one of series presented on behalf of the System Operation Subcommittee with the intent of focusing industry attention on power system restoration. Expert systems are being considered for restoring bulk power supplies. In general, there are three restoration periods following a major power disturbance: establishment of initial sources of power, re-integration of a skeleton of the bulk power supply, and minimization of the unserved loads. Expert systems together with analytical tools have the potential of addressing the restoration procedures over these three periods. This paper describes the expert system requirements from the point of view of the practicing power engineers with emphasis placed on the initial power sources and requirements. The paper draws on the previous reports by the Power System Restoration Working Group.

  16. INSENS sensor system

    SciTech Connect

    Myers, D.W.; Baker, J.; Benzel, D.M.; Fuess, D.A.

    1993-09-29

    This paper describes an unattended ground sensor system that has been developed for the immigration and Naturalization Service (INS). The system, known as INSENS, was developed at the Lawrence Livermore National Laboratory for use by the United States Border Patrol. This system assists in the detection of illegal entry of aliens and contraband (illegal drugs, etc.) into the United States along its land borders. Key to the system is its flexible modular design which allows future software and hardware enhancements to the system without altering the fundamental architecture of the system. Elements of the system include a sensor system capable of processing signals from multiple directional probes, a repeater system, and a handheld monitor system. Seismic, passive infrared (PIR), and magnetic probes are currently supported. The design of the INSENS system elements and their performance are described.

  17. Novel central nervous system drug delivery systems.

    PubMed

    Stockwell, Jocelyn; Abdi, Nabiha; Lu, Xiaofan; Maheshwari, Oshin; Taghibiglou, Changiz

    2014-05-01

    For decades, biomedical and pharmaceutical researchers have worked to devise new and more effective therapeutics to treat diseases affecting the central nervous system. The blood-brain barrier effectively protects the brain, but poses a profound challenge to drug delivery across this barrier. Many traditional drugs cannot cross the blood-brain barrier in appreciable concentrations, with less than 1% of most drugs reaching the central nervous system, leading to a lack of available treatments for many central nervous system diseases, such as stroke, neurodegenerative disorders, and brain tumors. Due to the ineffective nature of most treatments for central nervous system disorders, the development of novel drug delivery systems is an area of great interest and active research. Multiple novel strategies show promise for effective central nervous system drug delivery, giving potential for more effective and safer therapies in the future. This review outlines several novel drug delivery techniques, including intranasal drug delivery, nanoparticles, drug modifications, convection-enhanced infusion, and ultrasound-mediated drug delivery. It also assesses possible clinical applications, limitations, and examples of current clinical and preclinical research for each of these drug delivery approaches. Improved central nervous system drug delivery is extremely important and will allow for improved treatment of central nervous system diseases, causing improved therapies for those who are affected by central nervous system diseases.

  18. Immune System as a Sensory System

    PubMed Central

    Dozmorov, Igor M.; Dresser, D.

    2010-01-01

    As suggested by the well-known gestalt concept the immune system can be regarded as an integrated complex system, the functioning of which cannot be fully characterized by the behavior of its constituent elements. Similar approaches to the immune system in particular and sensory systems in general allows one to discern similarities and differences in the process of distinguishing informative patterns in an otherwise random background, thus initiating an appropriate and adequate response. This may lead to a new interpretation of difficulties in the comprehension of some immunological phenomena. PMID:21686066

  19. Hot Spot Removal System: System description

    SciTech Connect

    1997-09-01

    Hazardous wastes contaminated with radionuclides, chemicals, and explosives exist across the Department of Energy complex and need to be remediated due to environmental concerns. Currently, an opportunity is being developed to dramatically reduce remediation costs and to assist in the acceleration of schedules associated with these wastes by deploying a Hot Spot Removal System. Removing the hot spot from the waste site will remove risk driver(s) and enable another, more cost effective process/option/remedial alternative (i.e., capping) to be applied to the remainder of the site. The Hot Spot Removal System consists of a suite of technologies that will be utilized to locate and remove source terms. Components of the system can also be used in a variety of other cleanup activities. This Hot Spot Removal System Description document presents technologies that were considered for possible inclusion in the Hot Spot Removal System, technologies made available to the Hot Spot Removal System, industrial interest in the Hot Spot Removal System`s subsystems, the schedule required for the Hot Spot Removal System, the evaluation of the relevant technologies, and the recommendations for equipment and technologies as stated in the Plan section.

  20. Nonscanning confocal ranging system

    NASA Astrophysics Data System (ADS)

    Sun, P. C.; Arons, E.

    1995-03-01

    We demonstrate a nonscanning confocal ranging system based on spatially incoherent interferometry. Such a system has significant advantages over the conventional confocal imaging system and other interferometric systems. We develop the theory in terms of coherence cells and demonstrate the equivalence of our method to the conventional confocal methods. Experimental results are also provided.

  1. System Engineering Fundamentals

    DTIC Science & Technology

    2001-01-01

    currently valid OMB control number. 1. REPORT DATE JAN 2001 2. REPORT TYPE 3. DATES COVERED 00-00-2001 to 00-00-2001 4. TITLE AND SUBTITLE System...73 PART 3. SYSTEM ANALYSIS AND CONTROL Chapter 9. Work Breakdown Structure...divided into four parts: Introduction; Systems Engineering Process; Systems Analysis and Control ; and Planning, Organizing, and Managing. The first part

  2. Microsphere insulation systems

    NASA Technical Reports Server (NTRS)

    Allen, Mark S. (Inventor); Willen, Gary S. (Inventor); Mohling, Robert A. (Inventor)

    2005-01-01

    A new insulation system is provided that contains microspheres. This insulation system can be used to provide insulated panels and clamshells, and to insulate annular spaces around objects used to transfer, store, or transport cryogens and other temperature-sensitive materials. This insulation system provides better performance with reduced maintenance than current insulation systems.

  3. Medical imaging systems

    DOEpatents

    Frangioni, John V

    2013-06-25

    A medical imaging system provides simultaneous rendering of visible light and diagnostic or functional images. The system may be portable, and may include adapters for connecting various light sources and cameras in open surgical environments or laparascopic or endoscopic environments. A user interface provides control over the functionality of the integrated imaging system. In one embodiment, the system provides a tool for surgical pathology.

  4. Vehicle tracking systems

    SciTech Connect

    Schwalm, R.W.

    1987-01-01

    Several systems have been developed to accomplish vehicle location. The systems consist of three types: Dead Reckoning, Satellite, and LORAN C. If the information is to be sent back to a central location, some type of radiocommunication system is needed. One can use the existing voice radio or add a radio system just for transmitting the data.

  5. Intelligent inspection system

    NASA Astrophysics Data System (ADS)

    May, Jeniece; Dale, Ken; Holloway, Mike; Gaby, Willard

    1997-01-01

    The intelligent inspection system is an advanced controller and analysis system for dimensional measuring machines dedicated to measuring surface of revolution mechanical parts. IIS was developed by the Lockheed Martin Energy Systems, Inc. Oak Ridge Y-12 plant because no commercial product was available to replace the obsolete computing systems on these important machines.

  6. Integrated library systems.

    PubMed Central

    Goldstein, C M

    1983-01-01

    The development of integrated library systems is discussed. The four major discussion points are (1) initial efforts; (2) network resources; (3) minicomputer-based systems; and (4) beyond library automation. Four existing systems are cited as examples of current systems. PMID:6354321

  7. Noncooperative rendezvous radar system

    NASA Technical Reports Server (NTRS)

    1974-01-01

    A fire control radar system was developed, assembled, and modified. The baseline system and modified angle tracking system are described along with the performance characteristics of the baseline and modified systems. Proposed changes to provide additional techniques for radar evaluation are presented along with flight test data.

  8. Universal Index System

    NASA Technical Reports Server (NTRS)

    Kelley, Steve; Roussopoulos, Nick; Sellis, Timos; Wallace, Sarah

    1993-01-01

    The Universal Index System (UIS) is an index management system that uses a uniform interface to solve the heterogeneity problem among database management systems. UIS provides an easy-to-use common interface to access all underlying data, but also allows different underlying database management systems, storage representations, and access methods.

  9. Broad Bandwidth Telecommunications Systems.

    ERIC Educational Resources Information Center

    Sodolski, John

    Broad bandwidth transmission systems have been around for years. They include microwave, assorted cable systems, and recently, satellites. With the exception of some privately owned systems, broadband services have been furnished by the common carriers. Recently, a new element has been added--Cable Antenna Television (CATV) distribution systems.…

  10. Computer Center: CIBE Systems.

    ERIC Educational Resources Information Center

    Crovello, Theodore J.

    1982-01-01

    Differentiates between computer systems and Computers in Biological Education (CIBE) systems (computer system intended for use in biological education). Describes several CIBE stand alone systems: single-user microcomputer; single-user microcomputer/video-disc; multiuser microcomputers; multiuser maxicomputer; and local and long distance computer…

  11. Cogeneration power systems

    NASA Technical Reports Server (NTRS)

    Davis, H. S.

    1978-01-01

    Cogeneration is defined as the combination of electrical generation and process heat for more efficient use of fuel. Comparisons of energy utilization in conventional electric power plants and cogeneration electric power plants are presented. Characteristics of various cogeneration systems are also presented. Systems are analyzed for use in utility systems and industrial systems. Economic and cost analysis are reviewed.

  12. Theory of reliable systems

    NASA Technical Reports Server (NTRS)

    Meyer, J. F.

    1975-01-01

    An attempt was made to refine the current notion of system reliability by identifying and investigating attributes of a system which are important to reliability considerations. Techniques which facilitate analysis of system reliability are included. Special attention was given to fault tolerance, diagnosability, and reconfigurability characteristics of systems.

  13. Program (systems) engineering

    NASA Technical Reports Server (NTRS)

    Baroff, Lynn E.; Easter, Robert W.; Pomphrey, Richard B.

    2004-01-01

    Program Systems Engineering applies the principles of Systems Engineering at the program level. Space programs are composed of interrelated elements which can include collections of projects, advanced technologies, information systems, etc. Some program elements are outside traditional engineering's physical systems, such as education and public outreach, public relations, resource flow, and interactions within the political environments.

  14. Analyzing HVAC piping systems

    SciTech Connect

    Smith, W.W. )

    1993-10-01

    This article describes requirements and considerations for a software tool for analyzing both the hydraulic and heat transfer characteristics of a HVAC system to help in selecting systems components and predicting their performance. The topics of the article include analysis of installed system evolution, selection and analysis of pumps and valves, heat transfer in heating and cooling coils, and capacity to handle large systems.

  15. Image display system 511

    NASA Technical Reports Server (NTRS)

    Gross, M.

    1981-01-01

    The experience of the Idaho Department of Water Resources Remote Sensing Unit in bringing on line their System 511 is described. The system 511 is run on a PDP minicomputer. The minimum system hardware configuration is an 11/34 with a minimum core of 128 K word, 10 megabytes of direct access disk and a floating point processor. The required software configuration is an RSX 11M V 3.2 operating system with a FORTRAN IV plus compiler. The structure of System 511 is a series of hierarchical modular software units. Problems occurring during the systems installation are discussed, and the system operating and error detection capabilities and documentation evaluated.

  16. Lightning mapping system

    NASA Technical Reports Server (NTRS)

    Lennon, C.; Maier, L.

    1991-01-01

    A Lightning Detection and Ranging (LDAR) System is being implemented at KSC in Florida. The first operational use is expected in the late summer of 1991. The system is designed to map the location of in-cloud and cloud-to-ground lightning based on the time of arrival (TOA) of electromagnetic radiation. The system detects very high frequency (VHF) radiation and designed to map the volumetric extent of lightning. The system implements two independent antenna arrays to provide a fast data quality check, as necessary for a real-time warning system. The system performance goals and a comparison with a similar system implemented in the mid-1970's is made.

  17. What is systems engineering?

    SciTech Connect

    Bahill, A.T.

    1995-08-01

    Systems Engineering is an interdisciplinary process that ensures that the customers` needs are satisfied throughout a system`s entire life cycle. This process includes: understanding customer needs; stating the problem; specifying requirements; defining performance and cost measures, prescribing tests, validating requirements, conducting design reviews, exploring alternative concepts, sensitivity analyses, functional decomposition, system design, designing and managing interfaces, system integration, total system test, configuration management, risk management, reliability analysis; total quality management; project management; and documentation. Material for this paper was gathered from senior Systems Engineers at Sandia National Laboratories.

  18. The Shuttle inertial system

    NASA Technical Reports Server (NTRS)

    Swingle, W. L.; Kang, Y.

    1982-01-01

    The Space Shuttle inertial system is built around a sensor assembly called the inertial measurement unit (IMU). The system includes a redundant set of three structurally integrated IMU's that operate in conjunction with parallel strung data system computers to provide precise attitude and velocity information to user system functions. The inertial system is actually a separate subsystem function integrated into the overall avionics system. Software resident in the system computers is the final link in the inertial system. The inertial software is comprised of two major sets, including a subsystem operating program (SOP) called the IMU SOP and redundancy management. Attention is given to system applications, systems performance, attitude sensitivities, the IMU platform, IMU thermal management, aspects of IMU calibration, and Shuttle program experience.

  19. Performance, Performance System, and High Performance System

    ERIC Educational Resources Information Center

    Jang, Hwan Young

    2009-01-01

    This article proposes needed transitions in the field of human performance technology. The following three transitions are discussed: transitioning from training to performance, transitioning from performance to performance system, and transitioning from learning organization to high performance system. A proposed framework that comprises…

  20. Advanced information processing system: Local system services

    NASA Technical Reports Server (NTRS)

    Burkhardt, Laura; Alger, Linda; Whittredge, Roy; Stasiowski, Peter

    1989-01-01

    The Advanced Information Processing System (AIPS) is a multi-computer architecture composed of hardware and software building blocks that can be configured to meet a broad range of application requirements. The hardware building blocks are fault-tolerant, general-purpose computers, fault-and damage-tolerant networks (both computer and input/output), and interfaces between the networks and the computers. The software building blocks are the major software functions: local system services, input/output, system services, inter-computer system services, and the system manager. The foundation of the local system services is an operating system with the functions required for a traditional real-time multi-tasking computer, such as task scheduling, inter-task communication, memory management, interrupt handling, and time maintenance. Resting on this foundation are the redundancy management functions necessary in a redundant computer and the status reporting functions required for an operator interface. The functional requirements, functional design and detailed specifications for all the local system services are documented.

  1. ENVIRONMENTAL SYSTEMS MANAGEMENT AND SUSTAINABLE SYSTEMS THEORY

    EPA Science Inventory

    Environmental Systems Management is the management of environmental problems at the systems level fully accounting for the multi-dimensional nature of the environment. This includes socio-economic dimensions as well as the usual physical and life science aspects. This is importa...

  2. Efficient Evaluation System for Learning Management Systems

    ERIC Educational Resources Information Center

    Cavus, Nadire

    2009-01-01

    A learning management system (LMS) provides the platform for web-based learning environment by enabling the management, delivery, tracking of learning, testing, communication, registration process and scheduling. There are many LMS systems on the market that can be obtained for free or through payment. It has now become an important task to choose…

  3. EPICS system: system structure and user interface

    SciTech Connect

    West, R.E.; Bartlett, J.F.; Bobbitt, J.S.; Lahey, T.E.; Kramper, B.J.; MacKinnon, B.A.

    1984-02-01

    This paper present the user's view of and the general organization of the EPICS control system at Fermilab. Various subsystems of the EPICS control system are discussed. These include the user command language, software protection, the device database, remote computer interfaces, and several application utilities. This paper is related to two other papers on EPICS: an overview paper and a detailed implementation paper.

