Science.gov

Sample records for mammo-tomography cmt system

  1. A design for a linear array PIN photodiode for use in a Computed mammo-Tomography (CmT) system

    NASA Astrophysics Data System (ADS)

    Park, Shin-Woong; Yuk, Sunwoo; Park, Jung-Byung; Yi, Yun

    2009-10-01

    A p-i-n (PIN) photodiode has been used in a solid-state detector for X-ray detection as a photosensor of visible light from the scintillator. The most sensitive material used as low-energy X-ray detector in the mammography system is a Gd 2O 2S (GOS). As the light from GOS having a short wavelength in the range of 450-700 nm (peak at 510 nm) is absorbed within a very shallow layer near the surface of photodiode before arriving at depletion region and does not contribute to the signal. For designing the PIN photodiode, it is important to make p-layer as shallow as possible. In order to achieve shallow junction, the optimum conditions of ion implantation such as thickness of SiO 2 oxide barrier, tilting angle of the wafer with respect to incident ion beam, and annealing conditions, have been determined using simulation results. The penetration depths are about 2 μm for 510 nm, and 7 μm for 700 nm. It is necessary for adequate depletion depth (about 10 μm) to acquire the entire incident light. So far, wafers of ≥1000 and ≥150 Ω cm resistivity were chosen, which offer about 15 and 6 μm depletion depth, respectively. The pixel pitch of photodiode is 0.4 mm×3.0 mm and one module has 64 channels in linear array. Depth of the active p-layer is under 0.3 μm in zero bias. Measured leakage currents under 10 pA/mm 2 for both diodes and junction capacitances are 16 and 29 pF/mm 2 in zero bias for the diodes of ≥150 and ≥1000 Ω cm resistivity, respectively. The breast phantom, which was scanned by the Computed mammo-Tomography (CmT) system with two different detector modules and the data acquisition system, was developed. Little differences for distinct light absorption were shown in the three-dimensional images acquired in this study.

  2. CMT1

    MedlinePlus

    ... 1 is the most common subtype of CMT, accounting for roughly two-thirds of all cases. CMT1 ... CMT Signs and Symptoms Diagnosis Causes/Inheritance Medical Management Research Living With Charcot-Marie-Tooth Disease (CMT) ...

  3. Cytotoxic activity and inhibition of tumor cell invasion by derivatives of a chemically modified tetracycline CMT-3 (COL-3).

    PubMed

    Lokeshwar, B L; Escatel, E; Zhu, B

    2001-02-01

    Tetracyclines such as chlortetracycline and doxycycline with antimicrobial activity were reported to possess cytostatic and cytotoxic activity against mammalian tumor cells, often at high doses. Non-antimicrobial chemically modified tetracyclines (CMTs), with limited systemic toxicity but with significant tumor cell toxicity and antimetastatic activity, are attractive for long term treatment for cancer. We recently reported one such CMT, 6-deoxy,6-demethyl 4-dedimethylamino tetracycline (CMT-3) is a potent anti-tumor and anti-metastatic drug. Here we report on the anti-cell proliferation and anti-invasive activity of five nitro derivatives of CMT-3 (CMT-3N). All the five CMT-3Ns (CMT-302, CMT-303, CMT-306, CMT-308 and CMT-316) inhibited in vitro cell proliferation of prostate cancer cells. The 50% growth inhibition concentration (IC(50)) of CMT-3Ns was similar to that of CMT-3. Although CMT-3 was by far the most potent anti-cell proliferation drug, all CMT-3Ns except CMT-303 and CMT-308 had similar anti-cell proliferation activity (IC(50): 2.5 -5.7 microg/ml). IC(50)s for CMT-303 and CMT-308 were approximately 8.1 and -12.4 microg/ml, respectively. Activity against tumor cell invasion was tested in vitro using the Matrigel invasion assay. All CMT-3Ns had similar anti- invasive activity. While cytotoxic activity of CMT-3 was strongly associated with cell death-effector caspase activation, mitochondrial permeablization and apoptosis, the CMT-3Ns weakly induced apoptosis and did not activate Caspase-3. However, the CMT-3Ns were able to induce mitochondrial permeabilization. This dichotomous mechanism of cytotoxic activity of CMTs may have significance in their selection for clinical application. PMID:11172682

  4. 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

  5. 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.

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

    PubMed Central

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

    2014-01-01

    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. PMID:24705285

  7. 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.

  8. 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.

  9. Identification of Drug Modulators Targeting Gene-Dosage Disease CMT1A

    PubMed Central

    Jang, Sung-Wook; Lopez-Anido, Camila; MacArthur, Ryan; Svaren, John; Inglese, James

    2012-01-01

    The structural integrity of myelin formed by Schwann cells in the peripheral nervous system (PNS) is required for proper nerve conduction and is dependent on adequate expression of myelin genes including peripheral myelin protein 22 (PMP22). Consequently, excess PMP22 resulting from its genetic duplication and overexpression has been directly associated with the peripheral neuropathy called Charcot-Marie-Tooth disease type 1A (CMT1A), the most prevalent type of CMT. Here, in an attempt to identify transcriptional inhibitors with therapeutic value towards CMT1A, we developed a cross-validating pair of orthogonal reporter - firefly luciferase (FLuc) and β-lactamase (βLac) - assays capable of recapitulating PMP22 expression, utilizing the intronic regulatory element of the human PMP22 gene. Each compound from a collection of approximately 3,000 approved drugs was tested at multiple titration points to achieve a pharmacological endpoint in a 1536-well plate quantitative high-throughput screen (qHTS) format. In conjunction with an independent counter-screen for cytotoxicity, the design of our orthogonal screen platform effectively contributed to selection and prioritization of active compounds, among which three drugs (fenretinide, olvanil, and bortezomib) exhibited marked reduction of endogenous Pmp22 mRNA and protein. Overall, the findings of this study provide a strategic approach to assay development for gene-dosage diseases such as CMT1A. PMID:22530759

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

    MedlinePlus

    ... The peripheral nerve fibers, called axons , extend from sensory nerve cells in the body's periphery back toward ... which explains why CMT mostly causes motor and sensory problems in the body’s extremities. Inheritance patterns in ...

  11. Smart-actuated continuous moldline technology (CMT) mini wind tunnel test

    NASA Astrophysics Data System (ADS)

    Pitt, Dale M.; Dunne, James P.; Kilian, Kevin J.

    1999-07-01

    The Smart Aircraft and Marine Propulsion System Demonstration (SAMPSON) Program will culminate in two separate demonstrations of the application of Smart Materials and Structures technology. One demonstration will be for an aircraft application and the other for marine vehicles. The aircraft portion of the program will examine the application of smart materials to aircraft engine inlets which will deform the inlet in-flight in order to regulate the airflow rate into the engine. Continuous Moldline Technology (CMT), a load-bearing reinforced elastomer, will enable the use of smart materials in this application. The capabilities of CMT to withstand high-pressure subsonic and supersonic flows were tested in a sub-scale mini wind- tunnel. The fixture, used as the wind-tunnel test section, was designed to withstand pressure up to 100 psi. The top and bottom walls were 1-inch thick aluminum and the side walls were 1-inch thick LEXAN. High-pressure flow was introduced from the Boeing St. Louis poly-sonic wind tunnel supply line. CMT walls, mounted conformal to the upper and lower surfaces, were deflected inward to obtain a converging-diverging nozzle. The CMT walls were instrumented for vibration and deflection response. Schlieren photography was used to establish shock wave motion. Static pressure taps, embedded within one of the LEXAN walls, monitored pressure variation in the mini-wind tunnel. High mass flow in the exit region. This test documented the response of CMT technology in the presence of high subsonic flow and provided data to be used in the design of the SAMPSON Smart Inlet.

  12. Computational Analysis Reveals the Association of Threonine 118 Methionine Mutation in PMP22 Resulting in CMT-1A

    PubMed Central

    Swetha, Rayapadi G.

    2014-01-01

    The T118M mutation in PMP22 gene is associated with Charcot Marie Tooth, type 1A (CMT1A). CMT1A is a form of Charcot-Marie-Tooth disease, the most common inherited disorder of the peripheral nervous system. Mutations in CMT related disorder are seen to increase the stability of the protein resulting in the diseased state. We performed SNP analysis for all the nsSNPs of PMP22 protein and carried out molecular dynamics simulation for T118M mutation to compare the stability difference between the wild type protein structure and the mutant protein structure. The mutation T118M resulted in the overall increase in the stability of the mutant protein. The superimposed structure shows marked structural variation between the wild type and the mutant protein structures. PMID:25400662

  13. 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.

  14. Nonrelativistic limit of the abelianized ABJM model and the ADS/CMT correspondence

    NASA Astrophysics Data System (ADS)

    Lopez-Arcos, Cristhiam; Murugan, Jeff; Nastase, Horatiu

    2016-05-01

    We consider the nonrelativistic limit of the abelian reduction of the massive ABJM model proposed in [1], obtaining a supersymmetric version of the Jackiw-Pi model. The system exhibits an N=2 Super-Schrödinger symmetry with the Jackiw-Pi vortices emerging as BPS solutions. We find that this (2 + 1)-dimensional abelian field theory is dual to a certain (3+1)-dimensional gravity theory that differs somewhat from previously considered abelian condensed matter stand-ins for the ABJM model. We close by commenting on progress in the top-down realization of the AdS/CMT correspondence in a critical string theory.

  15. Use of 2-D cadmium mercury teluride (CMT) IRFPA for seeker applications

    NASA Astrophysics Data System (ADS)

    Bertrand, Francis; Riviere, Philippe; Migaud, Pascal; Chatard, Jean-Pierre

    1994-10-01

    For a wide variety of applications, imaging infrared seeker allows new generation weapon systems to reach high performance in terms of accuracy, standoff capability, target/false target discrimination, target recognition, etc. In the design of the seeker, the detector is a key component because the global performance is strongly related to the detector's and also because it has a large impact on the opto-mechanical concept. Thanks to the use of state-of- the-art CMT 2D IRFPA, new generation imaging infrared seeker (IIS) has very high performance (sensitivity, resolution, number of pixels) as well as a simple opto-mechanical architecture. Thomson-CSF Missile Electronics Division (DEM) has designed, manufactured, and tested breadboards of IIS with the support of French MoD. These IIS are based on 2D IRFPA provided by Sofradir under DEM requirements. The IRFPA selected for this development is a 128 X 128 CMT IRCCD sensitive in the 3-5 micrometers waveband integrated in an operational dewar cooled by a Joule-Thomson cooler. In this paper, first a technical description of the IR detector is given, then the electronic set developed is described, and finally the measured main figures are given.

  16. Charcot Marie Tooth disease (CMT): historical perspectives and evolution.

    PubMed

    Kazamel, Mohamed; Boes, Christopher J

    2015-01-01

    Prior to Charcot and Marie's and Tooth's reports, patients with peroneal muscular atrophy had been described by Virchow, Eulenburg, Friedreich, Osler, and others. In February 1886, Charcot and Marie published their original description of five patients who had what they called Progressive Muscular Atrophy. They surmised that the lesion could be in the spinal cord. Three months later, Tooth presented his M.D. degree thesis entitled "Peroneal Type of Progressive Muscular Atrophy", to the University of Cambridge, UK. Tooth localized the pathology to the peripheral nerves. Dyck and Lambert (Arch Neurol 18:619-625, 1968) classified several CMT kinships based on differences in modes of inheritance, natural history, biochemical features, nerve conduction velocity, and pathologic characteristics. This article will focus on historical landmarks and major discoveries pertinent to the disease since its original description through the second half of the twentieth century. PMID:25201224

  17. 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.

  18. 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.

  19. 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.

  20. Charcot-Marie-Tooth (CMT) disease 1A with superimposed inflammatory polyneuropathy in children.

    PubMed

    Desurkar, A; Lin, J-P; Mills, K; Al-Sarraj, S; Jan, W; Jungbluth, H; Wraige, E

    2009-04-01

    Charcot-Marie-Tooth (CMT) disease is genetically heterogeneous and subdivided into demyelinating (CMT 1) and axonal (CMT 2) types based on neurophysiology findings. CMT1A, the commonest form associated with duplication of the PMP22 segment on chromosome 17p, often arises in childhood but is generally a slowly progressive disease. We report 2 children presenting with clinical features of an acute inflammatory demyelinating polyneuropathy (AIDP) who were subsequently diagnosed with underlying CMT1A. Both children had neurophysiology and histopathology features consistent with CMT1. Immunoglobulin treatment was initiated considering the evidence of superimposed inflammation and appeared to modify disease progression. Our findings indicate that CMT1A predisposes to a superimposed inflammatory neuropathy. Recognition of this association is difficult, particularly in children without clear family history, but of great importance as immunomodulatory treatment may improve outcome. In addition, we postulate that an underlying genetic polyneuropathy should be suspected if the recovery from AIDP is slower than expected, or incomplete. PMID:19809938

  1. 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. PMID:10586249

  2. PMP22 expression in dermal nerve myelin from patients with CMT1A.

    PubMed

    Katona, Istvan; Wu, Xingyao; Feely, Shawna M E; Sottile, Stephanie; Siskind, Carly E; Miller, Lindsey J; Shy, Michael E; Li, Jun

    2009-07-01

    Charcot-Marie-Tooth disease type 1A (CMT1A) is caused by a 1.4 Mb duplication on chromosome 17p11.2, which contains the peripheral myelin protein-22 (PMP22) gene. Increased levels of PMP22 in compact myelin of peripheral nerves have been demonstrated and presumed to cause the phenotype of CMT1A. The objective of the present study was to determine whether an extra copy of the PMP22 gene in CMT1A disrupts the normally coordinated expression of PMP22 protein in peripheral nerve myelin and to evaluate PMP22 over-expression in patients with CMT1A and determine whether levels of PMP22 are molecular markers of disease severity. PMP22 expression was measured by taking skin biopsies from patients with CMT1A (n = 20) and both healthy controls (n = 7) and patients with Hereditary Neuropathy with liability to Pressure Palsies (HNPP) (n = 6), in which patients have only a single copy of PMP22. Immunological electron microscopy was performed on the skin biopsies to quantify PMP22 expression in compact myelin. Similar biopsies were analysed by real time PCR to measure PMP22 mRNA levels. Results were also correlated with impairment in CMT1A, as measured by the validated CMT Neuropathy Score. Most, but not all patients with CMT1A, had elevated PMP22 levels in myelin compared with the controls. The levels of PMP22 in CMT1A were highly variable, but not in HNPP or the controls. However, there was no correlation between neurological disabilities and the level of over-expression of PMP22 protein or mRNA in patients with CMT1A. The extra copy of PMP22 in CMT1A results in disruption of the tightly regulated expression of PMP22. Thus, variability of PMP22 levels, rather than absolute level of PMP22, may play an important role in the pathogenesis of CMT1A. PMID:19447823

  3. PMP22 expression in dermal nerve myelin from patients with CMT1A

    PubMed Central

    Katona, Istvan; Wu, Xingyao; Feely, Shawna M. E.; Sottile, Stephanie; Siskind, Carly E.; Miller, Lindsey J.; Shy, Michael E.

    2009-01-01

    Charcot-Marie-Tooth disease type 1A (CMT1A) is caused by a 1.4 Mb duplication on chromosome 17p11.2, which contains the peripheral myelin protein-22 (PMP22) gene. Increased levels of PMP22 in compact myelin of peripheral nerves have been demonstrated and presumed to cause the phenotype of CMT1A. The objective of the present study was to determine whether an extra copy of the PMP22 gene in CMT1A disrupts the normally coordinated expression of PMP22 protein in peripheral nerve myelin and to evaluate PMP22 over-expression in patients with CMT1A and determine whether levels of PMP22 are molecular markers of disease severity. PMP22 expression was measured by taking skin biopsies from patients with CMT1A (n = 20) and both healthy controls (n = 7) and patients with Hereditary Neuropathy with liability to Pressure Palsies (HNPP) (n = 6), in which patients have only a single copy of PMP22. Immunological electron microscopy was performed on the skin biopsies to quantify PMP22 expression in compact myelin. Similar biopsies were analysed by real time PCR to measure PMP22 mRNA levels. Results were also correlated with impairment in CMT1A, as measured by the validated CMT Neuropathy Score. Most, but not all patients with CMT1A, had elevated PMP22 levels in myelin compared with the controls. The levels of PMP22 in CMT1A were highly variable, but not in HNPP or the controls. However, there was no correlation between neurological disabilities and the level of over-expression of PMP22 protein or mRNA in patients with CMT1A. The extra copy of PMP22 in CMT1A results in disruption of the tightly regulated expression of PMP22. Thus, variability of PMP22 levels, rather than absolute level of PMP22, may play an important role in the pathogenesis of CMT1A. PMID:19447823