  4. Formal System Verification for Trustworthy Embedded Systems

    DTIC Science & Technology

    2011-04-19

    Koscher, Alexei Czeskis, Franziska Roesner, Shwetak Patel, Tadayoshi Kohno, Stephen Checkoway, Damon McCoy, Brian Kantor, Danny Anderson, Hovav...security analysis of a commercial real-time operating system kernel. In David S. Hardin , editor, Design and Verification of Microprocessor Systems for High

  5. Clinical application of low-dose phase contrast breast CT: methods for the optimization of the reconstruction workflow.

    PubMed

    Pacilè, S; Brun, F; Dullin, C; Nesterest, Y I; Dreossi, D; Mohammadi, S; Tonutti, M; Stacul, F; Lockie, D; Zanconati, F; Accardo, A; Tromba, G; Gureyev, T E

    2015-08-01

    Results are presented of a feasibility study of three-dimensional X-ray tomographic mammography utilising in-line phase contrast. Experiments were performed at SYRMEP beamline of Elettra synchrotron. A specially designed plastic phantom and a mastectomy sample containing a malignant lesion were used to study the reconstructed image quality as a function of different image processing operations. Detailed evaluation and optimization of image reconstruction workflows have been carried out using combinations of several advanced computed tomography algorithms with different pre-processing and post-processing steps. Special attention was paid to the effect of phase retrieval on the diagnostic value of the reconstructed images. A number of objective image quality indices have been applied for quantitative evaluation of the results, and these were compared with subjective assessments of the same images by three experienced radiologists and one pathologist. The outcomes of this study provide practical guidelines for the optimization of image processing workflows in synchrotron-based phase-contrast mammo-tomography.

  6. Clinical application of low-dose phase contrast breast CT: methods for the optimization of the reconstruction workflow

    PubMed Central

    Pacilè, S.; Brun, F.; Dullin, C.; Nesterest, Y. I.; Dreossi, D.; Mohammadi, S.; Tonutti, M.; Stacul, F.; Lockie, D.; Zanconati, F.; Accardo, A.; Tromba, G.; Gureyev, T. E.

    2015-01-01

    Results are presented of a feasibility study of three-dimensional X-ray tomographic mammography utilising in-line phase contrast. Experiments were performed at SYRMEP beamline of Elettra synchrotron. A specially designed plastic phantom and a mastectomy sample containing a malignant lesion were used to study the reconstructed image quality as a function of different image processing operations. Detailed evaluation and optimization of image reconstruction workflows have been carried out using combinations of several advanced computed tomography algorithms with different pre-processing and post-processing steps. Special attention was paid to the effect of phase retrieval on the diagnostic value of the reconstructed images. A number of objective image quality indices have been applied for quantitative evaluation of the results, and these were compared with subjective assessments of the same images by three experienced radiologists and one pathologist. The outcomes of this study provide practical guidelines for the optimization of image processing workflows in synchrotron-based phase-contrast mammo-tomography. PMID:26309770

  7. SPEMT imaging with a dedicated VAoR dual-head camera: preliminary results

    NASA Astrophysics Data System (ADS)

    Camarda, M.; Belcari, N.; Del Guerra, A.; Vecchio, S.; Bennati, P.; Cinti, M. N.; Pani, R.; Campanini, R.; Iampieri, E.; Lanconelli, N.

    2009-10-01

    We have developed a SPEMT (Single Photon Emission MammoTomography) scanner that is made up of two cameras rotating around the pendulous breast of the prone patient, in Vertical Axis of Rotation (VAoR) geometry. Monte Carlo simulations indicate that the device should be able to detect tumours of 8 mm diameter with a tumour/background uptake ratio of 5:1. The scanner field of view is 41.6 mm height and 147 mm in diameter. Each head is composed of one pixilated NaI(Tl) crystal matrix coupled to three Hamamatsu H8500 64-anodes PMT's read out via resistive networks. A dedicated software has been developed to combine data from different PMT's, thus recovering the dead areas between adjacent tubes. A single head has been fully characterized in stationary configuration both in active and dead areas using a point-like source in order to verify the effectiveness of the readout method in recovering the dead regions. The scanner has been installed at the Nuclear Medicine Division of the University of Pisa for its validation using breast phantoms. The very first tomographic images of a breast phantom show a good agreement with Monte Carlo simulation results.

  8. Rf systems for RHIC

    SciTech Connect

    Rose, J.; Brodowski, J.; Connolly, R.; Deng, D.P.; Kwiatkowski, S.; Pirkl, W.; Ratti, A.

    1995-05-01

    The RHIC rf systems must capture the injected beam, accelerate it through transition to top energy, shorten the bunches prior to rebucketing, and store the beam for 10 hours in the presence of strong intra-beam scattering. These different functions are met by three independent systems. An accelerating system at 26.7 Mhz (h = 342), a storage system at 196.1 MHz (h = 2508), and a wideband system for the damping of injection efforts.

  9. Robotics and expert systems

    SciTech Connect

    Not Available

    1986-01-01

    This volume contains papers presented at ROBEXS' 86, the Second Annual Workshop on Robotics and Expert Systems. Many diverse perspectives on automation problems, and on the merging of robotics and expert systems technology with conventional systems, are contained in this book. The contents include: Integrated Expert Systems Applications; Expert Systems Theory and Applications, Robotics, Intelligent Control, CAD/CAE/CAM, AI Tools, Human Factors, and intelligent Interfaces.

  10. Fluid infusion system

    NASA Technical Reports Server (NTRS)

    1974-01-01

    Performance testing carried out in the development of the prototype zero-g fluid infusion system is described and summarized. Engineering tests were performed in the course of development, both on the original breadboard device and on the prototype system. This testing was aimed at establishing baseline system performance parameters and facilitating improvements. Acceptance testing was then performed on the prototype system to verify functional performance. Acceptance testing included a demonstration of the fluid infusion system on a laboratory animal.

  11. Photovoltaic systems and applications

    SciTech Connect

    Not Available

    1982-01-01

    Abstracts are given of presentations given at a project review meeting held at Albuquerque, NM. The proceedings cover the past accomplishments and current activities of the Photovoltaic Systems Research, Balance-of-System Technology Development and System Application Experiments Projects at Sandia National Laboratories. The status of intermediate system application experiments and residential system analysis is emphasized. Some discussion of the future of the Photovoltaic Program in general, and the Sandia projects in particular is also presented.

  12. Automated Pilot Advisory System

    NASA Technical Reports Server (NTRS)

    Parks, J. L., Jr.; Haidt, J. G.

    1981-01-01

    An Automated Pilot Advisory System (APAS) was developed and operationally tested to demonstrate the concept that low cost automated systems can provide air traffic and aviation weather advisory information at high density uncontrolled airports. The system was designed to enhance the see and be seen rule of flight, and pilots who used the system preferred it over the self announcement system presently used at uncontrolled airports.

  13. Evolvable synthetic neural system

    NASA Technical Reports Server (NTRS)

    Curtis, Steven A. (Inventor)

    2009-01-01

    An evolvable synthetic neural system includes an evolvable neural interface operably coupled to at least one neural basis function. Each neural basis function includes an evolvable neural interface operably coupled to a heuristic neural system to perform high-level functions and an autonomic neural system to perform low-level functions. In some embodiments, the evolvable synthetic neural system is operably coupled to one or more evolvable synthetic neural systems in a hierarchy.

  14. Data Acquisition Systems

    NASA Technical Reports Server (NTRS)

    1989-01-01

    Technology developed during a joint research program with Langley and Kinetic Systems Corporation led to Kinetic Systems' production of a high speed Computer Automated Measurement and Control (CAMAC) data acquisition system. The study, which involved the use of CAMAC equipment applied to flight simulation, significantly improved the company's technical capability and produced new applications. With Digital Equipment Corporation, Kinetic Systems is marketing the system to government and private companies for flight simulation, fusion research, turbine testing, steelmaking, etc.

  15. Solar photovoltaic systems

    NASA Technical Reports Server (NTRS)

    Forney, R. G.

    1978-01-01

    The Department of Energy's photovoltaic program is outlined. The main objective of the program is the development of low cost reliable terrestrial photovoltaic systems. A second objective is to foster widespread use of the system in residential, industrial and commercial application. The system is reviewed by examining each component; silicon solar cell, silicon solar cell modules, advanced development modules and power systems. Cost and applications of the system are discussed.

  16. [Current Status of Genetic Diagnosis of Charcot-Marie-Tooth Disease: Variety of the Disease-causing Genes].

    PubMed

    Hashiguchi, Akihiro; Higuchi, Yujiro; Takashima, Hiroshi

    2016-01-01

    At least 40 genes have been associated with Charcot-Marie-Tooth disease (CMT) and the related inherited neuropathies. Genetic studies have revealed the following factors as causes of inherited neuropathies: myelin components, transcription factors for myelination, myelin maintenance systems, differentiation factors of the peripheral nerve, neurofilaments, protein transfer systems, mitochondrial proteins, DNA repair, RNA/protein synthesis, ion channels, and aminoacyl-tRNA synthetases. Since 2007, we have tried to screen for mutations in CMT patients using microarrays or next generation sequencers. As a result, the detection rate of gene mutations has improved to about 25%. In this study, we applied target resequencing to 72 genes. From the negative examples, we identified the cases based on clinical course, family history, and electrophysiological findings, and then performed exome analysis. We then tried to identify novel causative genes by analyzing the enormous data obtained from our exome analysis.

  17. Multi-static passive SAR imaging based on Bayesian compressive sensing

    NASA Astrophysics Data System (ADS)

    Wu, Qisong; Zhang, Yimin D.; Amin, Moeness G.; Himed, Braham

    2014-05-01

    Passive radar systems, which utilize broadcast and navigation signals as sources of opportunity, have attracted significant interests in recent years due to their low cost, covertness, and the availability of different illuminator sources. In this paper, we propose a novel method for synthetic aperture imaging in multi-static passive radar systems based on a group sparse Bayesian learning technique. In particular, the problem of imaging sparse targets is formulated as a group sparse signal reconstruction problem, which is solved using a complex multi- task Bayesian compressive sensing (CMT-BCS) method to achieve a high resolution. The proposed approach significantly improves the imaging resolution beyond the range resolution. Compared to the other group sparse signal reconstruction methods, such as the block orthogonal matching pursuit (BOMP) and group Lasso, the CMT-BCS provides significant performance improvement for the reconstruction of sparse targets in the redundant dictionary with high coherence. Simulations results demonstrate the superior performance of the proposed approach.

  18. On evolutionary systems.

    PubMed

    Alvarez de Lorenzana, J M; Ward, L M

    1987-01-01

    This paper develops a metatheoretical framework for understanding evolutionary systems (systems that develop in ways that increase their own variety). The framework addresses shortcomings seen in other popular systems theories. It concerns both living and nonliving systems, and proposes a metahierarchy of hierarchical systems. Thus, it potentially addresses systems at all descriptive levels. We restrict our definition of system to that of a core system whose parts have a different ontological status than the system, and characterize the core system in terms of five global properties: minimal length interval, minimal time interval, system cycle, total receptive capacity, and system potential. We propose two principles through the interaction of which evolutionary systems develop. The Principle of Combinatorial Expansion describes how a core system realizes its developmental potential through a process of progressive differentiation of the single primal state up to a limit stage. The Principle of Generative Condensation describes how the components of the last stage of combinatorial expansion condense and become the environment for and components of new, enriched systems. The early evolution of the Universe after the "big bang" is discussed in light of these ideas as an example of the application of the framework.

  19. A System of Systems Approach to the EU Energy System

    NASA Astrophysics Data System (ADS)

    Jess, Tom; Madani, Kaveh; Mahlooji, Maral; Ristic, Bora

    2016-04-01

    Around the world, measures to prevent dangerous climate change are being adopted and may change energy systems fundamentally. The European Union (EU) is committed to reducing greenhouse gas emission by 20% by 2020 and by 80-95% by 2050. In order to achieve this, EU member states aim to increase the share of renewables in the energy mix to 20% by 2020. This commitment comes as part of a series of other aims, principles, and policies to reform the EU's energy system. Cost-efficiency in the emissions reductions measures as well as strategic goals under the Resource Efficient Europe flagship initiative which would include a more prudent approach to other natural resources such as water and land. Using the "System of Systems Approach", as from Hadian and Madani (2015), energy sources' Relative Aggregate Footprints (RAF) in the EU are evaluated. RAF aggregates across four criteria: carbon footprint, water footprint, land footprint, and economic cost. The four criteria are weighted by resource availability across the EU and for each Member State. This provides an evaluation of the overall resource use efficiency of the EU's energy portfolio and gives insight into the differences in the desirability of energy sources across Member States. Broadly, nuclear, onshore wind, and geothermal are most desirable under equal criteria weights and EU average weighting introduces only small changes in the relative performance of only few technologies. The member state specific weightings show that most countries have similar energy technology preferences. However, the UK deviates most strongly from the average, with an even stronger preference for nuclear and coal. Sweden, Malta and Finland also deviate from the typical preferences indicating the complexity in play in reforming the EU energy system. Reference Hadian S, Madani K (2015) A System of Systems Approach to Energy Sustainability Assessment: Are All Renewables Really Green? Ecological Indicators, 52, 194-206.

  20. Intelligent systems technology infrastructure for integrated systems

    NASA Technical Reports Server (NTRS)

    Lum, Henry

    1991-01-01

    A system infrastructure must be properly designed and integrated from the conceptual development phase to accommodate evolutionary intelligent technologies. Several technology development activities were identified that may have application to rendezvous and capture systems. Optical correlators in conjunction with fuzzy logic control might be used for the identification, tracking, and capture of either cooperative or non-cooperative targets without the intensive computational requirements associated with vision processing. A hybrid digital/analog system was developed and tested with a robotic arm. An aircraft refueling application demonstration is planned within two years. Initially this demonstration will be ground based with a follow-on air based demonstration. System dependability measurement and modeling techniques are being developed for fault management applications. This involves usage of incremental solution/evaluation techniques and modularized systems to facilitate reuse and to take advantage of natural partitions in system models. Though not yet commercially available and currently subject to accuracy limitations, technology is being developed to perform optical matrix operations to enhance computational speed. Optical terrain recognition using camera image sequencing processed with optical correlators is being developed to determine position and velocity in support of lander guidance. The system is planned for testing in conjunction with Dryden Flight Research Facility. Advanced architecture technology is defining open architecture design constraints, test bed concepts (processors, multiple hardware/software and multi-dimensional user support, knowledge/tool sharing infrastructure), and software engineering interface issues.

  1. Modeling Power Systems as Complex Adaptive Systems

    SciTech Connect

    Chassin, David P.; Malard, Joel M.; Posse, Christian; Gangopadhyaya, Asim; Lu, Ning; Katipamula, Srinivas; Mallow, J V.

    2004-12-30

    Physical analogs have shown considerable promise for understanding the behavior of complex adaptive systems, including macroeconomics, biological systems, social networks, and electric power markets. Many of today's most challenging technical and policy questions can be reduced to a distributed economic control problem. Indeed, economically based control of large-scale systems is founded on the conjecture that the price-based regulation (e.g., auctions, markets) results in an optimal allocation of resources and emergent optimal system control. This report explores the state-of-the-art physical analogs for understanding the behavior of some econophysical systems and deriving stable and robust control strategies for using them. We review and discuss applications of some analytic methods based on a thermodynamic metaphor, according to which the interplay between system entropy and conservation laws gives rise to intuitive and governing global properties of complex systems that cannot be otherwise understood. We apply these methods to the question of how power markets can be expected to behave under a variety of conditions.

  2. Alkaline etch system qualification

    SciTech Connect

    Goldammer, S.E.; Pemberton, S.E.; Tucker, D.R.