  4. Developmental- and Tissue-Specific Expression of NbCMT3-2 Encoding a Chromomethylase in Nicotiana benthamiana.

    PubMed

    Lin, Yu-Ting; Wei, Huei-Mei; Lu, Hsueh-Yu; Lee, Yung-I; Fu, Shih-Feng

    2015-06-01

    The chromomethylase (CMT) protein family is unique to plants and controls non-CpG methylation. Here, we investigated the developmental expression of CMT3-2 in Nicotiana benthamiana (NbCMT3-2) and its significance by analyzing plants with silenced NbCMT3-2 and leaf tissues transiently expressing the N-terminal polypeptide. Alignment of the NbCMT3-2 amino acid sequence with that of other plant CMT3s showed a specific N-terminal extension required for nuclear localization. Transient expression of the N-terminal polypeptide in N. benthamiana resulted in chlorotic lesions. NbCMT3-2 was expressed mainly in proliferating tissues such as the shoot apex and developing leaves. We generated transgenic N. benthamiana harboring a fusion reporter construct linking the NbCMT3-2 promoter region and the β-glucuronidase (GUS) reporter (pNbCMT3-2::GUS) to analyze the tissue-specific expression of NbCMT3-2. NbCMT3-2 was expressed in the shoot and root apical meristem and leaf primordia in young seedlings and highly expressed in developing leaves and ovary as well as lateral buds in mature plants. Virus-induced gene silencing used to knock down the expression of NbCMT3 or NbCMT3-2 or both led to partial loss of genomic DNA methylation. Plants with suppressed NbCMT3 expression grew and developed normally, whereas leaves with NbCMT3-2 knockdown showed mild curling as compared with controls. Silencing NbCMT3/3-2 severely interfered with leaf development and directly or indirectly affected the expression of genes involved in jasmonate homeostasis. The differential roles of NbCMT3 and NbCMT3-2 were investigated and compared. We reveal the expression patterns of NbCMT3-2 in proliferating tissues. NbCMT3-2 may play an essential role in leaf development by modulating jasmonate pathways. PMID:25745030

  5. MFN2-related genetic and clinical features in a cohort of Chinese CMT2 patients.

    PubMed

    Xie, Yongzhi; Li, Xiaobo; Liu, Lei; Hu, Zhengmao; Huang, Shunxiang; Zhan, Yajin; Zi, Xiaohong; Xia, Kun; Tang, Beisha; Zhang, Ruxu

    2016-03-01

    Charcot-Marie-Tooth disease 2A (CMT2A), caused by mutations in the mitofusin 2 gene (MFN2), is the most common CMT2 subtype. The aim of our study is to assess the frequency and summarize the genetic and clinical characteristics of Chinese CMT2A patients. A total of 17 coding exons of MFN2 were detected by direct sequencing in 82 unrelated Chinese families diagnosed as CMT2. Clinical evaluations were analyzed among CMT2A patients. We identified 14 missense variants in 9 sporadic and 6 familial cases, including four novel mutations (T129A, S249F, Q367P, and Q674L), 4 known mutations (R94W, R94Q, T105M, C132Y, M376V and Q751X), and 4 rare missense variants (K120E, C217F, K307E and T356S). A total of 23 patients had early-onset phenotype. Two patients had a CMTNS score of 0 to 10; 16 had a score of 11 to 20; and 7 had a score greater than 20. Five patients were confirmed a de novo origin. Six of 14 variants were located or closed to the GTPase domain. We report four novel mutations and four rare missense variants. MFN2 mutations account for 18% of CMT2 families in mainland China. The common characteristics of Chinese pedigree are early disease onset and moderate phenotypes. PMID:26801520

  6. 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

  7. 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

  8. 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.

  9. 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.

  10. Electronic Processing And Advantages Of CMT Focal Plane Arrays

    NASA Astrophysics Data System (ADS)

    Murphy, Kevin S.; Dennis, Peter N.; Bradley, Derek J.

    1990-04-01

    There have been many advances in thermal imaging systems and components in recent years such that an infrared capability is now readily available and accepted in a variety of military and civilian applications. Conventional thermal imagers such as the UK common module imager use a mechanical scanning system to sweep a small array of detectors across the thermal scene to generate a high definition TV compatible output. Although excellent imagery can be obtained from this type of system, there are some inherent disadvantages, amongst which are the need for a high speed line scan mechanism and the fundamental limit in thermal resolution due to the low stare efficiency of the system. With the advent of two dimensional focal plane array detectors, staring array imagers can now be designed and constructed in which the scanning mechanism is removed. Excellent thermal resolution can be obtained from such imagers due to the relatively long stare times. The recent progress in this technology will be discussed in this paper together with a description of the signal processing requirements of this type of imaging system.

  11. CMT data inversion using a Bayesian information criterion to estimate seismogenic stress fields

    NASA Astrophysics Data System (ADS)

    Terakawa, Toshiko; Matsu'ura, Mitsuhiro

    2008-02-01

    We developed an inversion method to estimate the stress fields related to earthquake generation (seismogenic stress fields) from the centroid moment tensors (CMT) of seismic events by using Akaike's Bayesian information criterion (ABIC). On the idea that the occurrence of an earthquake releases some part of the seismogenic stress field around its hypocentre, we define the CMT of a seismic event by a weighted volume integral of the true but unknown seismogenic stress field. Representing each component of the seismogenic stress field by the superposition of a finite number of 3-D basis functions (tri-cubic B-splines), we obtain a set of linear observation equations to be solved for the expansion coefficients (model parameters). We introduce prior constraint on the roughness of the seismogenic stress field and combine it with observed data to construct a Bayesian model with hierarchic, highly flexible structure controlled by hyper-parameters. The optimum values of the hyper-parameters are objectively determined form observed data by using ABIC. Given the optimum values of the hyper-parameters, we can obtain the best estimates of model parameters by using a maximum likelihood algorithm. We tested the validity of the inversion method through numerical experiments on two synthetic CMT data sets, assuming the distribution of fault orientations to be aligned with the maximum shear stress plane in one case and to be random in the other case. Then we applied the inversion method to actual CMT data in northeast Japan, and obtained the pattern of the seismogenic stress field consistent with geophysical and geological observations.

  12. 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.

  13. CMT subtypes and disease burden in patients enrolled in the Inherited Neuropathies Consortium natural history study: a cross-sectional analysis

    PubMed Central

    Fridman, V; Bundy, B; Reilly, M M; Pareyson, D; Bacon, C; Burns, J; Day, J; Feely, S; Finkel, R S; Grider, T; Kirk, C A; Herrmann, D N; Laurá, M; Li, J; Lloyd, T; Sumner, C J; Muntoni, F; Piscosquito, G; Ramchandren, S; Shy, R; Siskind, C E; Yum, S W; Moroni, I; Pagliano, E; Zuchner, S; Scherer, S S; Shy, M E

    2015-01-01

    Background The international Inherited Neuropathy Consortium (INC) was created with the goal of obtaining much needed natural history data for patients with Charcot-Marie-Tooth (CMT) disease. We analysed clinical and genetic data from patients in the INC to determine the distribution of CMT subtypes and the clinical impairment associated with them. Methods We analysed data from 1652 patients evaluated at 13 INC centres. The distribution of CMT subtypes and pathogenic genetic mutations were determined. The disease burden of all the mutations was assessed by the CMT Neuropathy Score (CMTNS) and CMT Examination Score (CMTES). Results 997 of the 1652 patients (60.4%) received a genetic diagnosis. The most common CMT subtypes were CMT1A/PMP22 duplication, CMT1X/GJB1 mutation, CMT2A/MFN2 mutation, CMT1B/MPZ mutation, and hereditary neuropathy with liability to pressure palsy/PMP22 deletion. These five subtypes of CMT accounted for 89.2% of all genetically confirmed mutations. Mean CMTNS for some but not all subtypes were similar to those previously reported. Conclusions Our findings confirm that large numbers of patients with a representative variety of CMT subtypes have been enrolled and that the frequency of achieving a molecular diagnosis and distribution of the CMT subtypes reflects those previously reported. Measures of severity are similar, though not identical, to results from smaller series. This study confirms that it is possible to assess patients in a uniform way between international centres, which is critical for the planned natural history study and future clinical trials. These data will provide a representative baseline for longitudinal studies of CMT. Clinical trial registration ID number NCT01193075. PMID:25430934