    1997-04-01

    Based on the data from this qualification activity, the Atotech etch system, even with minimum characterization, was capable of etching production printed circuit products as good as those from the Chemcut system. Further characterization of the Atotech system will improve its etching capability. In addition to the improved etch quality expected from further characterization, the Atotech etch system has additional features that help reduce waste and provide for better consistency in the etching process. The programmable logic controller and computer will allow operators to operate the system manually or from pre-established recipes. The evidence and capabilities of the Atotech system made it as good as or better than the Chemcut system for etching WR products. The Printed Wiring Board Engineering Department recommended that the Atotech system be released for production. In December 1995, the Atotech system was formerly qualified for production.

  3. NASA systems engineering handbook

    NASA Astrophysics Data System (ADS)

    Shishko, Robert; Aster, Robert; Chamberlain, Robert G.; McDuffee, Patrick; Pieniazek, Les; Rowell, Tom; Bain, Beth; Cox, Renee I.; Mooz, Harold; Polaski, Lou

    1995-06-01

    This handbook brings the fundamental concepts and techniques of systems engineering to NASA personnel in a way that recognizes the nature of NASA systems and environment. It is intended to accompany formal NASA training courses on systems engineering and project management when appropriate, and is designed to be a top-level overview. The concepts were drawn from NASA field center handbooks, NMI's/NHB's, the work of the NASA-wide Systems Engineering Working Group and the Systems Engineering Process Improvement Task team, several non-NASA textbooks and guides, and material from independent systems engineering courses taught to NASA personnel. Five core chapters cover systems engineering fundamentals, the NASA Project Cycle, management issues in systems engineering, systems analysis and modeling, and specialty engineering integration. It is not intended as a directive.

  4. Rover waste assay system

    SciTech Connect

    Akers, D.W.; Stoots, C.M.; Kraft, N.C.; Marts, D.J.

    1997-11-01

    The Rover Waste Assay System (RWAS) is a nondestructive assay system designed for the rapid assay of highly-enriched {sup 235}U contaminated piping, tank sections, and debris from the Rover nuclear rocket fuel processing facility at the Idaho Chemical Processing Plant. A scanning system translates a NaI(Tl) detector/collimator system over the structural components where both relative and calibrated measurements for {sup 137}Cs are made. Uranium-235 concentrations are in operation and is sufficiently automated that most functions are performed by the computer system. These functions include system calibration, problem identification, collimator control, data analysis, and reporting. Calibration of the system was done through a combination of measurements on calibration standards and benchmarked modeling. A description of the system is presented along with the methods and uncertainties associated with the calibration and analysis of the system for components from the Rover facility. 4 refs., 2 figs., 4 tabs.

  5. NASA Systems Engineering Handbook

    NASA Technical Reports Server (NTRS)

    Shishko, Robert; Aster, Robert; Chamberlain, Robert G.; Mcduffee, Patrick; Pieniazek, Les; Rowell, Tom; Bain, Beth; Cox, Renee I.; Mooz, Harold; Polaski, Lou

    1995-01-01

    This handbook brings the fundamental concepts and techniques of systems engineering to NASA personnel in a way that recognizes the nature of NASA systems and environment. It is intended to accompany formal NASA training courses on systems engineering and project management when appropriate, and is designed to be a top-level overview. The concepts were drawn from NASA field center handbooks, NMI's/NHB's, the work of the NASA-wide Systems Engineering Working Group and the Systems Engineering Process Improvement Task team, several non-NASA textbooks and guides, and material from independent systems engineering courses taught to NASA personnel. Five core chapters cover systems engineering fundamentals, the NASA Project Cycle, management issues in systems engineering, systems analysis and modeling, and specialty engineering integration. It is not intended as a directive. Superseded by: NASA/SP-2007-6105 Rev 1 (20080008301).

  6. Enhanced nurse call systems.

    PubMed

    2001-04-01

    This Evaluation focuses on high-end computerized nurse call systems--what we call enhanced systems. These are highly flexible systems that incorporate microprocessor and communications technologies to expand the capabilities of the nurse call function. Enhanced systems, which vary in configuration from one installation to the next, typically consist of a basic system that provides standard nurse call functionality and a combination of additional enhancements that provide the added functionality the facility desires. In this study, we examine the features that distinguish enhanced nurse call systems from nonenhanced systems, focusing on their application and benefit to healthcare facilities. We evaluated seven systems to determine how well they help (1) improve patient care, as well as increase satisfaction with the care provided, and (2) improve caregiver efficiency, as well as increase satisfaction with the work environment. We found that all systems meet these objectives, but not all systems perform equally well for all implementations. Our ratings will help facilities identify those systems that offer the most effective features for their intended use. The study also includes a Technology Management Guide to help readers (1) determine whether they'll benefit from the capabilities offered by enhanced systems and (2) target a system for purchase and equip the system for optimum performance and cost-effective operation.

  7. Open systems storage platforms

    NASA Technical Reports Server (NTRS)

    Collins, Kirby

    1992-01-01

    The building blocks for an open storage system includes a system platform, a selection of storage devices and interfaces, system software, and storage applications CONVEX storage systems are based on the DS Series Data Server systems. These systems are a variant of the C3200 supercomputer with expanded I/O capabilities. These systems support a variety of medium and high speed interfaces to networks and peripherals. System software is provided in the form of ConvexOS, a POSIX compliant derivative of 4.3BSD UNIX. Storage applications include products such as UNITREE and EMASS. With the DS Series of storage systems, Convex has developed a set of products which provide open system solutions for storage management applications. The systems are highly modular, assembled from off the shelf components with industry standard interfaces. The C Series system architecture provides a stable base, with the performance and reliability of a general purpose platform. This combination of a proven system architecture with a variety of choices in peripherals and application software allows wide flexibility in configurations, and delivers the benefits of open systems to the mass storage world.

  8. Space shuttle avionics system

    NASA Technical Reports Server (NTRS)

    Hanaway, John F.; Moorehead, Robert W.

    1989-01-01

    The Space Shuttle avionics system, which was conceived in the early 1970's and became operational in the 1980's represents a significant advancement of avionics system technology in the areas of systems and redundacy management, digital data base technology, flight software, flight control integration, digital fly-by-wire technology, crew display interface, and operational concepts. The origins and the evolution of the system are traced; the requirements, the constraints, and other factors which led to the final configuration are outlined; and the functional operation of the system is described. An overall system block diagram is included.

  9. Introducing Systems Approaches

    NASA Astrophysics Data System (ADS)

    Reynolds, Martin; Holwell, Sue

    Systems Approaches to Managing Change brings together five systems approaches to managing complex issues, each having a proven track record of over 25 years. The five approaches are: System Dynamics (SD) developed originally in the late 1950s by Jay Forrester Viable Systems Model (VSM) developed originally in the late 1960s by Stafford Beer Strategic Options Development and Analysis (SODA: with cognitive mapping) developed originally in the 1970s by Colin Eden Soft Systems Methodology (SSM) developed originally in the 1970s by Peter Checkland Critical Systems Heuristics (CSH) developed originally in the late 1970s by Werner Ulrich

  10. Flash evaporator systems test

    NASA Technical Reports Server (NTRS)

    Dietz, J. B.

    1976-01-01

    A flash evaporator heat rejection system representative of that proposed for the space shuttle orbiter underwent extensive system testing at the NASA Johnson Space Center (JSC) to determine its operational suitability and to establish system performance/operational characteristics for use in the shuttle system. During the tests the evaporator system demonstrated its suitability to meet the shuttle requirements by: (1) efficient operation with 90 to 95% water evaporation efficiency, (2) control of outlet temperature to 40 + or - 2 F for partial heat load operation, (3) stability of control system for rapid changes in Freon inlet temperature, and (4) repeated dormant-to-active device operation without any startup procedures.

  11. Data Acquisition Systems

    NASA Technical Reports Server (NTRS)

    1994-01-01

    In the mid-1980s, Kinetic Systems and Langley Research Center determined that high speed CAMAC (Computer Automated Measurement and Control) data acquisition systems could significantly improve Langley's ARTS (Advanced Real Time Simulation) system. The ARTS system supports flight simulation R&D, and the CAMAC equipment allowed 32 high performance simulators to be controlled by centrally located host computers. This technology broadened Kinetic Systems' capabilities and led to several commercial applications. One of them is General Atomics' fusion research program. Kinetic Systems equipment allows tokamak data to be acquired four to 15 times more rapidly. Ford Motor company uses the same technology to control and monitor transmission testing facilities.

  12. MLS: Airplane system modeling

    NASA Technical Reports Server (NTRS)

    Thompson, A. D.; Stapleton, B. P.; Walen, D. B.; Rieder, P. F.; Moss, D. G.

    1981-01-01

    Analysis, modeling, and simulations were conducted as part of a multiyear investigation of the more important airplane-system-related items of the microwave landing system (MLS). Particular emphasis was placed upon the airplane RF system, including the antenna radiation distribution, the cabling options from the antenna to the receiver, and the overall impact of the airborne system gains and losses upon the direct-path signal structure. In addition, effort was expended toward determining the impact of the MLS upon the airplane flight management system and developing the initial stages of a fast-time MLS automatic control system simulation model. Results ot these studies are presented.

  13. Cooperating systems: Layered MAS

    NASA Technical Reports Server (NTRS)

    Rochowiak, Daniel

    1990-01-01

    Distributed intelligent systems can be distinguished by the models that they use. The model developed focuses on layered multiagent system conceived of as a bureaucracy in which a distributed data base serves as a central means of communication. The various generic bureaus of such a system is described and a basic vocabulary for such systems is presented. In presenting the bureaus and vocabularies, special attention is given to the sorts of reasonings that are appropriate. A bureaucratic model has a hierarchy of master system and work group that organizes E agents and B agents. The master system provides the administrative services and support facilities for the work groups.

  14. Continuous system modeling

    NASA Technical Reports Server (NTRS)

    Cellier, Francois E.

    1991-01-01

    A comprehensive and systematic introduction is presented for the concepts associated with 'modeling', involving the transition from a physical system down to an abstract description of that system in the form of a set of differential and/or difference equations, and basing its treatment of modeling on the mathematics of dynamical systems. Attention is given to the principles of passive electrical circuit modeling, planar mechanical systems modeling, hierarchical modular modeling of continuous systems, and bond-graph modeling. Also discussed are modeling in equilibrium thermodynamics, population dynamics, and system dynamics, inductive reasoning, artificial neural networks, and automated model synthesis.

  15. Fundamentals of systems ergonomics.

    PubMed

    Wilson, John R

    2012-01-01

    Ergonomics/human factors is, above anything else, a systems discipline applying a systems philosophy and systems approaches. Many things are labeled as "systems X" in today's world, and this paper specifies just what attributes and notions define ergonomics/human factors in systems terms. These are concern for context, acknowledgement of interactions and complexity, a holistic approach, recognition of emergence and embedding of the professional effort involved within organization systems. These five notions are illustrated with examples from a large body of work on rail human factors.

  16. Business System Planning Project System Requirements Specification

    SciTech Connect

    NELSON, R.E.

    2000-09-08

    The purpose of the Business Systems Planning Project System Requirements Specification (SRS) is to provide the outline and contents of the requirements for the CH2M HILL Hanford Group, Inc. (CHG) integrated business and technical information systems. The SRS will translate proposed objectives into the statement of the functions that are to be performed and data and information flows that they require. The requirements gathering methodology will use (1) facilitated group requirement sessions; (2) individual interviews; (3) surveys; and (4) document reviews. The requirements will be verified and validated through coordination of the technical requirement team and CHG Managers. The SRS document used the content and format specified in Lockheed Martin Services, Inc. Organization Standard Software Practices in conjunction with the Institute of Electrical and Electronics Engineers Standard 8340-1984 for Systems Requirements Documents.

  17. NASA's SPICE System Models the Solar System

    NASA Technical Reports Server (NTRS)

    Acton, Charles

    1996-01-01

    SPICE is NASA's multimission, multidiscipline information system for assembling, distributing, archiving, and accessing space science geometry and related data used by scientists and engineers for mission design and mission evaluation, detailed observation planning, mission operations, and science data analysis.

  18. Power system interface and umbilical system study

    NASA Technical Reports Server (NTRS)

    1980-01-01

    System requirements and basic design criteria were defined for berthing or docking a payload to the 25 kW power module which will provide electrical power and attitude control, cooling, data transfer, and communication services to free-flying and Orbiter sortie payloads. The selected umbilical system concept consists of four assemblies and command and display equipment to be installed at the Orbiter payload specialist station: (1) a movable platen assembly which is attached to the power system with EVA operable devices; (2) a slave platen assembly which is attached to the payload with EVA operable devices; (3) a fixed secondary platen permanently installed in the power system; and (4) a fixed secondary platen permanently installed on the payload. Operating modes and sequences are described.

  19. Unmanned Aerial System, New System Manning Prediction

    DTIC Science & Technology

    2006-01-01

    during the same mission, the area under surveillance is subject to a chemical attack during a period of inclement weather, say low-level fog or rain...these potential fog -of-war situations occur that systems design, particularly manning, must be able to cope with the imposed load. This concept is...of some of the data, that is, because of the dispersion in manning values for the five systems chosen, a rank-based regression would provide a more

  20. Combat Systems Department Employee Recognition System

    DTIC Science & Technology

    1996-08-01

    the individual’s view of positive reinforcement . Include them in discussions. Ask for their opinions. 4 NSWCDD/MP-96/137 SECTION 3 INSTRUCTIONS 3.1...PROVIDES POSITIVE REINFORCEMENT . THE EASIER IT IS TO DO, THE MORE LIKELY IT IS TO GET DONE. N-DEPARTMENT EMPLOYEE RECOGNITION SYSTEM PRI NCI PLES THERE ARE...INDIVIDUAL’S VIEW OF POSITIVE REINFORCEMENT . ASK THEM I Papa .18Iv 15 N-DEPARTMENT EMPLOYEE RECOGNITION SYSTEM * OUTLINE A. TASK FORCE MEMBERSHIP

  1. Integrated Systems Health Management for Intelligent Systems

    NASA Technical Reports Server (NTRS)

    Figueroa, Fernando; Melcher, Kevin

    2011-01-01

    The implementation of an integrated system health management (ISHM) capability is fundamentally linked to the management of data, information, and knowledge (DIaK) with the purposeful objective of determining the health of a system. Management implies storage, distribution, sharing, maintenance, processing, reasoning, and presentation. ISHM is akin to having a team of experts who are all individually and collectively observing and analyzing a complex system, and communicating effectively with each other in order to arrive at an accurate and reliable assessment of its health. In this chapter, concepts, procedures, and approaches are presented as a foundation for implementing an ISHM capability relevant to intelligent systems. The capability stresses integration of DIaK from all elements of a system, emphasizing an advance toward an on-board, autonomous capability. Both ground-based and on-board ISHM capabilities are addressed. The information presented is the result of many years of research, development, and maturation of technologies, and of prototype implementations in operational systems.

  2. System and method for creating expert systems

    NASA Technical Reports Server (NTRS)

    Hughes, Peter M. (Inventor); Luczak, Edward C. (Inventor)

    1998-01-01

    A system and method provides for the creation of a highly graphical expert system without the need for programming in code. An expert system is created by initially building a data interface, defining appropriate Mission, User-Defined, Inferred, and externally-generated GenSAA (EGG) data variables whose data values will be updated and input into the expert system. Next, rules of the expert system are created by building appropriate conditions of the rules which must be satisfied and then by building appropriate actions of rules which are to be executed upon corresponding conditions being satisfied. Finally, an appropriate user interface is built which can be highly graphical in nature and which can include appropriate message display and/or modification of display characteristics of a graphical display object, to visually alert a user of the expert system of varying data values, upon conditions of a created rule being satisfied. The data interface building, rule building, and user interface building are done in an efficient manner and can be created without the need for programming in code.

  3. Absorption heat pump system

    DOEpatents

    Grossman, G.

    1982-06-16

    The efficiency of an absorption heat pump system is improved by conducting liquid from a second stage evaporator thereof to an auxiliary heat exchanger positioned downstream of a primary heat exchanger in the desorber of the system.

  4. Intensive care alarm system

    NASA Technical Reports Server (NTRS)

    Christensen, J. L.; Herbert, A. L.

    1973-01-01

    Inductive loop has been added to commercially available call system fitted with earphone receiver. System transmits high frequency signals to nurse's receiver to announce patient's need for help without disturbing others.

  5. Data-Retrieval System

    NASA Technical Reports Server (NTRS)

    Lawrence, P. R.

    1982-01-01

    NASA Graphics and Display System (NGDS) helps solve problem of data retrieval for management. Since NGDS is interactive online system, user controls display content and format, and has capability of always working with latest version of his data.

  6. Semiotic labelled deductive systems

    SciTech Connect

    Nossum, R.T.

    1996-12-31

    We review the class of Semiotic Models put forward by Pospelov, as well as the Labelled Deductive Systems developed by Gabbay, and construct an embedding of Semiotic Models into Labelled Deductive Systems.

  7. Septic Systems Case Studies

    EPA Pesticide Factsheets

    A collection of septic systems case studies to help community planners, elected officials, health department staff, state officials, and interested citizens explore alternatives for managing their decentralized wastewater treatment systems.

  8. Central nervous system

    MedlinePlus

    The central nervous system is composed of the brain and spinal cord. Your brain and spinal cord serve as the main "processing center" for your entire nervous system. They control all the workings of your body.

  9. Information retrieval system

    NASA Technical Reports Server (NTRS)

    Berg, R. F.; Holcomb, J. E.; Kelroy, E. A.; Levine, D. A.; Mee, C., III

    1970-01-01

    Generalized information storage and retrieval system capable of generating and maintaining a file, gathering statistics, sorting output, and generating final reports for output is reviewed. File generation and file maintenance programs written for the system are general purpose routines.

  10. Male Reproductive System

    MedlinePlus

    ... gamete, the egg or ovum, meet in the female's reproductive system to create a baby. Both the male and female reproductive systems are essential for reproduction. Humans pass certain characteristics ...

  11. Modular optical detector system

    DOEpatents

    Horn, Brent A.; Renzi, Ronald F.

    2006-02-14

    A modular optical detector system. The detector system is designed to detect the presence of molecules or molecular species by inducing fluorescence with exciting radiation and detecting the emitted fluorescence. Because the system is capable of accurately detecting and measuring picomolar concentrations it is ideally suited for use with microchemical analysis systems generally and capillary chromatographic systems in particular. By employing a modular design, the detector system provides both the ability to replace various elements of the detector system without requiring extensive realignment or recalibration of the components as well as minimal user interaction with the system. In addition, the modular concept provides for the use and addition of a wide variety of components, including optical elements (lenses and filters), light sources, and detection means, to fit particular needs.

  12. Test processing system (SEE)

    NASA Technical Reports Server (NTRS)

    Gaulene, P.

    1986-01-01

    The SEE data processing system, developed in 1985, manages and process test results. General information is provided on the SEE system: objectives, characteristics, basic principles, general organization, and operation. Full documentation is accessible by computer using the HELP SEE command.

  13. Double Degenerate Binary Systems

    SciTech Connect

    Yakut, K.

    2011-09-21

    In this study, angular momentum loss via gravitational radiation in double degenerate binary (DDB)systems (NS + NS, NS + WD, WD + WD, and AM CVn) is studied. Energy loss by gravitational waves has been estimated for each type of systems.

  14. Instructional Systems: Which One?

    ERIC Educational Resources Information Center

    Lee, A. Maughan

    1970-01-01

    Discusses the mystical aura attached to the notion of a systems approach to instruction, briefly reviews some of the instructional systems which have been advanced, and suggests a method for successful implementation of the process. (LS)

  15. OPTIMUM SYSTEMS CONTROL,

    DTIC Science & Technology

    Variational calculus and continuous optimal control, (4) The maximum principle and Hamilton Jacobi theory, (5) Optimum systems control examples, (6...Discrete variational calculus and the discrete maximum principle, (7) Optimum control of distributed parameter systems, (8) Optimum state estimation in

  16. Pneumonia - weakened immune system

    MedlinePlus

    ... treatments to remove fluid and mucus from the respiratory system are often needed. Outlook (Prognosis) Factors that may ... immunocompromised host Images Pneumococci organism Lungs The lungs Respiratory system References Donnelly JP, Blijlevens NMA, van der Velden ...

  17. Avian respiratory system disorders

    USGS Publications Warehouse

    Olsen, G.H.

    1989-01-01

    Diagnosing and treating respiratory diseases in avian species requires a basic knowledge about the anatomy and physiology of this system in birds. Differences between mammalian and avian respiratory system function, diagnosis, and treatment are highlighted.

  18. Advanced dive monitoring system.

    PubMed

    Sternberger, W I; Goemmer, S A

    1999-01-01

    The US Navy supports deep diving operations with a variety of mixed-gas life support systems. A systems engineering study was conducted for the Naval Experimental Dive Unit (Panama City, FL) to develop a concept design for an advanced dive monitoring system. The monitoring system is intended primarily to enhance diver safety and secondarily to support diving medicine research. Distinct monitoring categories of diver physiology, life support system, and environment are integrated in the monitoring system. A system concept is proposed that accommodates real-time and quantitative measurements, noninvasive physiological monitoring, and a flexible and expandable implementation architecture. Human factors and ergonomic design considerations have been emphasized to assure that there is no impact on the diver's primary mission. The Navy has accepted the resultant system requirements and the basic design concept. A number of monitoring components have been implemented and successfully support deep diving operations.

  19. Immune System and Disorders

    MedlinePlus

    Your immune system is a complex network of cells, tissues, and organs that work together to defend against germs. It ... t, to find and destroy them. If your immune system cannot do its job, the results can be ...

  20. System status display evaluation

    NASA Technical Reports Server (NTRS)

    Summers, Leland G.

    1988-01-01

    The System Status Display is an electronic display system which provides the crew with an enhanced capability for monitoring and managing the aircraft systems. A flight simulation in a fixed base cockpit simulator was used to evaluate alternative design concepts for this display system. The alternative concepts included pictorial versus alphanumeric text formats, multifunction versus dedicated controls, and integration of the procedures with the system status information versus paper checklists. Twelve pilots manually flew approach patterns with the different concepts. System malfunctions occurred which required the pilots to respond to the alert by reconfiguring the system. The pictorial display, the multifunction control interfaces collocated with the system display, and the procedures integrated with the status information all had shorter event processing times and lower subjective workloads.

  1. Absorption heat pump system

    DOEpatents

    Grossman, Gershon

    1984-01-01

    The efficiency of an absorption heat pump system is improved by conducting liquid from a second stage evaporator thereof to an auxiliary heat exchanger positioned downstream of a primary heat exchanger in the desorber of the system.

  2. Female Reproductive System

    MedlinePlus

    ... Ectopic Pregnancy A Week-by-Week Pregnancy Calendar Male Reproductive System Five Things Girls Want to Know About Periods ... Coping With Common Period Problems All About Menstruation Male Reproductive System Everything You Wanted to Know About Puberty Polycystic ...

  3. Renewable Energy Tracking Systems

    EPA Pesticide Factsheets

    Renewable energy generation ownership can be accounted through tracking systems. Tracking systems are highly automated, contain specific information about each MWh, and are accessible over the internet to market participants.

  4. Leasing Residential PV Systems

    SciTech Connect

    Rutberg, Michael; Bouza, Antonio

    2013-11-01

    The article discusses the adoption, consequences and current market status of the leasing of residential photovoltaic systems. It addresses attributed energy savings and market potential of residential system leasing.

  5. Henry Ford Health Systems

    Cancer.gov

    Henry Ford Health Systems evolved from a hospital into a system delivering care to 2.5 million patients and includes the Cancer Epidemiology, Prevention and Control Program, which focuses on epidemiologic and public health aspects of cancer.

  6. INL Autonomous Navigation System

    SciTech Connect

    2005-03-30

    The INL Autonomous Navigation System provides instructions for autonomously navigating a robot. The system permits high-speed autonomous navigation including obstacle avoidance, waypoing navigation and path planning in both indoor and outdoor environments.

  7. Drinking Water Distribution Systems

    EPA Pesticide Factsheets

    Learn about an overview of drinking water distribution systems, the factors that degrade water quality in the distribution system, assessments of risk, future research about these risks, and how to reduce cross-connection control risk.

  8. Silver recovery system data

    SciTech Connect

    Boulineau, B.

    1991-08-26

    In August of 1990 the Savannah River Site Photography Group began testing on a different type of silver recovery system. This paper describes the baseline study and the different phases of installation and testing of the system.

  9. Designing automatic resupply systems.

    PubMed

    Harding, M L

    1999-02-01

    This article outlines the process for designing and implementing autoresupply systems. The planning process includes determination of goals and appropriate participation. Different types of autoresupply mechanisms include kanban, breadman, consignment, systems contracts, and direct shipping from an MRP schedule.

  10. Validation of multiprocessor systems

    NASA Technical Reports Server (NTRS)

    Siewiorek, D. P.; Segall, Z.; Kong, T.

    1982-01-01

    Experiments that can be used to validate fault free performance of multiprocessor systems in aerospace systems integrating flight controls and avionics are discussed. Engineering prototypes for two fault tolerant multiprocessors are tested.

  11. Multiplex television transmission system

    NASA Technical Reports Server (NTRS)

    Reed, W. R.

    1967-01-01

    Time-multiplexing system enables several cameras to share a single commercial television transmission channel. This system is useful in industries for visually monitoring several operating areas or instrument panels from a remote location.

  12. Alarm Notification System

    SciTech Connect

    1995-03-12

    AN/EMS, the Alarm Notification Energy Management System, is used to monitor digital sensors in PETC buildings and to notify the safety/security operator by both a video and an audio system when a possibly hazardous condition arises.

  13. Nonsurvivable momentum exchange system

    NASA Technical Reports Server (NTRS)

    Roder, Russell (Inventor); Ahronovich, Eliezer (Inventor); Davis, III, Milton C. (Inventor)

    2007-01-01

    A demiseable momentum exchange system includes a base and a flywheel rotatably supported on the base. The flywheel includes a web portion defining a plurality of web openings and a rim portion. The momentum exchange system further includes a motor for driving the flywheel and a cover for engaging the base to substantially enclose the flywheel. The system may also include components having a melting temperature below 1500 degrees Celsius. The momentum exchange system is configured to demise on reentry.

  14. Propulsion system needs

    NASA Technical Reports Server (NTRS)

    Gunn, Stanley

    1991-01-01

    The needs of the designer of a solid core nuclear rocket engine are discussed. Some of the topics covered include: (1) a flight thrust module/feed system module assembly; (2) a nuclear thermal rocket (NTR), expander cycle, dual T/P; (3) turbopump operating conditions; (4) typical system parameters; (5) growth capability composite fuel elements; (6) a NTR radiation cooled nozzle extension; (7) a NFS-3B Feed System; and (8) a NTR Integrated Pneumatic-Fluidics Control System.

  15. Immersive Visual Programming System

    DTIC Science & Technology

    1995-03-16

    improved virtual programming system. 20 It is another object to provide a virtual programming system that optimally increases a user’s comprehensibility...of program structures. NAVY CASE NO. 75554 It is still another object to provide a virtual programming system that is useful for defining the high...level flow control and data pathways within a program. It is yet another object to provide a virtual programming system that allows for dynamic

  16. Space power systems technology

    NASA Technical Reports Server (NTRS)

    Coulman, George A.

    1994-01-01

    Reported here is a series of studies which examine several potential catalysts and electrodes for some fuel cell systems, some materials for space applications, and mathematical modeling and performance predictions for some solid oxide fuel cells and electrolyzers. The fuel cell systems have a potential for terrestrial applications in addition to solar energy conversion in space applications. Catalysts and electrodes for phosphoric acid fuel cell systems and for polymer electrolyte membrane (PEM) fuel cell and electrolyzer systems were examined.

  17. Linear system theory

    NASA Technical Reports Server (NTRS)

    Callier, Frank M.; Desoer, Charles A.

    1991-01-01

    The aim of this book is to provide a systematic and rigorous access to the main topics of linear state-space system theory in both the continuous-time case and the discrete-time case; and the I/O description of linear systems. The main thrusts of the work are the analysis of system descriptions and derivations of their properties, LQ-optimal control, state feedback and state estimation, and MIMO unity-feedback systems.

  18. Visual Alert System

    NASA Technical Reports Server (NTRS)

    1985-01-01

    A visual alert system resulted from circuitry developed by Applied Cybernetics Systems for Langley as part of a space related telemetry system. James Campman, Applied Cybernetics president, left the company and founded Grace Industries, Inc. to manufacture security devices based on the Langley technology. His visual alert system combines visual and audible alerts for hearing impaired people. The company also manufactures an arson detection device called the electronic nose, and is currently researching additional applications of the NASA technology.

  19. Thermochemical energy systems research

    NASA Astrophysics Data System (ADS)

    Nix, R. G.

    1983-08-01

    Research on Heat-pump thermochemical energy systems and thermochemical reduction of CO2 to CO for open-loop solar energy transport is described. Analysis of the NaOH-H2O heat-pumped system indicted cost effectiveness relative to hot oil solar system with parabolic trough receivers for production of 0.101 MPa saturated steam high-temperature heat-pumped systems are being defined.

  20. Portable Dental System

    NASA Technical Reports Server (NTRS)

    1980-01-01

    Portable dental system provides dental care in isolated communities. System includes a patient's chair and a dentist's stool, an X-ray machine and a power unit, all of which fold into compact packages. A large yellow "pumpkin" is a collapsible compressed air tank. Portable system has been used successfully in South America in out of the way communities with this back-packable system, and in American nursing homes. This product is no longer manufactured.

  1. The DISCUS Hardware System,

    DTIC Science & Technology

    1982-07-01

    it physically impossible for one processor to access another’s local program or workspace. It should be noted that the system - generator should handle...this gateway would be complex and how it would be used and linked to the system generator would also be difficult. Because of this it worth asking...which is by no means trivial to write, identified several faults in the system generator and operating system that would have otherwise been

  2. Intermediate water recovery system

    NASA Technical Reports Server (NTRS)

    Deckman, G.; Anderson, A. R. (Editor)

    1973-01-01

    A water recovery system for collecting, storing, and processing urine, wash water, and humidity condensates from a crew of three aboard a spacecraft is described. The results of a 30-day test performed on a breadboard system are presented. The intermediate water recovery system produced clear, sterile, water with a 96.4 percent recovery rate from the processed urine. Recommendations for improving the system are included.

  3. Goddard Ground System Environment

    NASA Technical Reports Server (NTRS)

    Liu, Ben

    2009-01-01

    This slide presentation reviews the Goddard Mission Services Evolution Center's work in providing the Ground System Infrastructure to allow for standard interfaces, and allow for a mix of heritage and new components. This software has been used by NASA and other Government users. Telemetry and command services are also provided as are mission planning and scheduling systems. Other areas that the presentation covers are work on trending systems, and data management system.

  4. Automated RTOP Management System

    NASA Technical Reports Server (NTRS)

    Hayes, P.