  14. Effects of three-dimensional Earth structure on CMT earthquake parameters

    NASA Astrophysics Data System (ADS)

    Hjörleifsdóttir, Vala; Ekström, Göran

    2010-04-01

    We investigate errors in centroid earthquake parameters due to unmodeled structural heterogeneity. We generate a simulated dataset consisting of synthetic seismograms for 50 earthquakes and 150 stations distributed globally. To generate the synthetic seismograms we use a spectral-element wave-propagation package (SPECFEM3D_GLOBE) that accounts for the Earth's three-dimensional structure. An established centroid-moment-tensor inversion algorithm from the Global CMT project is used to invert the synthetic dataset, with and without added noise, for earthquake source parameters. This algorithm uses a one-dimensional earth structure, together with approximate corrections for three-dimensional structure, to model the seismograms. We interpret the differences between the estimated source parameters and the parameters used to compute the synthetic dataset as errors due to unmodeled structural heterogeneity and the presence of noise. We expect that the errors obtained in this study are representative of the errors in the Global CMT catalogue. We find that the errors in scalar moment, moment-tensor elements and location are small on average. The depth and centroid time are, however, biased by a small amount. We find that the error in depth can be reduced significantly by applying corrections for the difference in the velocity structure at the source and receiver locations from the Earth's average structure in the CMT inversions. This modification has a minimal effect on the errors in centroid time. We do not find large errors in scalar moments, even where the crustal thickness at the source is very different from the Earth's average crustal thickness.

  15. Exonic duplication CNV of NDRG1 associated with autosomal-recessive HMSN-Lom/CMT4D

    PubMed Central

    Pehlivan, Davut; Beck, Christine R.; Gonzaga-Jauregui, Claudia; Muzny, Donna M.; Atik, Mehmed M.; Carvalho, Claudia M.B.; Matur, Zeliha; Bayraktar, Serife; Boone, Philip M.; Akyuz, Kaya; Gibbs, Richard A.; Battaloglu, Esra; Parman, Yesim; Lupski, James R.

    2014-01-01

    Purpose Copy-number variations as a mutational mechanism contribute significantly to human disease. Approximately one-half of the patients with Charcot–Marie–Tooth (CMT) disease have a 1.4 Mb duplication copy-number variation as the cause of their neuropathy. However, non-CMT1A neuropathy patients rarely have causative copy-number variations, and to date, autosomal-recessive CMT disease has not been associated with copy-number variation as a mutational mechanism. Methods We performed Agilent 8 × 60K array comparative genomic hybridization on DNA from 12 recessive Turkish families with CMT disease. Additional molecular studies were conducted to detect breakpoint junctions and to evaluate gene expression levels in a family in which we detected an intragenic duplication copy-number variation. Results We detected an ~6.25 kb homozygous intragenic duplication in NDRG1, a gene known to be causative for recessive HMSNL/CMT4D, in three individuals from a Turkish family with CMT neuropathy. Further studies showed that this intragenic copy-number variation resulted in a homozygous duplication of exons 6–8 that caused decreased mRNA expression of NDRG1. Conclusion Exon-focused high-resolution array comparative genomic hybridization enables the detection of copy-number variation carrier states in recessive genes, particularly small copy-number variations encompassing or disrupting single genes. In families for whom a molecular diagnosis has not been elucidated by conventional clinical assays, an assessment for copy-number variations in known CMT genes might be considered. PMID:24136616

  16. 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.

  17. 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

  18. Non-radioactive detection of 17p11.2 duplication in CMT1A: a study of 78 patients.

    PubMed Central

    Schiavon, F; Mostacciuolo, M L; Saad, F; Merlini, L; Siciliano, G; Angelini, C; Danieli, G A

    1994-01-01

    Charcot-Marie-Tooth disease type 1 (CMT1) is a peripheral neuropathy characterised by progressive distal muscular atrophy and sensory loss with markedly decreased nerve conduction velocity, mostly inherited as an autosomal dominant trait. The most common form, type 1A, is associated with a 1.5Mb DNA duplication in region p11.2-p12 of chromosome 17 in many patients. In this study a non-radioactive test for detection of the CMT1A duplication based on an RM11-GT microsatellite polymorphism is presented. Although different methods have been devised for this purpose, the present method has the advantage of being rapid, informative, economical, easily interpretable, and, therefore, it represents a very useful tool for diagnosis of CMT1A, especially before clear manifestation of clinical symptoms. Seventy-eight patients diagnosed clinically as having CMT and evaluated by electrophysiological methods were tested with an RM11-GT microsatellite and with probe pVAW409R3. The CMT1A duplication was found in 76% of the 56 unrelated patients. RM11-GT was the most informative marker with a heterozygosity of 89%. Images PMID:7853375

  19. Visual appearance and CMT score of foremilk of individual quarters in relation to cell count of cows milked automatically.

    PubMed

    Rasmussen, Morten D; Bjerring, Martin; Skjøth, Flemming

    2005-02-01

    The objectives of the study were: to evaluate the interaction between visual appearance and California mastitis test (CMT) score of the foremilk in relation to the cell count of the milk; to evaluate the consequences of sorting milk according to these criteria; and to explore whether visual appearance and CMT score of foremilk depended on the time interval between milkings. Measuring somatic cell count (SCC) in composite milk only and discarding milk above certain thresholds will not ensure that milk from all cows with visually abnormal foremilk is withheld from delivery. Low thresholds of SCC will reduce the frequency of cows with abnormal milk but increase the discarding of milk from cows with visually normal foremilk. CMT score of foremilk differentiated better between cows with high and low SCC in composite milk than visual inspection of foremilk. CMT scores of foremilk decreased with increasing interval between milkings within cow, whereas the visual appearance was independent of the interval. We propose that visual appearance of the foremilk should be kept as a criterion for sorting milk at time of milking. For test purposes, the use of visual appearance of foremilk for differentiation between normal and abnormal milk has to be done on multiple milkings. Additionally, CMT scoring of foremilk improves correct classification of normal and abnormal quarters and especially when including data from the previous milking. PMID:15747731

  20. Near-regional CMT solution for the September 5, 2012 14:42 Costa Rica earthquake

    NASA Astrophysics Data System (ADS)

    Ronnie, Q.; Jiri, Z.; Sokos, E.

    2013-05-01

    We use acceleration data from the OVSICORI-UNA seismic network for a preliminary moment tensor solution of the event September 5, 2012 Mw 7.6, 14:42:03, Guanacaste, Costa Rica earthquake. The full-waveform inversion was made using the ISOLA code (Sokos and Zahradnik, 2008), with the 1D velocity model of Quintero and Kissling (2001). Acceleration records at 10 stations (43-251 km) and at 0.03 < f < 0.06 Hz were used in the inversion. The vertical grid search, resulting in the CMT solution at the depth of 25-45 km below epicenter, continued with a horizontal 10x10 km grid search of trial sources inland. The best stable CMT solution was found at Lat=10.146°, Lon=-85.478°, i.e. of about 50 km NNE from the OVSICORI epicenter. The centroid time is 14:42:21 UTC, i.e. 18 sec later relative to the location-based origin time; Mw 7.5, source time function duration 10-15 seconds. The nodal plane (strike=330°, dip=30° and rake=129°) is the fault plane, as proved by the H-C consistency test (Zahradnik et al., 2008), and in agreement with geometry of the subduction.

  1. Natural CMT2 Variation Is Associated With Genome-Wide Methylation Changes and Temperature Seasonality

    PubMed Central

    Shen, Xia; De Jonge, Jennifer; Forsberg, Simon K. G.; Pettersson, Mats E.; Sheng, Zheya; Hennig, Lars; Carlborg, Örjan

    2014-01-01

    As Arabidopsis thaliana has colonized a wide range of habitats across the world it is an attractive model for studying the genetic mechanisms underlying environmental adaptation. Here, we used public data from two collections of A. thaliana accessions to associate genetic variability at individual loci with differences in climates at the sampling sites. We use a novel method to screen the genome for plastic alleles that tolerate a broader climate range than the major allele. This approach reduces confounding with population structure and increases power compared to standard genome-wide association methods. Sixteen novel loci were found, including an association between Chromomethylase 2 (CMT2) and temperature seasonality where the genome-wide CHH methylation was different for the group of accessions carrying the plastic allele. Cmt2 mutants were shown to be more tolerant to heat-stress, suggesting genetic regulation of epigenetic modifications as a likely mechanism underlying natural adaptation to variable temperatures, potentially through differential allelic plasticity to temperature-stress. PMID:25503602

  2. 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

  3. 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

  4. 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.

  5. Modeling of high-precision wavefront sensing with new generation of CMT avalanche photodiode infrared detectors.

    PubMed

    Gousset, Silvère; Petit, Cyril; Michau, Vincent; Fusco, Thierry; Robert, Clelia

    2015-12-01

    Near-infrared wavefront sensing allows for the enhancement of sky coverage with adaptive optics. The recently developed HgCdTe avalanche photodiode arrays are promising due to their very low detector noise, but still present an imperfect cosmetic that may directly impact real-time wavefront measurements for adaptive optics and thus degrade performance in astronomical applications. We propose here a model of a Shack-Hartmann wavefront measurement in the presence of residual fixed pattern noise and defective pixels. To adjust our models, a fine characterization of such an HgCdTe array, the RAPID sensor, is proposed. The impact of the cosmetic defects on the Shack-Hartmann measurement is assessed through numerical simulations. This study provides both a new insight on the applicability of cadmium mercury telluride (CMT) avalanche photodiodes detectors for astronomical applications and criteria to specify the cosmetic qualities of future arrays. PMID:26836674

  6. U1 snRNA mis-binding: a new cause of CMT1B.

    PubMed

    Crehalet, Hervé; Latour, Philippe; Bonnet, Véronique; Attarian, Shahram; Labauge, Pierre; Bonello, Nathalie; Bernard, Rafaelle; Millat, Gilles; Rousson, Robert; Bozon, Dominique

    2010-02-01

    We report the molecular characterization of two splice mutations in two different French families affected with a late onset form of Charcot-Marie-Tooth disease type 1B (CMT1B), an autosomal dominant inherited disorder caused by mutations in the myelin protein zero gene. The first substitution, c.306G>A, located in exon 3, does not change the codon p.Val102Val but is co-transmitted with the disease in the first family. The second substitution, c.675+3dup, is an insertion of a T at position +3 of intron 5. To identify the functional impact of these nucleotide changes on splicing and because no RNA sample was available, we used in silico prediction and in vitro splicing assay. Mutation c.306G>A increases the strength of a preexisting cryptic donor site at position c.304 which becomes stronger than the normal donor site of intron 3. This variation creates a sequence that better matches the U1 small nuclear RNA (snRNA) binding consensus, and HeLa cells, transfected with the mutant minigene, produce a truncated exon 3 messenger RNA (mRNA). Mutation c.675+3dup was predicted to abolish the donor site of intron 5, and, indeed, HeLa cells transfected with the mutant minigene completely skip exon 5 from the transcript. The mutated sequence abolishes U1 snRNA binding and co-transfection of a mutated complementary U1 snRNA restored exon 5 inclusion in the mRNA. This work provides valuable information regarding the molecular basis of two forms of late onset of CMT1B, U1 snRNA mis-binding, and provides more evidence that a "silent" polymorphism may be a disease causing mutation. PMID:19475438

  7. The antiprogestins mifepristone and onapristone reduce cell proliferation in the canine mammary carcinoma cell line CMT-U27.

    PubMed

    Guil-Luna, Silvia; Hellmén, Eva; Sánchez-Céspedes, Raquel; Millán, Yolanda; Martín de las Mulas, Juana

    2014-07-01

    Canine mammary tumours (CMTs) represent nearly half of all tumours in female dogs and some 50% have malignant behaviour. Simple epithelial carcinomas have shorter disease free periods after surgery and a higher reduction of the proliferation index reduction after antiprogestin aglepristone treatment in vivo related to the expression of progesterone receptors (PR). These findings make simple carcinomas good candidates for endocrine therapy. To further explore this possibility, the effects of the antiprogestins mifepristone (RU486) and onapristone (ZK299) on cell viability and PR expression of the canine mammary carcinoma cell line isolated from a simple epithelial carcinoma CMT-U27 were studied. Twenty five percent of CMT-U27 control cells expressed PR. RU486 (p<0.05) and ZK299 (p<0.05) reduced the number of viable cells (WST-8 test) at 24h but only the latter treatment reduced significantly PR expression in viable tumour cells at 24h of incubation. The results suggest that both RU486 and ZK299 induce a decrease in the number of viable CMT-U27 tumour cells with different effects on PR expression. The canine mammary carcinoma cell line CMT-U27 is sensitive to the effects of antiprogestins and may serve to further explore the role of these drugs in canine mammary carcinomas. PMID:24500783

  8. 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

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

    PubMed

    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

    2015-01-15

    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

  10. 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. PMID:25342198

  11. The function of the Periaxin gene during nerve repair in a model of CMT4F *

    PubMed Central

    Williams, Anna C; Brophy, Peter J

    2002-01-01

    Mutations in the Periaxin (PRX) gene are known to cause autosomal recessive demyelinating Charcot-Marie-Tooth (CMT4F) and Dejerine-Sottas disease. The pathogenesis of these diseases is not fully understood. However, progress is being made by studying both the periaxin-null mouse, a mouse model of the disease, and the protein–protein interactions of periaxin. L-periaxin is a constituent of the dystroglycan–dystrophin-related protein-2 complex linking the Schwann cell cytoskeleton to the extracellular matrix. Although periaxin-null mice myelinate normally, they develop a demyelinating peripheral neuropathy later in life. This suggests that periaxin is required for the stable maintenance of a normal myelin sheath. We carried out sciatic nerve crushes in 6-week-old periaxin-null mice, and, 6 weeks later, found that although the number of myelinated axons had returned to normal, the axon diameters remained smaller than in the contralateral uncrushed nerve. Not only do periaxin-null mice have more hypermyelinated axons than their wild-type counterparts but they also recapitulate this hypermyelination during regeneration. Therefore, periaxin-null mice can undergo peripheral nerve remyelination, but the regulation of peripheral myelin thickness is disrupted. PMID:12090399

  12. 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

  13. Rapid Centroid Moment Tensor (CMT) Inversion in 3D Earth Structure Model for Earthquakes in Southern California

    NASA Astrophysics Data System (ADS)

    Chen, P.; Lee, E.; Jordan, T. H.; Maechling, P. J.

    2009-12-01

    Accurate and rapid CMT inversion is important for seismic hazard analysis. We have developed an algorithm for very rapid CMT inversions in a 3D Earth structure model and applied it on small to medium-sized earthquakes recorded by the Southern California Seismic Network (SCSN). Our CMT inversion algorithm is an integral component of the scattering-integral (SI) method for full-3D waveform tomography (F3DT). In the SI method for F3DT, the sensitivity (Fréchet) kernels are constructed through the temporal convolution between the earthquake wavefield (EWF) and the receiver Green tensor (RGT), which is the wavefield generated by 3 orthogonal unit impulsive body forces acting at the receiver location. The RGTs are also the partial derivatives of the waveform with respect to the moment tensors. In this study, our RGTs are computed in a 3D seismic structure model for Southern California (CVM4SI1) using the finite-difference method, which allows us to account for 3D path effects in our source inversion. We used three component broadband waveforms below 0.2 Hz. An automated waveform-picking algorithm based on continuous wavelet transform is applied on observed waveforms to pick P, S and surface waves. A multi-scale grid-searching algorithm is then applied on the picked waveforms to find the optimal strike, dip and rake values that minimize the amplitude misfit and maximize the correlation coefficient. In general, our CMT solutions agree with solutions inverted using other methods and provide better fit to the observed waveforms.