    1984-01-01

    The structure of NASA's Office of Aeronautics and Space Technology electronic information system network from 1983 to 1985 is illustrated. The RTOP automated system takes advantage of existing hardware, software, and expertise, and provides: (1) computerized cover sheet and resources forms; (2) electronic signature and transmission; (3) a data-based information system; (4) graphics; (5) intercenter communications; (6) management information; and (7) text editing. The system is coordinated with Headquarters efforts in codes R,E, and T.

  5. Lithium battery management system

    DOEpatents

    Dougherty, Thomas J [Waukesha, WI

    2012-05-08

    Provided is a system for managing a lithium battery system having a plurality of cells. The battery system comprises a variable-resistance element electrically connected to a cell and located proximate a portion of the cell; and a device for determining, utilizing the variable-resistance element, whether the temperature of the cell has exceeded a predetermined threshold. A method of managing the temperature of a lithium battery system is also included.

  6. Space power systems technology

    NASA Astrophysics Data System (ADS)

    Coulman, George A.

    1994-03-01

    Reported here is a series of studies which examine several potential catalysts and electrodes for some fuel cell systems, some materials for space applications, and mathematical modeling and performance predictions for some solid oxide fuel cells and electrolyzers. The fuel cell systems have a potential for terrestrial applications in addition to solar energy conversion in space applications. Catalysts and electrodes for phosphoric acid fuel cell systems and for polymer electrolyte membrane (PEM) fuel cell and electrolyzer systems were examined.

  7. Space station power system

    NASA Technical Reports Server (NTRS)

    Baraona, Cosmo R.

    1987-01-01

    The major requirements and guidelines that affect the space station configuration and power system are explained. The evolution of the space station power system from the NASA program development-feasibility phase through the current preliminary design phase is described. Several early station concepts are described and linked to the present concept. Trade study selections of photovoltaic system technologies are described in detail. A summary of present solar dynamic and power management and distribution systems is also given.

  8. GRAPS: Graphical Plotting System.

    DTIC Science & Technology

    1985-07-01

    stand-alone unit, but could (with a few modifications) be incorporated into a larger system; e.g., the IGUANA system (ref 3). The GRAPS system...the GRAPS software into another system (e.g., the IGUANA ) could possibly introduce additional requirements. I have simply listed those things...Graphics Utility For Army NEC Automation ( IGUANA ), May 1985. 4. Hewlett-Packard Corp., Interfacing and Programming Manual - HP 7470A Graphics Plotter

  9. Compact Antispin Parachute System

    NASA Technical Reports Server (NTRS)

    Whipple, R. D.

    1985-01-01

    Costs and complexity of parachute system greatly reduced. Rigidtowline parachute system operates in three steps: (1) When aircraft begins to spin, parachute compartment opened up, and parachute unfolds; (2) Parachute deployed and spin terminated; and (3) Parachute released. Advantage of towline system allows use of much smaller parachute, reducing design loads on structural reinforcement. Also, complex pyrotechnic-deployment or jettison systems no longer necessary.

  10. Energy Systems Laboratory Groundbreaking

    SciTech Connect

    Hill, David; Otter, C.L.; Simpson, Mike; Rogers, J.W.

    2011-01-01

    INL recently broke ground for a research facility that will house research programs for bioenergy, advanced battery systems, and new hybrid energy systems that integrate renewable, fossil and nuclear energy sources. Here's video from the groundbreaking ceremony for INL's new Energy Systems Laboratory. You can learn more about CAES research at http://www.facebook.com/idahonationallaboratory.

  11. Solar tracking system

    DOEpatents

    Okandan, Murat; Nielson, Gregory N.

    2016-07-12

    Solar tracking systems, as well as methods of using such solar tracking systems, are disclosed. More particularly, embodiments of the solar tracking systems include lateral supports horizontally positioned between uprights to support photovoltaic modules. The lateral supports may be raised and lowered along the uprights or translated to cause the photovoltaic modules to track the moving sun.

  12. Circulation Systems on Microcomputers.

    ERIC Educational Resources Information Center

    Carlson, Gary

    1984-01-01

    Reports on the use of microcomputers in comprehensive library circulation systems. Topics covered include system requirements (reliability, completeness); determining circulation system needs (saving money, improving service, modernization); limitations of microcomputers (capacity, kinds of data stored, number of stations or terminals); system…

  13. Types of Data Systems

    ERIC Educational Resources Information Center

    Gould, Tate; Nicholas, Amy; Ruggiero, Tony; Blandford, William; Thayer, Sara; Bull, Bruce

    2015-01-01

    There are several types of data systems that support data from Part C/619 programs. Although the system types have similarities, each has its own unique characteristics and purposes. The attributes that make one type of data system a particularly good fit for one data-related need or function can be less desirable for another need or function. In…

  14. Spleen and Lymphatic System

    MedlinePlus

    ... A Week of Healthy Breakfasts Shyness Spleen and Lymphatic System KidsHealth > For Teens > Spleen and Lymphatic System A A A What's in this article? Why ... español El bazo y el sistema linfático The lymphatic system is an extensive drainage network that helps keep ...

  15. Microelectronics bioinstrumentation systems

    NASA Technical Reports Server (NTRS)

    Ko, W. H.

    1977-01-01

    Microelectronic bioinstrumentation systems to be employed in the Cardiovascular Deconditioning Program were developed. Implantable telemetry systems for long-term monitoring of animals on earth were designed to collect physiological data necessary for the understanding of the mechanisms of cardiovascular deconditioning. In-flight instrumentation systems, microelectronic instruments, and RF powering techniques for other life science experiments in the NASA program were studied.

  16. Unified Science - System.

    ERIC Educational Resources Information Center

    Thomson, Barbara S.

    The goals of this curriculum, developed as a summer course for the Young Scholars Program at The Ohio State University-Columbus, are as follows: (1) enable students to develop an understanding of the concept of a system; (2) help students gain an appreciation of the value of systems; (3) develop skills in working with systems; (4) expand skills in…

  17. Control Oriented System Identification

    DTIC Science & Technology

    1993-08-01

    The research goals for this grant were to obtain algorithms for control oriented system identification is to construct dynamical models of systems...and measured information. Algorithms for this type of nonlinear system identification have been given that produce models suitable for gain scheduled

  18. Female Reproductive System

    MedlinePlus

    ... Old Feeding Your 1- to 2-Year-Old Female Reproductive System KidsHealth > For Parents > Female Reproductive System A A ... the egg or sperm. continue Parts of the Female Reproductive System Unlike the male, the human female has a ...

  19. MILITARY INFORMATION SYSTEMS,

    DTIC Science & Technology

    upward are usually indications of how effectively the system is developing or operating. The use of computers in information systems tends to increase...computers into information systems must always begin at the lowest level of aggregation in the job hierarchy. Only those information-processing jobs

  20. Rapid response deluge system

    NASA Astrophysics Data System (ADS)

    Mille, J. R.

    1984-08-01

    The development of a rapid response deluge system by the Ammunition Equipment Directorate (AED) for use in suppressing propellant fires during demilitarization shows great promise. Prototype systems have been tested and data acquired on their efficiencies. Present system vs previous generations and lessons learned are discussed.

  1. System Documentation Manual.

    ERIC Educational Resources Information Center

    Semmel, Melvyn I.; Olson, Jerry

    The document is a system documentation manual of the Computer-Assisted Teacher Training System (CATTS) developed by the Center for Innovation in Teaching the Handicapped (Indiana University). CATTS is characterized as a system capable of providing continuous, instantaneous, and/or delayed feedback of relevant teacher-student interaction data to a…

  2. No System? No Software!

    ERIC Educational Resources Information Center

    Conley, Donna

    1989-01-01

    Noting that the computer software cataloging system at the media center of the Washington (Illinois) Center for Continuing Education is essentially a card catalog in notebook form, this article describes aspects of the development and utilization of the system. Major sections describe: (1) software cataloging system terminology; (2) steps for…

  3. FUNDAMENTALS OF TELEVISION SYSTEMS.

    ERIC Educational Resources Information Center

    KESSLER, WILLIAM J.

    DESIGNED FOR A READER WITHOUT SPECIAL TECHNICAL KNOWLEDGE, THIS ILLUSTRATED RESOURCE PAPER EXPLAINS THE COMPONENTS OF A TELEVISION SYSTEM AND RELATES THEM TO THE COMPLETE SYSTEM. SUBJECTS DISCUSSED ARE THE FOLLOWING--STUDIO ORGANIZATION AND COMPATIBLE COLOR TELEVISION PRINCIPLES, WIRED AND RADIO TRANSMISSION SYSTEMS, DIRECT VIEW AND PROJECTION…

  4. System integration report

    NASA Technical Reports Server (NTRS)

    Badler, N. I.; Korein, J. D.; Meyer, C.; Manoochehri, K.; Rovins, J.; Beale, J.; Barr, B.

    1985-01-01

    Several areas that arise from the system integration issue were examined. Intersystem analysis is discussed as it relates to software development, shared data bases and interfaces between TEMPUS and PLAID, shaded graphics rendering systems, object design (BUILD), the TEMPUS animation system, anthropometric lab integration, ongoing TEMPUS support and maintenance, and the impact of UNIX and local workstations on the OSDS environment.

  5. IDC System Specification Document.

    SciTech Connect

    Clifford, David J.

    2014-12-01

    This document contains the system specifications derived to satisfy the system requirements found in the IDC System Requirements Document for the IDC Reengineering Phase 2 project. Revisions Version Date Author/Team Revision Description Authorized by V1.0 12/2014 IDC Reengineering Project Team Initial delivery M. Harris

  6. Economical space power systems

    NASA Technical Reports Server (NTRS)

    Burkholder, J. H.

    1980-01-01

    A commercial approach to design and fabrication of an economical space power system is investigated. Cost projections are based on a 2 kW space power system conceptual design taking into consideration the capability for serviceability, constraints of operation in space, and commercial production engineering approaches. A breakdown of the system design, documentation, fabrication, and reliability and quality assurance estimated costs are detailed.

  7. Energy Systems Laboratory Groundbreaking

    ScienceCinema

    Hill, David; Otter, C.L.; Simpson, Mike; Rogers, J.W.

    2016-07-12

    INL recently broke ground for a research facility that will house research programs for bioenergy, advanced battery systems, and new hybrid energy systems that integrate renewable, fossil and nuclear energy sources. Here's video from the groundbreaking ceremony for INL's new Energy Systems Laboratory. You can learn more about CAES research at http://www.facebook.com/idahonationallaboratory.

  8. Neutron radiographic viewing system

    NASA Technical Reports Server (NTRS)

    Leysath, W.; Brown, R. L.

    1972-01-01

    Neutron radiographic viewing system consisting of camera head and control processor is developed for use in nondestructive testing applications. Camera head consists of neutron-sensitive image intensifier system, power supply, and SEC vidicon camera head. Both systems, with their optics, are housed on test mount.

  9. Lungs and Respiratory System

    MedlinePlus

    ... A Week of Healthy Breakfasts Shyness Lungs and Respiratory System KidsHealth > For Teens > Lungs and Respiratory System Print ... didn't breathe, you couldn't live. Lungs & Respiratory System Basics Each day we breathe about 20,000 ...

  10. Immune System Quiz

    MedlinePlus

    ... los dientes Video: Getting an X-ray Quiz: Immune System KidsHealth > For Kids > Quiz: Immune System Print A A A How much do you know about your immune system? Find out by taking this quiz! About KidsHealth ...

  11. Facilities Data System Manual.

    ERIC Educational Resources Information Center

    Acridge, Charles W.; Ford, Tim M.

    The purposes of this manual are to set forth the scope and procedures for the maintenance and operation of the University of California facilities Data System (FDX) and to serve as a reference document for users of the system. FDX is an information system providing planning and management data about the existing physical plant. That is, it…

  12. Personal Food System Mapping

    ERIC Educational Resources Information Center

    Wilsey, David; Dover, Sally

    2014-01-01

    Personal food system mapping is a practical means to engage community participants and educators in individualized and shared learning about food systems, decisions, and behaviors. Moreover, it is a useful approach for introducing the food system concept, which is somewhat abstract. We developed the approach to capture diversity of personal food…

  13. Lungs and Respiratory System

    MedlinePlus

    ... A Week of Healthy Breakfasts Shyness Lungs and Respiratory System KidsHealth > For Teens > Lungs and Respiratory System A ... didn't breathe, you couldn't live. Lungs & Respiratory System Basics Each day we breathe about 20,000 ...

  14. Arkansas Technology Information System.

    ERIC Educational Resources Information Center

    VanBiervliet, Alan; Parette, Howard P., Jr.

    The Arkansas Technology Information System (ARTIS) was developed to fill a significant void in existing systems of technical support to Arkansans with disabilities by creating and maintaining a consumer-responsive statewide system of data storage and retrieval regarding assistive technology and services. ARTIS goals also include establishment of a…

  15. Power system restoration issues

    SciTech Connect

    Adibi, M.M. ); Kafka, R.J. )

    1991-04-01

    This article describes some of the problems encountered in the three phases of power system restoration (PSR). The three phases of PSR are: Planning for restart and reintegration of the bulk power supply; Actions during system degradation for saving and retaining critical sources of power; Restoration when the power system has stabilized at some degraded level.

  16. Polymerase chain reaction system

    DOEpatents

    Benett, William J.; Richards, James B.; Stratton, Paul L.; Hadley, Dean R.; Milanovich, Fred P.; Belgrader, Phil; Meyer, Peter L.

    2004-03-02

    A portable polymerase chain reaction DNA amplification and detection system includes one or more chamber modules. Each module supports a duplex assay of a biological sample. Each module has two parallel interrogation ports with a linear optical system. The system is capable of being handheld.

  17. Male Reproductive System

    MedlinePlus

    ... Old Feeding Your 1- to 2-Year-Old Male Reproductive System KidsHealth > For Parents > Male Reproductive System Print A ... understand your son's reproductive health. continue About the Male Reproductive System Most species have two sexes: male and female. ...

  18. Jupiter System Observer

    NASA Technical Reports Server (NTRS)

    Senske, Dave; Prockter, Louise

    2008-01-01

    This slide presentation reviews the scientific philosophy that is guiding the planning behind the Jupiter System Observer (JSO). The JSO would be a long-term platform for studying Jupiter and the complete Jovian system. The goal is to advance the understanding of the fundamental processes of planetary systems, their formation and evolution.

  19. Enterprise Systems Analysis

    DTIC Science & Technology

    2016-03-14

    Systems ...Report No. SERC-2016-TR-103 Copyright © 2016 Stevens Institute of Technology The Systems Engineering Research Center (SERC) is a federally...Stevens Institute of Technology and Systems Engineering Research Center Material is furnished on an “as-is” basis. Stevens Institute of

  20. Astromag data system concept

    NASA Technical Reports Server (NTRS)

    Roos, Darrell; Cheng, Chieh-San; Newsome, Penny; Nath, Nitya

    1989-01-01

    A feasible, top-level data system is defined that could accomplish and support the Astromag Data System functions and interfaces necessary to support the scientific objectives of Astromag. This data system must also be able to function in the environment of the Space Station Freedom Manned Base (SSFMB) and other anticipated NASA elements.

  1. Wastewater Collection Systems.

    PubMed

    Vallabhaneni, Srinivas

    2015-10-01

    This chapter presents a review of the literature published in 2014 on topics relating to wastewater collection systems. It presents advances in noteworthy research and industry experiences selected from major literature sources. This review is divided into the following sections: sewer system planning; sewer condition assessment/rehabilitation; pump stations and system design; operation and maintenance; and regulatory issues/ integrated planning.

  2. Desiccant humidity control system

    NASA Technical Reports Server (NTRS)

    Amazeen, J. (Editor)

    1973-01-01

    A regenerable sorbent system was investigated for controlling the humidity and carbon dioxide concentration of the space shuttle cabin atmosphere. The sorbents considered for water and carbon dioxide removal were silica gel and molecular sieves. Bed optimization and preliminary system design are discussed along with system optimization studies and weight penalites.

  3. Special Education Resource System.

    ERIC Educational Resources Information Center

    Kampert, George J.

    The guide to the Special Education Resource System (SERS) of the Flour Bluff (Texas) school system contains policies and procedures regarding organization, services, and process of the system. Noted is the SERS purpose of providing information and access to appropriate instructional media and materials for special education personnel. Briefly…

  4. The Global Positioning System

    DTIC Science & Technology

    1989-05-01

    Global Positioning System ( GPS ) and current program status are provided. The importance of...the NAVSTAR Global Positioning System (NAVSTAR GPS or GPS ) will have on future weapons employment. The study will: examine why we need a high...Washington, D.C., 24 January, 1984. Malone, Daniel K. GPS /NAVSTAR. Military Review, March 1986. Nepean, Phillip The NAVSTAR Global Positioning System

  5. Information extraction system

    DOEpatents

    Lemmond, Tracy D; Hanley, William G; Guensche, Joseph Wendell; Perry, Nathan C; Nitao, John J; Kidwell, Paul Brandon; Boakye, Kofi Agyeman; Glaser, Ron E; Prenger, Ryan James

    2014-05-13

    An information extraction system and methods of operating the system are provided. In particular, an information extraction system for performing meta-extraction of named entities of people, organizations, and locations as well as relationships and events from text documents are described herein.

  6. Electric system restructuring and system reliability

    NASA Astrophysics Data System (ADS)

    Horiuchi, Catherine Miller

    In 1996 the California legislature passed AB 1890, explicitly defining economic benefits and detailing specific mechanisms for initiating a partial restructuring the state's electric system. Critics have since sought re-regulation and proponents have asked for patience as the new institutions and markets take shape. Other states' electric system restructuring activities have been tempered by real and perceived problems in the California model. This study examines the reduced regulatory controls and new constraints introduced in California's limited restructuring model using utility and regulatory agency records from the 1990's to investigate effects of new institutions and practices on system reliability for the state's five largest public and private utilities. Logit and negative binomial regressions indicate negative impact from the California model of restructuring on system reliability as measured by customer interruptions. Time series analysis of outage data could not predict the wholesale power market collapse and the subsequent rolling blackouts in early 2001; inclusion of near-outage reliability disturbances---load shedding and energy emergencies---provided a measure of forewarning. Analysis of system disruptions, generation capacity and demand, and the role of purchased power challenge conventional wisdom on the causality of Californian's power problems. The quantitative analysis was supplemented by a targeted survey of electric system restructuring participants. Findings suggest each utility and the organization controlling the state's electric grid provided protection from power outages comparable to pre-restructuring operations through 2000; however, this reliability has come at an inflated cost, resulting in reduced system purchases and decreased marginal protection. The historic margin of operating safety has fully eroded, increasing mandatory load shedding and emergency declarations for voluntary and mandatory conservation. Proposed remedies focused

  7. Chemical Technology Division. Annual technical report, 1995

    SciTech Connect

    Laidler, J.J.; Myles, K.M.; Green, D.W.; McPheeters, C.C.

    1996-06-01

    Highlights of the Chemical Technology (CMT) Division`s activities during 1995 are presented. In this period, CMT conducted research and development in the following areas: (1) electrochemical technology, including advanced batteries and fuel cells; (2) methods for treatment of hazardous waste and mixed hazardous/radioactive waste; (3) the reaction of nuclear waste glass and spent fuel under conditions expected for an unsaturated repository; (4) processes for separating and recovering selected elements from waste streams, concentrating low-level radioactive waste streams with advanced evaporator technology, and producing {sup 99}Mo from low-enriched uranium; (5) electrometallurgical treatment of different types of spent nuclear fuel in storage at Department of Energy sites; and (6) physical chemistry of selected materials in environments simulating those of fission and fusion energy systems.

  8. Effect of Solcoseryl on the clinical course of experimental Escherichia coli-endotoxin mastitis.

    PubMed

    Ziv, G; Jöchle, W

    1981-09-01

    Experimental Escherichia coli-endotoxin mastitis was induced in a single quarter in twenty-eight cows. The inflamed quarters of six of the cows were treated with 50 mg Solcoseryl infusion in an oil base starting 4-6 h after endotoxin infusion, and four additional infusions were given at 12-h intervals. The inflamed quarters of eight of the cows were similarly treated with 200 mg Solcoseryl whereas the remaining fourteen cows served as control. Solcoseryl treatment did not alter the systemic or local course of acute mastitis. Local reaction, as assessed by the California Mastitis Test (CMT), however, subsided considerably sooner in quarters treated with Solcoseryl, and the effect of Solcoseryl in hastening udder tissue repair processes appeared to be dose dependent. The 200 mg dose of Solcoseryl resulted in pre-endotoxin CMT scores as early as 6 days after endotoxin infusion.

  9. SNS Cryogenic Systems Commissioning

    SciTech Connect

    D. Hatfield; F. Casagrande; I. Campisi; P. Gurd; M. Howell; D. Stout; H. Strong; D. Arenius; J. Creel; K. Dixon; V. Ganni; and P. Knudsen

    2005-08-29

    The Spallation Neutron Source (SNS) is under construction at Oak Ridge National Laboratory. The cold section of the Linac consists of 81 superconducting radio frequency cavities cooled to 2.1K by a 2400 watt cryogenic refrigeration system. The major cryogenic system components include warm helium compressors with associated oil removal and gas management, 4.5K cold box, 7000L liquid helium dewar, 2.1K cold box (consisting of 4 stages of cold compressors), gaseous helium storage, helium purification and gas impurity monitoring system, liquid nitrogen storage and the cryogenic distribution transfer line system. The overall system commissioning and future plans will be presented.

  10. Benchmarking expert system tools

    NASA Technical Reports Server (NTRS)

    Riley, Gary

    1988-01-01

    As part of its evaluation of new technologies, the Artificial Intelligence Section of the Mission Planning and Analysis Div. at NASA-Johnson has made timing tests of several expert system building tools. Among the production systems tested were Automated Reasoning Tool, several versions of OPS5, and CLIPS (C Language Integrated Production System), an expert system builder developed by the AI section. Also included in the test were a Zetalisp version of the benchmark along with four versions of the benchmark written in Knowledge Engineering Environment, an object oriented, frame based expert system tool. The benchmarks used for testing are studied.

  11. Text Exchange System

    NASA Technical Reports Server (NTRS)

    Snyder, W. V.; Hanson, R. J.

    1986-01-01

    Text Exchange System (TES) exchanges and maintains organized textual information including source code, documentation, data, and listings. System consists of two computer programs and definition of format for information storage. Comprehensive program used to create, read, and maintain TES files. TES developed to meet three goals: First, easy and efficient exchange of programs and other textual data between similar and dissimilar computer systems via magnetic tape. Second, provide transportable management system for textual information. Third, provide common user interface, over wide variety of computing systems, for all activities associated with text exchange.

  12. Superconductor rotor cooling system

    DOEpatents

    Gamble, Bruce B.; Sidi-Yekhlef, Ahmed; Schwall, Robert E.; Driscoll, David I.; Shoykhet, Boris A.

    2004-11-02

    A system for cooling a superconductor device includes a cryocooler located in a stationary reference frame and a closed circulation system external to the cryocooler. The closed circulation system interfaces the stationary reference frame with a rotating reference frame in which the superconductor device is located. A method of cooling a superconductor device includes locating a cryocooler in a stationary reference frame, and transferring heat from a superconductor device located in a rotating reference frame to the cryocooler through a closed circulation system external to the cryocooler. The closed circulation system interfaces the stationary reference frame with the rotating reference frame.

  13. General curvilinear coordinate systems

    NASA Technical Reports Server (NTRS)

    Thompson, J. P.

    1982-01-01

    The basic ideas of the construction and use of numerically-generated boundary-fitted coordinate systems for the numerical solution of partial differential equations are discussed. With such coordinate systems, all computation can be done on a fixed square grid in the rectangular transformed region regardless of the shape or movement of the physical boundaries. A number of different types of configurations for the transformed region and the basic transformation relations from a cartesian system to a general curvilinear system are given. The material of this paper is applicable to all types of coordinate system generation.

  14. Systems Biology of Embryogenesis

    PubMed Central

    Edelman, Lucas B.; Chandrasekaran, Sriram; Price, Nathan D.

    2010-01-01

    The development of a complete organism from a single cell involves extraordinarily complex orchestration of biological processes that vary intricately across space and time. Systems biology seeks to describe how all elements of a biological system interact in order to understand, model, and ultimately predict aspects of emergent biological processes. Embryogenesis represents an extraordinary opportunity – and challenge – for the application of systems biology. Systems approaches have already been used successfully to study various aspects of development, from complex intracellular networks to 4D models of organogenesis. Going forward, great advancements and discoveries can be expected from systems approaches applied to embryogenesis and developmental biology. PMID:20003850

  15. Photovoltaic power systems workshop

    NASA Technical Reports Server (NTRS)

    Killian, H. J.; Given, R. W.

    1978-01-01

    Discussions are presented on apparent deficiencies in NASA planning and technology development relating to a standard power module (25-35 kW) and to future photovoltaic power systems in general. Topics of discussion consider the following: (1) adequate studies on power systems; (2) whether a standard power system module should be developed from a standard spacecraft; (3) identification of proper approaches to cost reduction; (4) energy storage avoidance; (5) attitude control; (6) thermal effects of heat rejection on solar array configuration stability; (7) assembly of large power systems in space; and (8) factoring terrestrial photovoltaic work into space power systems for possible payoff.

  16. Medical Imaging System

    NASA Technical Reports Server (NTRS)

    1991-01-01

    The MD Image System, a true-color image processing system that serves as a diagnostic aid and tool for storage and distribution of images, was developed by Medical Image Management Systems, Huntsville, AL, as a "spinoff from a spinoff." The original spinoff, Geostar 8800, developed by Crystal Image Technologies, Huntsville, incorporates advanced UNIX versions of ELAS (developed by NASA's Earth Resources Laboratory for analysis of Landsat images) for general purpose image processing. The MD Image System is an application of this technology to a medical system that aids in the diagnosis of cancer, and can accept, store and analyze images from other sources such as Magnetic Resonance Imaging.

  17. THERMAL DISTRIBUTION SYSTEM EXPERIMENT

    SciTech Connect

    KRAJEWSKI,R.F.; ANDREWS,J.W.; WEI,G.

    1999-09-01

    A laboratory experiment has been conducted which tests for the effects of distribution system purging on system Delivery Effectiveness (DE) as defined in ASHRAE 152P. The experiment is described in its configuration, instrumentation, and data acquisition system. Data gathered in the experiment is given and discussed. The results show that purging of the distribution system alone does not offer any improvement of the system DE. Additional supporting tests were conducted regarding experimental simulations of buffer zones and bare pipe and are also discussed.

  18. Nuclear propulsion systems engineering

    SciTech Connect

    Madsen, W.W.; Neuman, J.E.: Van Haaften, D.H.

    1992-12-31

    The Nuclear Energy for Rocket Vehicle Application (NERVA) program of the 1960`s and early 1970`s was dramatically successful, with no major failures during the entire testing program. This success was due in large part to the successful development of a systems engineering process. Systems engineering, properly implemented, involves all aspects of the system design and operation, and leads to optimization of theentire system: cost, schedule, performance, safety, reliability, function, requirements, etc. The process must be incorporated from the very first and continued to project completion. This paper will discuss major aspects of the NERVA systems engineering effort, and consider the implications for current nuclear propulsion efforts.

  19. Nuclear propulsion systems engineering

    SciTech Connect

    Madsen, W.W.; Neuman, J.E.: Van Haaften, D.H.

    1992-01-01

    The Nuclear Energy for Rocket Vehicle Application (NERVA) program of the 1960's and early 1970's was dramatically successful, with no major failures during the entire testing program. This success was due in large part to the successful development of a systems engineering process. Systems engineering, properly implemented, involves all aspects of the system design and operation, and leads to optimization of theentire system: cost, schedule, performance, safety, reliability, function, requirements, etc. The process must be incorporated from the very first and continued to project completion. This paper will discuss major aspects of the NERVA systems engineering effort, and consider the implications for current nuclear propulsion efforts.

  20. FRIB cryogenic distribution system

    SciTech Connect

    Ganni, Venkatarao; Dixon, Kelly D.; Laverdure, Nathaniel A.; Knudsen, Peter N.; Arenius, Dana M.; Barrios, Matthew N.; Jones, S.; Johnson, M.; Casagrande, Fabio

    2014-01-01

    The Michigan State University Facility for Rare Isotope Beams (MSU-FRIB) helium distribution system has been revised to include bayonet/warm valve type disconnects between each cryomodule and the transfer line distribution system, similar to the Thomas Jefferson National Accelerator Facility (JLab) and the Spallation Neutron Source (SNS) cryogenic distribution systems. The heat loads at various temperature levels and some of the features in the design of the distribution system are outlined. The present status, the plans for fabrication, and the procurement approach for the helium distribution system are also included.

  1. SNS Cryogenic Systems Commissioning

    NASA Astrophysics Data System (ADS)

    Hatfield, D.; Casagrande, F.; Campisi, I.; Gurd, P.; Howell, M.; Stout, D.; Strong, H.; Arenius, D.; Creel, J.; Dixon, K.; Ganni, V.; Knudsen, P.

    2006-04-01

    The Spallation Neutron Source (SNS) is under construction at Oak Ridge National Laboratory. The cold section of the Linac consists of 81 superconducting radio frequency cavities cooled to 2.1K by a 2400 watt cryogenic refrigeration system. The major cryogenic system components include warm helium compressors with associated oil removal and gas management, 4.5K cold box, 7000L liquid helium dewar, 2.1K cold box (consisting of 4 stages of cold compressors), gaseous helium storage, helium purification and gas impurity monitoring system, liquid nitrogen storage and the cryogenic distribution transfer line system. The overall system commissioning and future plans will be presented.

  2. Smart vehicular transportation systems

    SciTech Connect

    Little, C.Q.; Wilson, C.W.

    1997-05-01

    This work builds upon established Sandia intelligent systems technology to develop a unique approach for the integration of intelligent system control into the US Highway and urban transportation systems. The Sandia developed concept of the COPILOT controller integrates a human driver with computer control to increase human performance while reducing reliance on detailed driver attention. This research extends Sandia expertise in sensor based, real-time control of robotics systems to high speed transportation systems. Knowledge in the form of maps and performance characteristics of vehicles provides the automatic decision making intelligence needed to plan optimum routes, maintain safe driving speeds and distances, avoid collisions, and conserve fuel.

  3. Range Safety Systems

    NASA Technical Reports Server (NTRS)

    Schrock, Kenneth W.; Humphries, Ricky H. (Technical Monitor)

    2002-01-01

    The high kinetic and potential energy of a launch vehicle mandates there be a mechanism to minimize possible damage to provide adequate safety for the launch facilities, range, and, most importantly, the general public. The Range Safety System, sometimes called the Flight Termination System or Flight Safety System, provides the required level of safety. The Range Safety System section of the Avionics chapter will attempt to describe how adequate safety is provided, the system's design, operation, and it's interface with the rest of the launch vehicle.

  4. Space engine safety system

    NASA Technical Reports Server (NTRS)

    Maul, William A.; Meyer, Claudia M.