  14. Rapid Centroid Moment Tensor (CMT) Inversion in 3D Earth Structure Model for Earthquakes in Southern California

    NASA Astrophysics Data System (ADS)

    Mu, D.; Lee, E.; Chen, P.; Jordan, T. H.; Maechling, P. J.

    2010-12-01

    Accurate and rapid CMT inversion is important for seismic hazard analysis. We have developed an algorithm for very rapid CMT inversions in a 3D Earth structure model and applied it on small to medium-sized earthquakes recorded by the Southern California Seismic Network (SCSN). Our CMT inversion algorithm is an integral component of the scattering-integral (SI) method for full-3D waveform tomography (F3DT). In the SI method for F3DT, the sensitivity (Fréchet) kernels are constructed through the temporal convolution between the earthquake wavefield (EWF) from the source and the receiver Green tensor (RGT) from the receiver. In this study, our RGTs were computed in a 3D seismic structure model for Southern California (CVM4SI1) using the finite-difference method, which allows us to account for 3D path effects in our source inversion. By storing the RGTs, synthetic seismograms for any source in our modeling volume could be generated rapidly by applying the reciprocity principle. An automated waveform-picking algorithm based on continuous wavelet transform is applied on observed waveforms to pick P, S and surface waves. A grid-searching algorithm is then applied on the picked waveforms to find an optimal focal mechanism that minimizes the amplitude misfit and maximize the weighted correlation coefficient. The grid-search result is then used as the initial solution in a gradient-based optimization algorithm that minimizes the L2 norm of the generalized seismological data functionals (GSDF), which quantifies waveform differences between observed and synthetic seismograms using frequencies-dependent phase-delay and amplitude anomalies. In general, our CMT solutions agree with solutions inverted using other methods and provide better fit to the observed waveforms.

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

    PubMed

    Bakirel, Tülay; Alkan, Fulya Üstün; Üstüner, Oya; Çinar, Suzan; Yildirim, Funda; Erten, Gaye; Bakirel, Utku

    2016-05-01

    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

  16. 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. PMID:26662885

  17. 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.

  18. 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

  19. Tolerability and efficacy study of P2X7 inhibition in experimental Charcot-Marie-Tooth type 1A (CMT1A) neuropathy.

    PubMed

    Sociali, Giovanna; Visigalli, Davide; Prukop, Thomas; Cervellini, Ilaria; Mannino, Elena; Venturi, Consuelo; Bruzzone, Santina; Sereda, Michael W; Schenone, Angelo

    2016-11-01

    Charcot-Marie-Tooth 1A (CMT1A) is a demyelinating hereditary neuropathy for which pharmacological treatments are not yet available. An abnormally high intracellular Ca(2+) concentration was observed in Schwann cells (SC) from CMT1A rats, caused by the PMP22-mediated overexpression of the P2X7 purinoceptor. The purpose of this study was to investigate the tolerability and therapeutic potential of a pharmacological antagonist of the P2X7 receptor (A438079) in CMT1A. A438079 ameliorated in vitro myelination of organotypic DRG cultures from CMT1A rats. Furthermore, we performed an experimental therapeutic trial in PMP22 transgenic and in wild-type rats. A preliminary dose-escalation trial showed that 3mg/kg A438079 administered via intraperitoneal injection every 24h for four weeks was well tolerated by wild type and CMT1A rats. Affected rats treated with 3mg/kg A438079 revealed a significant improvement of the muscle strength, when compared to placebo controls. Importantly, histologic analysis revealed a significant increase of the total number of myelinated axons in tibial nerves. Moreover, a significant decrease of the hypermyelination of small caliber axons and a significant increase of the frequency and diameter of large caliber myelinated axons was highlighted. An improved distal motor latencies was recorded, whereas compound muscle action potentials (CMAP) remained unaltered. A438079 reduced the SC differentiation defect in CMT1A rats. These results show that pharmacological inhibition of the P2X7 receptor is well tolerated in CMT1A rats and represents a proof-of-principle that antagonizing this pathway may correct the molecular derangements and improve the clinical phenotype in the CMT1A neuropathy. PMID:27431093

  20. Transcriptional and functional studies of a Cd(II)/Pb(II)-responsive transcriptional regulator(CmtR) from Acidithiobacillus ferrooxidans ATCC 23270.

    PubMed

    Zheng, Chunli; Li, Yanjun; Nie, Li; Qian, Lin; Cai, Lu; Liu, Jianshe

    2012-08-01

    The acidophilic Acidithiobacillus ferrooxidans can resist exceptionally high cadmium (Cd) concentrations. This property is important for its use in biomining processes, where Cd and other metal levels range usually between 15 and 100 mM. To learn about the mechanisms that allow A. ferrooxidans cells to survive in this environment, a bioinformatic search of its genome showed the presence of that a Cd(II)/Pb(II)-responsive transcriptional regulator (CmtR) was possibly related to Cd homeostasis. The expression of the CmtR was studied by real-time reverse transcriptase PCR using A. ferrooxidans cells adapted for growth in the presence of high concentrations of Cd. The putative A. ferrooxidans Cd resistance determinant was found to be upregulated when this bacterium was exposed to Cd in the range of 15-30 mM. The CmtR from A. ferrooxidans was cloned and expressed in Escherichia coli, the soluble protein was purified by one-step affinity chromatography to apparent homogeneity. UV-Vis spectroscopic measurements showed that the reconstruction CmtR was able to bind Cd(II) forming Cd(II)-CmtR complex in vitro. The sequence alignment and molecular modeling showed that the crucial residues for CmtR binding were likely to be Cys77, Cys112, and Cys121. The results reported here strongly suggest that the high resistance of the extremophilic A. ferrooxidans to Cd including the Cd(II)/Pb(II)-responsive transcriptional regulator. PMID:22555344

  1. 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. PMID:25085517

  2. Absolute Strength of the San Andreas Fault Inferred from Tectonic Loading Simulation and CMT Data Inversion

    NASA Astrophysics Data System (ADS)

    Terakawa, T.; Matsu'Ura, M.

    2006-12-01

    In order to estimate the absolute strength of the big-bend segment (BBS) of the San Andreas Fault (SAF) we combined two different approaches, one of which is the numerical simulation of tectonic stress accumulation at and around plate boundaries and the other is the inversion analysis of seismic events to estimate tectonic stress release. With the 3-D tectonic loading model based on elastic dislocation theory, we numerically computed the absolute tectonic stress fields at and around BBS for six representative cases with different friction coefficients (0.6, 0.3 and 0.1) of SAF and surrounding thrust faults. In order to compare the theoretical results with seismological observations, we extracted only the stress field related to shear faulting (seismogenic stress field) from the computed absolute stress field. The patterns of the stress field for the representative cases are significantly different from each other within the distance range of 50 km from BBS. In this range, the rotation angle of the maximum horizontal compressive principal stress axis measured from the strike of BBS changes from 45o to 90o with distance from BBS. The range of the stress rotation becomes broader as the absolute strength of BBS becomes higher. The expected type of faulting in this range also depends on the absolute strength of BBS. On the other hand, we obtained the pattern of seismogenic stress field around BBS through an inversion analysis with CMT data. The type of faulting expected from the inverted stress field changes with distance from BBS as follows: thrust faulting with a strike oblique to BBS in the vicinity of BBS, thrust faulting with the dip-angle of 45o and a strike parallel to BBS in the range of 50-100 km from BBS, and vertical strike-slip faulting with a strike oblique to BBS in the region farther than 100 km. From the inverted stress field we can find a fault-parallel zone with high moment release rates at about 40 km southwest of BBS, which can be considered to play

  3. Exome sequencing reveals homozygous TRIM2 mutation in a patient with early onset CMT and bilateral vocal cord paralysis

    PubMed Central

    Pehlivan, Davut; Akdemir, Zeynep Coban; Karaca, Ender; Bayram, Yavuz; Jhangiani, Shalini; Yildiz, Edibe; Muzny, Donna; Uluc, Kayihan; Gibbs, Richard A.; Elcioglu, Nursel; Lupski, James R.; Harel, Tamar

    2015-01-01

    Charcot-Marie-Tooth disease (CMT) is a heterogeneous group of inherited distal symmetric polyneuropathies associated with mutations in genes encoding components essential for normal functioning of the Schwann cell and axon. TRIM2, encoding a ligase that ubiquitinates the neurofilament light chain, was recently associated with early onset neuropathy in a single patient. We report a TRIM2 homozygous missense mutation (c.2000A>C; p.D667A) in a patient with peripheral neuropathy and bilateral vocal cord paralysis, allowing for further delineation of the associated phenotypic spectrum. PMID:25893792

  4. 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. PMID:20493460

  5. 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.