    1991-01-01

    A rocket engine safety system is designed to initiate control procedures which will minimize damage to the engine and vehicle or test stand in the event of an engine failure. This report describes the features and the implementation issues associated with rocket engine safety systems. Specific concerns of safety systems applied to a space-based engine and long duration space missions are discussed. Examples of safety system features and architectures are given from recent safety monitoring investigations conducted for the Space Shuttle Main Engine and for future liquid rocket engines. Also, a general design and implementation process for rocket engine safety systems is presented.

  5. Superconductor rotor cooling system

    DOEpatents

    Gamble, Bruce B.; Sidi-Yekhlef, Ahmed; Schwall, Robert E.; Driscoll, David I.; Shoykhet, Boris A.

    2002-01-01

    A system for cooling a superconductor device includes a cryocooler located in a stationary reference frame and a closed circulation system external to the cryocooler. The closed circulation system interfaces the stationary reference frame with a rotating reference frame in which the superconductor device is located. A method of cooling a superconductor device includes locating a cryocooler in a stationary reference frame, and transferring heat from a superconductor device located in a rotating reference frame to the cryocooler through a closed circulation system external to the cryocooler. The closed circulation system interfaces the stationary reference frame with the rotating reference frame.

  6. CNEOST Control Software System

    NASA Astrophysics Data System (ADS)

    Wang, X.; Zhao, H. B.; Xia, Y.; Lu, H.; Li, B.

    2015-03-01

    In 2013, CNEOST (China Near Earth Object Survey Telescope) adapted its hardware system for the new CCD camera. Based on the new system architecture, the control software is re-designed and implemented. The software system adopts the message passing mechanism via WebSocket protocol, and improves its flexibility, expansibility, and scalability. The user interface with responsive web design realizes the remote operating under both desktop and mobile devices. The stable operating of software system has greatly enhanced the operation efficiency while reducing the complexity, and has also made a successful attempt for the future system design of telescope and telescope cloud.

  7. Spacecraft Docking System

    NASA Technical Reports Server (NTRS)

    Ghofranian, Siamak (Inventor); Chuang, Li-Ping Christopher (Inventor); Motaghedi, Pejmun (Inventor)

    2016-01-01

    A method and apparatus for docking a spacecraft. The apparatus comprises elongate members, movement systems, and force management systems. The elongate members are associated with a docking structure for a spacecraft. The movement systems are configured to move the elongate members axially such that the docking structure for the spacecraft moves. Each of the elongate members is configured to move independently. The force management systems connect the movement systems to the elongate members and are configured to limit a force applied by the each of the elongate members to a desired threshold during movement of the elongate members.

  8. Cross-functional systems

    NASA Technical Reports Server (NTRS)

    Lee, Mark

    1991-01-01

    Many companies, including Xerox and Texas Instruments, are using cross functional systems to deal with the increasingly complex and competitive business environment. However, few firms within the aerospace industry appear to be aware of the significant benefits that cross functional systems can provide. Those benefits are examined and a flexible methodology is discussed that companies can use to identify and develop cross functional systems that will help improve organizational performance. In addition, some of the managerial issues are addressed that cross functional systems may raise and specific examples are used to explore networking's contributions to cross functional systems.

  9. Positive lubrication system

    NASA Technical Reports Server (NTRS)

    Smith, Dennis W.; Hooper, Fred L.

    1990-01-01

    As part of the development of an autonomous lubrication system for spin bearings, a system was developed to deliver oil to grease-lubricated bearings upon demand. This positive oil delivery system (PLUS) consists of a pressurized reservoir with a built-in solenoid valve that delivers a predictable quantity of oil to the spin bearing through a system of stainless steel tubes. Considerable testing was performed on the PLUS to characterize its performance and verify its effectiveness, along with qualifying it for flight. Additional development is underway that will lead to the fully autonomous active lubrication system.

  10. Power system restoration planning

    SciTech Connect

    Adibi, M.M. ); Fink, L.H.

    1994-02-01

    System restoration, as an extraordinary mode of system operation, requires careful planning and operator training. The generic tasks of restoration include determination of system and equipment status, preparation of plants and network for systematic restoration, reenergization of the network, and system rebuilding. The procedures for developing an effective restoration plan include formation of a qualified planning team, review of relevant system characteristics, formulation of assumptions regarding blackout scenarios, agreement on restoration goals, development of strategy and tactics, validation of the plan, training, and documentation.

  11. 2014 Runtime Systems Summit. Runtime Systems Report

    SciTech Connect

    Sarkar, Vivek; Budimlic, Zoran; Kulkani, Milind

    2016-09-19

    This report summarizes runtime system challenges for exascale computing, that follow from the fundamental challenges for exascale systems that have been well studied in past reports, e.g., [6, 33, 34, 32, 24]. Some of the key exascale challenges that pertain to runtime systems include parallelism, energy efficiency, memory hierarchies, data movement, heterogeneous processors and memories, resilience, performance variability, dynamic resource allocation, performance portability, and interoperability with legacy code. In addition to summarizing these challenges, the report also outlines different approaches to addressing these significant challenges that have been pursued by research projects in the DOE-sponsored X-Stack and OS/R programs. Since there is often confusion as to what exactly the term “runtime system” refers to in the software stack, we include a section on taxonomy to clarify the terminology used by participants in these research projects. In addition, we include a section on deployment opportunities for vendors and government labs to build on the research results from these projects. Finally, this report is also intended to provide a framework for discussing future research and development investments for exascale runtime systems, and for clarifying the role of runtime systems in exascale software.

  12. Japanese neuropathy patients with peripheral myelin protein-22 gene aneuploidy

    SciTech Connect

    Lebo, R.V.; Li, L.Y.; Flandermeyer, R.R.

    1994-09-01

    Peripheral myelin protein (PMP-22) gene aneuploidy results in Charcot-Marie-Tooth disease Type 1A (CMT1A) and the Hereditary Neuropathy with Liability to Pressure Palsy (HNPP) in Japanese patients as well as Caucasian Americans. Charcot-Marie-Tooth disease (CMT), the most common genetic neuropathy, results when expression of one of at least seven genes is defective. CMT1A, about half of all CMT mutations, is usually associated with a duplication spanning the peripheral myelin protein-22 gene on distal chromosome band 17p11.2. Autosomal dominant HNPP (hereditary pressure and sensory neuropathy, HPSN) results from a deletion of the CMT1A gene region. Multicolor in situ hybridization with PMP-22 gene region probe characterized HNPP deletion reliably and detected all different size duplications reported previously. In summary, 72% of 28 Japanese CMT1 (HMSNI) patients tested had the CMT1A duplication, while none of the CMT2 (HMSNII) or CMT3 (HMSNIII) patients had a duplication. Three cases of HNPP were identified by deletion of the CMT1A gene region on chromosome 17p. HNPP and CMT1A have been reported to result simultaneously from the same unequal recombination event. The lower frequency of HNPP compared to CMT1A suggests that HNPP patients have a lower reproductive fitness than CMT1A patients. This result, along with a CMT1A duplication found in an Asian Indian family, demonstrates the broad geographic distribution and high frequency of PMP-22 gene aneuploidy.

  13. Active optical zoom system

    DOEpatents

    Wick, David V.

    2005-12-20

    An active optical zoom system changes the magnification (or effective focal length) of an optical imaging system by utilizing two or more active optics in a conventional optical system. The system can create relatively large changes in system magnification with very small changes in the focal lengths of individual active elements by leveraging the optical power of the conventional optical elements (e.g., passive lenses and mirrors) surrounding the active optics. The active optics serve primarily as variable focal-length lenses or mirrors, although adding other aberrations enables increased utility. The active optics can either be LC SLMs, used in a transmissive optical zoom system, or DMs, used in a reflective optical zoom system. By appropriately designing the optical system, the variable focal-length lenses or mirrors can provide the flexibility necessary to change the overall system focal length (i.e., effective focal length), and therefore magnification, that is normally accomplished with mechanical motion in conventional zoom lenses. The active optics can provide additional flexibility by allowing magnification to occur anywhere within the FOV of the system, not just on-axis as in a conventional system.

  14. TPC magnet cryogenic system

    SciTech Connect

    Green, M.A.; Burns, W.A.; Taylor, J.D.; Van Slyke, H.W.

    1980-03-01

    The Time Projection Chamber (TPC) magnet at LBL and its compensation solenoids are adiabatically stable superconducting solenoid magnets. The cryogenic system developed for the TPC magnet is discussed. This system uses forced two-phase tubular cooling with the two cryogens in the system. The liquid helium and liquid nitrogen are delivered through the cooled load by forced tubular flow. The only reservoirs of liquid cryogen exist in the control dewar (for liquid helium) and the conditioner dewar (for liquid nitrogen). The operation o these systems during virtually all phases of system operation are described. Photographs and diagrams of various system components are shown, and cryogenic system data are presented in the following sections: (1) heat leaks into the TPC coil package and the compensation solenoids; (2) heat leaks to various components of the TPC magnet cryogenics system besides the magnets and control dewar; (3) the control dewar and its relationship to the rest of the system; (4) the conditioner system and its role in cooling down the TPC magnet; (5) gas-cooled electrical leads and charging losses; and (6) a summation of the liquid helium and liquid nitrogen requirements for the TPC superconducting magnet system.

  15. Model-Based Systems

    NASA Technical Reports Server (NTRS)

    Frisch, Harold P.

    2007-01-01

    Engineers, who design systems using text specification documents, focus their work upon the completed system to meet Performance, time and budget goals. Consistency and integrity is difficult to maintain within text documents for a single complex system and more difficult to maintain as several systems are combined into higher-level systems, are maintained over decades, and evolve technically and in performance through updates. This system design approach frequently results in major changes during the system integration and test phase, and in time and budget overruns. Engineers who build system specification documents within a model-based systems environment go a step further and aggregate all of the data. They interrelate all of the data to insure consistency and integrity. After the model is constructed, the various system specification documents are prepared, all from the same database. The consistency and integrity of the model is assured, therefore the consistency and integrity of the various specification documents is insured. This article attempts to define model-based systems relative to such an environment. The intent is to expose the complexity of the enabling problem by outlining what is needed, why it is needed and how needs are being addressed by international standards writing teams.

  16. Intelligent pumping system developed

    SciTech Connect

    Not Available

    1983-06-01

    The oil field's first intelligent rod pumping system designed specifically to reduce the cost of pumping oil wells now is a reality. As a plus benefit, the system (called Liftronic) is compact and quiet. The new system combines an efficient mechanical design with a computer control system to reduce pumping costs. The unit stands less than 8 ft high, or approx. one-fourth the height of a comparable beam unit. It also mounts directly on the wellhead. The entire system can be concealed behind a fence or enclosed within a small building to make it a more attractive neighbor in residential, commercial, or recreational areas. It is useful also for agricultural areas where overhead irrigation systems restrict the use of many oil field pumping systems.

  17. Microgravity particle reduction system

    NASA Technical Reports Server (NTRS)

    Brandon, Vanessa; Joslin, Michelle; Mateo, Lili; Tubbs, Tracey

    1988-01-01

    The Controlled Ecological Life Support System (CELSS) project, sponsored by NASA, is assembling the knowledge required to design, construct, and operate a system which will grow and process higher plants in space for the consumption by crew members of a space station on a long term space mission. The problem of processing dry granular organic materials in microgravity is discussed. For the purpose of research and testing, wheat was chosen as the granular material to be ground into flour. Possible systems which were devised to transport wheat grains into the food processor, mill the wheat into flour, and transport the flour to the food preparation system are described. The systems were analyzed and compared and two satisfactory systems were chosen. Prototypes of the two preferred systems are to be fabricated next semester. They will be tested under simulated microgravity conditions and revised for maximum effectiveness.

  18. Space Station information systems

    NASA Technical Reports Server (NTRS)

    Swingle, W. L.; Mckay, C. W.

    1983-01-01

    The space operations information system is defined and characterized in a wide perspective. Interactive subsets of the total system are defined and discussed. Particular attention is paid to the concept of end-to-end systems and their repetitive population within the total system. High level program goals are reviewed and related to more explicit system requirements and user needs. Emphasis is placed on the utility and cost effectiveness of data system services from a user standpoint. Productivity, as a quantitative goal, in both development and operational phases is also addressed. Critical aspects of the approach to successful development of the data management system are discussed along with recommendations important to advanced development activities. Current and planned activity in both technology and advanced development areas are reviewed with emphasis on their importance to program success.

  19. Quantum system identification.

    PubMed

    Burgarth, Daniel; Yuasa, Kazuya

    2012-02-24

    The aim of quantum system identification is to estimate the ingredients inside a black box, in which some quantum-mechanical unitary process takes place, by just looking at its input-output behavior. Here we establish a basic and general framework for quantum system identification, that allows us to classify how much knowledge about the quantum system is attainable, in principle, from a given experimental setup. We show that controllable closed quantum systems can be estimated up to unitary conjugation. Prior knowledge on some elements of the black box helps the system identification. We present an example in which a Bell measurement is more efficient to identify the system. When the topology of the system is known, the framework enables us to establish a general criterion for the estimability of the coupling constants in its Hamiltonian.

  20. Coupled nonlinear dynamical systems

    NASA Astrophysics Data System (ADS)

    Sun, Hongyan

    In this dissertation, we study coupled nonlinear dynamical systems that exhibit new types of complex behavior. We numerically and analytically examine a variety of dynamical models, ranging from systems of ordinary differential equations (ODE) with novel elements of feedback to systems of partial differential equations (PDE) that model chemical pattern formation. Chaos, dynamical uncertainty, synchronization, and spatiotemporal pattern formation constitute the primary topics of the dissertation. Following the introduction in Chapter 1, we study chaos and dynamical uncertainty in Chapter 2 with coupled Lorenz systems and demonstrate the existence of extreme complexity in high-dimensional ODE systems. In Chapter 3, we demonstrate that chaos synchronization can be achieved by mutual and multiplicative coupling of dynamical systems. Chapter 4 and 5 focus on pattern formation in reaction-diffusion systems, and we investigate segregation and integration behavior of populations in competitive and cooperative environments, respectively.

  1. Synchronization of chaotic systems

    SciTech Connect

    Pecora, Louis M.; Carroll, Thomas L.

    2015-09-15

    We review some of the history and early work in the area of synchronization in chaotic systems. We start with our own discovery of the phenomenon, but go on to establish the historical timeline of this topic back to the earliest known paper. The topic of synchronization of chaotic systems has always been intriguing, since chaotic systems are known to resist synchronization because of their positive Lyapunov exponents. The convergence of the two systems to identical trajectories is a surprise. We show how people originally thought about this process and how the concept of synchronization changed over the years to a more geometric view using synchronization manifolds. We also show that building synchronizing systems leads naturally to engineering more complex systems whose constituents are chaotic, but which can be tuned to output various chaotic signals. We finally end up at a topic that is still in very active exploration today and that is synchronization of dynamical systems in networks of oscillators.

  2. Thermodynamics of Nonadditive Systems.

    PubMed

    Latella, Ivan; Pérez-Madrid, Agustín; Campa, Alessandro; Casetti, Lapo; Ruffo, Stefano

    2015-06-12

    The usual formulation of thermodynamics is based on the additivity of macroscopic systems. However, there are numerous examples of macroscopic systems that are not additive, due to the long-range character of the interaction among the constituents. We present here an approach in which nonadditive systems can be described within a purely thermodynamics formalism. The basic concept is to consider a large ensemble of replicas of the system where the standard formulation of thermodynamics can be naturally applied and the properties of a single system can be consequently inferred. After presenting the approach, we show its implementation in systems where the interaction decays as 1/r(α) in the interparticle distance r, with α smaller than the embedding dimension d, and in the Thirring model for gravitational systems.

  3. Power Systems Control Architecture

    SciTech Connect

    James Davidson

    2005-01-01

    A diagram provided in the report depicts the complexity of the power systems control architecture used by the national power structure. It shows the structural hierarchy and the relationship of the each system to those other systems interconnected to it. Each of these levels provides a different focus for vulnerability testing and has its own weaknesses. In evaluating each level, of prime concern is what vulnerabilities exist that provide a path into the system, either to cause the system to malfunction or to take control of a field device. An additional vulnerability to consider is can the system be compromised in such a manner that the attacker can obtain critical information about the system and the portion of the national power structure that it controls.

  4. Space Nuclear Power Systems

    NASA Technical Reports Server (NTRS)

    Houts, Michael G.

    2012-01-01

    Fission power and propulsion systems can enable exciting space exploration missions. These include bases on the moon and Mars; and the exploration, development, and utilization of the solar system. In the near-term, fission surface power systems could provide abundant, constant, cost-effective power anywhere on the surface of the Moon or Mars, independent of available sunlight. Affordable access to Mars, the asteroid belt, or other destinations could be provided by nuclear thermal rockets. In the further term, high performance fission power supplies could enable both extremely high power levels on planetary surfaces and fission electric propulsion vehicles for rapid, efficient cargo and crew transfer. Advanced fission propulsion systems could eventually allow routine access to the entire solar system. Fission systems could also enable the utilization of resources within the solar system.

  5. Multisource energy system project

    NASA Astrophysics Data System (ADS)

    Dawson, R. W.; Cowan, R. A.

    1987-03-01

    The mission of this project is to investigate methods of providing uninterruptible power to Army communications and navigational facilities, many of which have limited access or are located in rugged terrain. Two alternatives are currently available for deploying terrestrial stand-alone power systems: (1) conventional electric systems powered by diesel fuel, propane, or natural gas, and (2) alternative power systems using renewable energy sources such as solar photovoltaics (PV) or wind turbines (WT). The increased cost of fuels for conventional systems and the high cost of energy storage for single-source renewable energy systems have created interest in the hybrid or multisource energy system. This report will provide a summary of the first and second interim reports, final test results, and a user's guide for software that will assist in applying and designing multi-source energy systems.

  6. Pellet feed system

    SciTech Connect

    Whitfield, O.J.

    1990-08-07

    This patent describes an improvement in a stoker assembly for a solid particulate fuel burning stove. The stove including a combustion chamber, at least one remote substantially airtight fuel storage bin and a conveyor system for transporting fuel from the fuel storage bin to the combustion chamber. The improvement comprises: a conduit separate from the conveyor system, the conduit communicating between the combustion chamber and the fuel storage bin for minimizing the pressure difference along the conveyor system, such that oxygen supplied to the conveyor system is insufficient to support combustion of fuel in the conveyor system, the conduit being positioned above the conveyor system, the conveyor system including an auger and a casing for the auger.

  7. Information systems vulnerability: A systems analysis perspective

    SciTech Connect

    Wyss, G.D.; Daniel, S.L.; Schriner, H.K.; Gaylor, T.R.

    1996-07-01

    Vulnerability analyses for information systems are complicated because the systems are often geographically distributed. Sandia National Laboratories has assembled an interdisciplinary team to explore the applicability of probabilistic logic modeling (PLM) techniques (including vulnerability and vital area analysis) to examine the risks associated with networked information systems. The authors have found that the reliability and failure modes of many network technologies can be effectively assessed using fault trees and other PLM methods. The results of these models are compatible with an expanded set of vital area analysis techniques that can model both physical locations and virtual (logical) locations to identify both categories of vital areas simultaneously. These results can also be used with optimization techniques to direct the analyst toward the most cost-effective security solution.

  8. The Planetary Data System Distributed Inventory System

    NASA Technical Reports Server (NTRS)

    Hughes, J. Steven; McMahon, Susan K.

    1996-01-01

    The advent of the World Wide Web (Web) and the ability to easily put data repositories on-line has resulted in a proliferation of digital libraries. The heterogeneity of the underlying systems, the autonomy of the individual sites, and distributed nature of the technology has made both interoperability across the sites and the search for resources within a site major research topics. This article will describe a system that addresses both issues using standard Web protocols and meta-data labels to implement an inventory of on-line resources across a group of sites. The success of this system is strongly dependent on the existence of and adherence to a standards architecture that guides the management of meta-data within participating sites.

  9. Continuous recovery system for electrorefiner system

    DOEpatents

    Williamson, Mark A.; Wiedmeyer, Stanley G.; Willit, James L.; Barnes, Laurel A.; Blaskovitz, Robert J.

    2014-06-10

    A continuous recovery system for an electrorefiner system may include a trough having a ridge portion and a furrow portion. The furrow portion may include a first section and a second section. An inlet and exit pipe may be connected to the trough. The inlet pipe may include an outlet opening that opens up to the first section of the furrow portion of the trough. The exit pipe may include an entrance opening that opens up to the second section of the furrow portion of the trough. A chain may extend through the inlet and exit pipes and along the furrow portion of the trough. The chain may be in a continuous loop form. A plurality of flights may be secured to the chain. Accordingly, the desired product may be continuously harvested from the electrorefiner system without having to halt the electrical power and/or remove the cathode and anode assemblies.

  10. Control system health test system and method

    DOEpatents

    Hoff, Brian D.; Johnson, Kris W.; Akasam, Sivaprasad; Baker, Thomas M.

    2006-08-15

    A method is provided for testing multiple elements of a work machine, including a control system, a component, a sub-component that is influenced by operations of the component, and a sensor that monitors a characteristic of the sub-component. In one embodiment, the method is performed by the control system and includes sending a command to the component to adjust a first parameter associated with an operation of the component. Also, the method includes detecting a sensor signal from the sensor reflecting a second parameter associated with a characteristic of the sub-component and determining whether the second parameter is acceptable based on the command. The control system may diagnose at least one of the elements of the work machine when the second parameter of the sub-component is not acceptable.

  11. Automated Information System (AIS) Alarm System

    SciTech Connect

    Hunteman, W.

    1997-05-01

    The Automated Information Alarm System is a joint effort between Los Alamos National Laboratory, Lawrence Livermore National Laboratory, and Sandia National Laboratory to demonstrate and implement, on a small-to-medium sized local area network, an automated system that detects and automatically responds to attacks that use readily available tools and methodologies. The Alarm System will sense or detect, assess, and respond to suspicious activities that may be detrimental to information on the network or to continued operation of the network. The responses will allow stopping, isolating, or ejecting the suspicious activities. The number of sensors, the sensitivity of the sensors, the assessment criteria, and the desired responses may be set by the using organization to meet their local security policies.

  12. Automated biowaste sampling system feces monitoring system

    NASA Technical Reports Server (NTRS)

    Hunt, S. R.; Glanfield, E. J.

    1979-01-01

    The Feces Monitoring System (FMS) Program designed, fabricated, assembled and tested an engineering model waste collector system (WCS) to be used in support of life science and medical experiments related to Shuttle missions. The FMS design was patterned closely after the Shuttle WCS, including: interface provisions; mounting; configuration; and operating procedures. These similarities make it possible to eventually substitute an FMS for the Shuttle WCS of Orbiter. In addition, several advanced waste collection features, including the capability of real-time inertial fecal separation and fecal mass measurement and sampling were incorporated into the FMS design.

  13. Nemesis Autonomous Test System

    NASA Technical Reports Server (NTRS)

    Barltrop, Kevin J.; Lee, Cin-Young; Horvath, Gregory A,; Clement, Bradley J.

    2012-01-01

    A generalized framework has been developed for systems validation that can be applied to both traditional and autonomous systems. The framework consists of an automated test case generation and execution system called Nemesis that rapidly and thoroughly identifies flaws or vulnerabilities within a system. By applying genetic optimization and goal-seeking algorithms on the test equipment side, a "war game" is conducted between a system and its complementary nemesis. The end result of the war games is a collection of scenarios that reveals any undesirable behaviors of the system under test. The software provides a reusable framework to evolve test scenarios using genetic algorithms using an operation model of the system under test. It can automatically generate and execute test cases that reveal flaws in behaviorally complex systems. Genetic algorithms focus the exploration of tests on the set of test cases that most effectively reveals the flaws and vulnerabilities of the system under test. It leverages advances in state- and model-based engineering, which are essential in defining the behavior of autonomous systems. It also uses goal networks to describe test scenarios.

  14. Paravirtualization for HPC Systems

    SciTech Connect

    Youseff, L; Wolski, R; Gorda, B; Krintz, C

    2006-10-12

    Virtualization has become increasingly popular for enabling full system isolation, load balancing, and hardware multiplexing. This wide-spread use is the result of novel techniques such as paravirtualization that make virtualization systems practical and efficient. Paravirtualizing systems export an interface that is slightly different from the underlying hardware but that significantly streamlines and simplifies the virtualization process. In this work, we investigate the efficacy of using paravirtualizing software for performance-critical HPC kernels and applications. Such systems are not currently employed in HPC environments due to their perceived overhead. However, virtualization systems offer tremendous potential for benefiting HPC systems by facilitating application isolation, portability, operating system customization, and program migration. We present a comprehensive performance evaluation of Xen, a low-overhead, Linux-based, virtual machine monitor (VMM), for paravirtualization of HPC cluster systems at Lawrence Livermore National Lab (LLNL). We consider four categories of micro-benchmarks from the HPC Challenge (HPCC) and LLNL ASCI Purple suites to evaluate a wide range of subsystem-specific behaviors. In addition, we employ macro-benchmarks and HPC application to evaluate overall performance in a real setting. We also employ statistically sound methods to compare the performance of a paravirtualized kernel against three popular Linux operating systems: RedHat Enterprise 4 (RHEL4) for build versions 2.6.9 and 2.6.12 and the LLNL CHAOS kernel, a specialized version of RHEL4. Our results indicate that Xen is very efficient and practical for HPC systems.

  15. Managing hybrid marketing systems.

    PubMed

    Moriarty, R T; Moran, U

    1990-01-01

    As competition increases and costs become critical, companies that once went to market only one way are adding new channels and using new methods - creating hybrid marketing systems. These hybrid marketing systems hold the promise of greater coverage and reduced costs. But they are also hard to manage; they inevitably raise questions of conflict and control: conflict because marketing units compete for customers; control because new indirect channels are less subject to management authority. Hard as they are to manage, however, hybrid marketing systems promise to become the dominant design, replacing the "purebred" channel strategy in all kinds of businesses. The trick to managing the hybrid is to analyze tasks and channels within and across a marketing system. A map - the hybrid grid - can help managers make sense of their hybrid system. What the chart reveals is that channels are not the basic building blocks of a marketing system; marketing tasks are. The hybrid grid forces managers to consider various combinations of channels and tasks that will optimize both cost and coverage. Managing conflict is also an important element of a successful hybrid system. Managers should first acknowledge the inevitability of conflict. Then they should move to bound it by creating guidelines that spell out which customers to serve through which methods. Finally, a marketing and sales productivity (MSP) system, consisting of a central marketing database, can act as the central nervous system of a hybrid marketing system, helping managers create customized channels and service for specific customer segments.

  16. Modeling Sustainable Food Systems

    NASA Astrophysics Data System (ADS)

    Allen, Thomas; Prosperi, Paolo

    2016-05-01

    The processes underlying environmental, economic, and social unsustainability derive in part from the food system. Building sustainable food systems has become a predominating endeavor aiming to redirect our food systems and policies towards better-adjusted goals and improved societal welfare. Food systems are complex social-ecological systems involving multiple interactions between human and natural components. Policy needs to encourage public perception of humanity and nature as interdependent and interacting. The systemic nature of these interdependencies and interactions calls for systems approaches and integrated assessment tools. Identifying and modeling the intrinsic properties of the food system that will ensure its essential outcomes are maintained or enhanced over time and across generations, will help organizations and governmental institutions to track progress towards sustainability, and set policies that encourage positive transformations. This paper proposes a conceptual model that articulates crucial vulnerability and resilience factors to global environmental and socio-economic changes, postulating specific food and nutrition security issues as priority outcomes of food systems. By acknowledging the systemic nature of sustainability, this approach allows consideration of causal factor dynamics. In a stepwise approach, a logical application is schematized for three Mediterranean countries, namely Spain, France, and Italy.

  17. Distributed Deliberative Recommender Systems

    NASA Astrophysics Data System (ADS)

    Recio-García, Juan A.; Díaz-Agudo, Belén; González-Sanz, Sergio; Sanchez, Lara Quijano

    Case-Based Reasoning (CBR) is one of most successful applied AI technologies of recent years. Although many CBR systems reason locally on a previous experience base to solve new problems, in this paper we focus on distributed retrieval processes working on a network of collaborating CBR systems. In such systems, each node in a network of CBR agents collaborates, arguments and counterarguments its local results with other nodes to improve the performance of the system's global response. We describe D2ISCO: a framework to design and implement deliberative and collaborative CBR systems that is integrated as a part of jcolibritwo an established framework in the CBR community. We apply D2ISCO to one particular simplified type of CBR systems: recommender systems. We perform a first case study for a collaborative music recommender system and present the results of an experiment of the accuracy of the system results using a fuzzy version of the argumentation system AMAL and a network topology based on a social network. Besides individual recommendation we also discuss how D2ISCO can be used to improve recommendations to groups and we present a second case of study based on the movie recommendation domain with heterogeneous groups according to the group personality composition and a group topology based on a social network.

  18. Man-systems distributed system for Space Station Freedom

    NASA Technical Reports Server (NTRS)

    Lewis, J. L.

    1990-01-01

    Viewgraphs on man-systems distributed system for Space Station Freedom are presented. Topics addressed include: description of man-systems (definition, requirements, scope, subsystems, and topologies); implementation (approach, tools); man-systems interfaces (system to element and system to system); prime/supporting development relationship; selected accomplishments; and technical challenges.

  19. Power system commonality study

    NASA Astrophysics Data System (ADS)

    Littman, Franklin D.

    1992-07-01

    A limited top level study was completed to determine the commonality of power system/subsystem concepts within potential lunar and Mars surface power system architectures. A list of power system concepts with high commonality was developed which can be used to synthesize power system architectures which minimize development cost. Examples of potential high commonality power system architectures are given in this report along with a mass comparison. Other criteria such as life cycle cost (which includes transportation cost), reliability, safety, risk, and operability should be used in future, more detailed studies to select optimum power system architectures. Nineteen potential power system concepts were identified and evaluated for planetary surface applications including photovoltaic arrays with energy storage, isotope, and nuclear power systems. A top level environmental factors study was completed to assess environmental impacts on the identified power system concepts for both lunar and Mars applications. Potential power system design solutions for commonality between Mars and lunar applications were identified. Isotope, photovoltaic array (PVA), regenerative fuel cell (RFC), stainless steel liquid-metal cooled reactors (less than 1033 K maximum) with dynamic converters, and in-core thermionic reactor systems were found suitable for both lunar and Mars environments. The use of SP-100 thermoelectric (TE) and SP-100 dynamic power systems in a vacuum enclosure may also be possible for Mars applications although several issues need to be investigated further (potential single point failure of enclosure, mass penalty of enclosure and active pumping system, additional installation time and complexity). There are also technical issues involved with development of thermionic reactors (life, serviceability, and adaptability to other power conversion units). Additional studies are required to determine the optimum reactor concept for Mars applications. Various screening

  20. A contemporary view of systems engineering. [definition of system and discussion of systems approach

    NASA Technical Reports Server (NTRS)

    Miles, R. F., Jr.

    1974-01-01

    The concept of a 'system' is defined, and the 'systems approach' is discussed. Four contemporary examples of the systems approach are presented: an operations research project, the planning-programming-budgeting system, an information processing system, and aerospace programs.