  6. Rapid CMT determination through the joint inversion of GPS and strain data

    NASA Astrophysics Data System (ADS)

    Mencin, D.; Bilham, R. G.; Hodgkinson, K. M.; Mattioli, G. S.

    2013-12-01

    The success of recent efforts to rapidly determine centroid moment tensors of large earthquakes (M>6.5) using geodetic data (Melgar et. al. 2011, Crowell et. al. 2012) has opened up a new area of outreach with the potential to enhance early warning systems and emergency response planning. It would be beneficial to expand these efforts to volcanic activity, but because earthquakes associated with eruptions tend to be smaller, the rapid assessment of centroid moment tensors is limited by noise in the data. Joint inversion of GPS, strainmeter and tiltmeter data potentially provides an enhanced method to determine centroid moments to lower magnitudes. Geodetic instrumentation tends to surround a volcano and hence the inversions care potentially well constrained. We present an algorithm to determine the moment tensors and centroid location of M≈5 earthquakes based on the joint inversions of GPS and borehole strainmeter data. We show that the inversions can be undertaken in near real time. We explore the relative weighting of the data based on instrument characteristics, SNR, event magnitude and distance to achieve optimal inversions and compare the results with seismically determined moment tensors and earthquake location. By simultaneously analyzing data from multiple monitoring systems we will improve our understanding of earthquake characteristics in volcanic settings. Using many different instruments and tools is beneficial in tectonic regions where typically not all types of monitoring systems are available either spatially or temporally due to difficult access, instrument failure or network outages.

  7. Clinical spectrum of CMT4C disease in patients homozygous for the p.Arg1109X mutation in SH3TC2.

    PubMed

    Colomer, Jaume; Gooding, Rebecca; Angelicheva, Dora; King, Rosalind H M; Guillén-Navarro, Encarna; Parman, Yesim; Nascimento, Andres; Conill, Joan; Kalaydjieva, Luba

    2006-07-01

    We investigated the manifestations of CMT4C disease in a genetically homogeneous group of patients homozygous for the recently identified Gypsy founder mutation p.Arg1109X in SH3TC2. We observed a surprising degree of variation in age at onset, rate of progression, extent and severity of motor and sensory involvement, scoliosis, and cranial nerve involvement, suggesting that the phenotypic spectrum of CMT4C disease is much broader than the classical diagnostic criteria. Phenotype similarity in first degree relatives and increasing heterogeneity in more distantly related subjects point to the involvement of genetic modifiers, possibly variants in the genes encoding protein partners interacting with SH3TC2. PMID:16806930

  8. 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

  9. Comparison of California Mastitis Test (CMT), Somatic Cell Counts (SCC) and bacteriological examinations for detection of camel (Camelus dromedarius) mastitis in Ethiopia.

    PubMed

    Abdel Gadir Atif, E; Hildebrandt, Goetz; Kleer, Josef N; Molla, Bayleyegn; Kyule, Moses N; Baumann, Maximilian P O

    2006-01-01

    A total of 956 quarter milk samples from 253 traditionally managed lactating camels were collected aseptically from Negele (Borena Region), Dire Dawa, and Gewane (Afar Region), Ethiopia, according to multi-stage sampling. The quarter milk samples were subjected to California Mastitis Test (CMT), Somatic Cell Counts (SCC) and bacteriological examinations. Five hundred and seventy one (59.7%) quarter milk samples had microorganisms. Of these, 428 (75.0%) had isolates that were identified as major pathogens (MAP) and 143 (25.0%) as minor pathogens (MIP). A positive correlation was found between CMT scores and bacteriological classes (MAP, MIP) (p-value = 0.00). Strong correlation (p-value = 0.00) between CMT scores and SCC was recorded. The differences among the median log SCC of bacteriological classes (MAP, MIP) were not significant (p-value = 0.24). Similarly, the application of the cut-off level of 2.5 x 10(5) ml(-1) indicated less agreement (p-value = 0.32) for bacteriological classes MAP and MIP. PMID:16450708

  10. Studies of Bulk Properties of CZT and CMT Crystals for X-Ray and Gamma-Ray Detection at Ambient Temperature

    NASA Astrophysics Data System (ADS)

    Egarievwe, S. U.; Bolotnikov, A. E.; Roy, U. N.; Babalola, S. O.; Kassu, A.; Jow, J.; Camarda, G.; Fochuk, P.; Chan, W.; Kim, K. H.; Stein, J.; James, R. B.; Burger, A.

    2010-10-01

    Cadmium Zinc Telluride (CZT) and Cadmium Manganese Telluride (CMT) crystals have emerged as promising advanced materials for X-ray and gamma-ray detection at ambient temperature. An understanding of the bulk properties in relation to energy resolution and device performance has led to the development of CZT into commercial devices, and is contributing to the improvement of CMT. The key detector attributes are large band-gap energy (˜1.6 eV, tunable by Zn or Mn concentration), high atomic number, and high density (˜6 g/cm^3). The techniques used in this study include infrared transmission microscopy, synchrotron X-ray diffraction topography, micro-scale X-ray mapping, and Pockels effect. We found that point defects and Te inclusions in CZT and CMT can trap the charge carriers generated by the absorption of X-rays, gamma-rays, and charged particles. We propose the use of thermal annealing and doping techniques to eliminate the deleterious effects caused by Te inclusions.

  11. Types of CMT

    MedlinePlus

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  12. 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.

  13. Behavioral and molecular exploration of the AR-CMT2A mouse model Lmna (R298C/R298C).

    PubMed

    Poitelon, Yannick; Kozlov, Serguei; Devaux, Jerôme; Vallat, Jean-Michel; Jamon, Marc; Roubertoux, Pierre; Rabarimeriarijaona, Sitraka; Baudot, Cécile; Hamadouche, Tarik; Stewart, Colin L; Levy, Nicolas; Delague, Valérie

    2012-03-01

    In 2002, we identified LMNA as the first gene responsible for an autosomal recessive axonal form of Charcot-Marie-Tooth disease, AR-CMT2A. All patients were found to be homozygous for the same mutation in the LMNA gene, p.Arg298Cys. In order to investigate the physiopathological mechanisms underlying AR-CMT2A, we have generated a knock-in mouse model for the Lmna p.Arg298Cys mutation. We have explored these mice through an exhaustive series of behavioral tests and histopathological analyses, but were not able to find any peripheral nerve phenotype, even at 18 months of age. Interestingly at the molecular level, however, we detect a downregulation of the Lmna gene in all tissues tested from the homozygous knock-in mouse Lmna (R298C/R298C) (skeletal muscle, heart, peripheral nerve, spinal cord and cerebral trunk). Importantly, we further reveal a significant upregulation of Pmp22, specifically in the sciatic nerves of Lmna (R298C/R298C) mice. These results indicate that, despite the absence of a perceptible phenotype, abnormalities exist in the peripheral nerves of Lmna (R298C/R298C) mice that are absent from other tissues. Although the mechanisms leading to deregulation of Pmp22 in Lmna (R298C/R298C) mice are still unclear, our results support a relation between Lmna and Pmp22 and constitute a first step toward understanding AR-CMT2A physiopathology. PMID:22331516

  14. Non-destructive 3D Imaging of Extraterrestrial Materials by Synchrotron X-ray Micro- tomography (XR-CMT) and Laser Confocal Scanning Microscopy (LCSM): Beyond Pretty Pictures

    NASA Astrophysics Data System (ADS)

    Ebel, D. S.; Greenberg, M.

    2009-05-01

    We report scientific results made possible only by the use these two non-destructive 3D imaging techniques. XR-CMT provides 3D image reconstructions at spatial resolutions of 1 to 17 micron/voxel edge. We use XR- CMT to locate potential melt-inclusion-bearing phenocrysts in batches of 100-200 micron lunar fire-fountain spherules; to locate and visualize the morphology of 1-2mm size, irregular, unmelted Ca-, Al-rich inclusions (CAIs) and to quantify chondrule/matrix ratios and chondrule size distributions in 6x6x20mm chunks of carbonaceous chondrites; to quantify the modal abundance of opaque phases in similar sized Martian meteorite fragments, and in individual 1-2mm diameter chondrules from chondrites. LCSM provides 3D image stacks at resolutions < 100 nm/pixel. We are the only group creating deconvolved image stacks of 100 to over 1000 micron long comet particle tracks in aerogel keystones from the Stardust mission. We present measurements of track morphology in 3D, and locate high-value particles using complementary synchrotron x- ray fluorescence (XRF) examination. We show that bench-top LCSM extracts maximum information about tracks and particles rapidly and cheaply prior to destructive disassembly. Using XR-CMT we quantify, for the first time, the volumetric abundances of metal grains in 1-2 mm diameter CR chondrite chondrules. Metal abundances vary from 1 to 37 vol.% between 8 chondrules (and more by inspection), in a meteorite with solar (chondritic) Fe/Si ratio, indicating that chondrules formed and accreted locally from bulk solar composition material. They are 'complementary' to each other in Fe/Si ratios. Void spaces in chondritic CAIs and chondrules are shown to be a primary feature, not due to plucking during sectioning. CAI morphology in 3D reveals pre-accretionary impact features, and various types of mineralogical layering, seen in 3D, reveal the formation history of these building blocks of planets and asteroids. We also quantify the x

  15. 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.

  16. Caveats for poly(methimazolyl)borate chemistry: the novel inorganic heterocycles [H2C(mt)2BR2]Cl (mt = methimazolyl; BR2 = BH2, BH(mt), 9-BBN).

    PubMed

    Crossley, Ian R; Hill, Anthony F; Humphrey, Elizabeth R; Smith, Matthew K; Tshabang, Never; Willis, Anthony C

    2004-08-21

    Whilst frequently used for reactions of poly(methimazolyl)borates, dichloromethane is not an innocent solvent, but rather slowly forms heterocyclic salts [H(2)C(mt)(2)BR(2)]Cl, three examples of which (BR(2) = BH(2), BH(mt), 9-borabicyclononyl) have been structurally characterised to confirm the unprecedented B(NCS)(2)C connectivity. PMID:15306929

  17. Overlap phenotype between CMT1A and hereditary neuropathy with liability to pressure palsies caused by the novel small in-frame deletion c.407_418del12 in PMP22 gene.

    PubMed

    Vill, Katharina; Kuhn, Marius; Gläser, Dieter; Müller-Felber, Wolfgang

    2015-02-01

    We report monozygotic twins, who presented with a clinical picture of Charcot-Marie-Tooth disease type 1 (CMT1) with bilateral foot drop, pes cavus, thoracic kyphosis, and scoliosis. Hereditary neuropathy with liability to pressure palsies (HNPP) showed up in one of them. Neurography showed demyelinating neuropathy, typical for CMT1, and transient conduction block in the ulnar nerve correlating with clinical ulnar palsy due to minor mechanical stress in only one of them. Genetic analysis revealed novel small de novo deletion c.407_418del12 in the PMP22 gene. Our patient shows the rarely reported combination of CMT1A and HNPP, caused by an in-frame deletion in the PMP22 gene. HNPP is in the majority of cases correlated with heterozygous deletion of the whole PMP22 gene or other mutations leading to functional haploinsufficiency. The cases give further evidence that pathogenesis of HNPP is not completely understood and can obviously result from existence of a defective protein, too. The intrafamiliar phenotypic variability, even in monozygotic twins, confirms the well-known fact that factors apart from genetics contribute to the clinical course. PMID:25265422

  18. Using DART-recorded Rayleigh waves for rapid CMT and finite fault analyses of large megathrust earthquakes.

    NASA Astrophysics Data System (ADS)

    Thio, H. K.; Polet, J.; Ryan, K. J.

    2015-12-01

    We study the use of long-period Rayleigh waves recorded by the DART-type ocean bottom pressure sensors. The determination of accurate moment and slip distribution after a megathrust subduction zone earthquake is essential for tsunami early warning. The two main reasons why the DART data are o interest to this problem are; 1 - contrary to the broadband data used in the early stages of earthquake analysis, the DART data do not saturate for large magnitude earthquakes, and 2 - DART stations are located offshore and thus often fill gaps in the instrumental coverage at local and regional distances. Thus, by including DART recorded Rayleigh waves into the rapid response systems we may be able to gain valuable time in determining accurate moment estimates and slip distributions needed for tsunami warning and other rapid response products. Large megathrust earthquakes are among the most destructive natural disasters in history but also pose a significant challenge real-time analysis. The scales involved in such large earthquakes, with ruptures as long as a thousand kilometers and durations of several minutes are formidable. There are still issues with rapid analysis at the short timescales, such as minutes after the event since many of the nearby seismic stations will saturate due to the large ground motions. Also, on the seaward side of megathrust earthquakes, the nearest seismic stations are often thousands of kilometers away on oceanic islands. The deployment of DART buoys can fill this gap, since these instruments do not saturate and are located close in on the seaward side of the megathrusts. We are evaluating the use of DART-recorded Rayleigh waves, by including them in the dataset used for Centroid Moment Tensor analyses, and by using the near-field DART stations to constrain source finiteness for megathrust earthquakes such as the recent Tohoku, Haida Gwaii and Chile earthquakes.

  19. 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.

  20. 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.

  1. A meta-analysis of randomized double-blind clinical trials in CMT1A to assess the change from baseline in CMTNS and ONLS scales after one year of treatment.

    PubMed

    Mandel, Jonas; Bertrand, Viviane; Lehert, Philippe; Attarian, Shahram; Magy, Laurent; Micallef, Joëlle; Chumakov, Ilya; Scart-Grès, Catherine; Guedj, Mickael; Cohen, Daniel

    2015-01-01

    CMT1A is the most common inherited peripheral neuropathy. There is currently no approved treatment. We performed a meta-analysis including four randomized, double-blind, Placebo-controlled clinical trials to assess the disease progression after one year under Placebo, Ascorbic Acid (AA) or PXT3003, a combination of three repurposed drugs. We observed a weak deterioration in patients under Placebo, well below the reported natural disease progression. Patients treated with AA were stable after one year but not significantly different from Placebo. Patients undergoing PXT3003 treatment showed an improvement in CMTNS and ONLS, statistically significant versus Placebo and potentially precursory of a meaningful change in the disease course. PMID:26070802

  2. Investigation of the local expression of the relaxin system in canine mammary tumours.

    PubMed

    Lamp, O; Honscha, K U; Jakob, J; Lamp, J; Schweizer, S; Reischauer, A; Gottschalk, J; Hahn, A; Ebert, M; Rothemund, S; Blaschzik, S; Einspanier, A

    2009-07-01

    While mammary tumours are the main reasons of death in bitches, early detection of tumours and metastases is crucial for survival of affected dogs. Invasiveness and angiogenesis, which are important processes of tumour growth and spreading, require connective tissue remodelling. This process is dominantly mediated by matrix metalloproteinases (MMP), which are well known to be positively regulated by relaxin (RLX) in various tissues, including human breast cancer. So far, the presence of RLX and its receptor RXFP-1 as well as their linkage with MMP in canine mammary tumours (CMT) is completely unknown. In the first part of the present study, concentrations of RLX, oestradiol and progesterone from plasma samples of bitches with CMT were compared with clinical and survival data to investigate the predictive value of these hormones. In the second part, the expressions of RLX, RXFP-1 and MMP-2, -9 and -13 were examined by real-time reverse transcriptase polymerase chain reaction (RT-PCR) in 31 CMT samples. Finally, relationships of systemic plasma RLX or locally expressed RLX with expression of MMP in CMT were analyzed for the first time. Comparison of hormone concentrations in 93 bitches in terms of benign or malignant nature of the CMT, lung metastases, recidivation and 12-month survival discovered no significances. The expressions of RLX, RXFP-1 and MMP were independent from plasma RLX, but expressions of local RLX and RXFP-1 showed a strong correlation (p = 0.00004, r = 0.671) as well as RXFP-1 and MMP-2 (p = 0.009, r = 0.463), indicating a possible significant role of the locally produced RLX in CMT pathogenesis as an inducer of connective tissue remodelling. PMID:19754574

  3. 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. PMID:21643116

  4. 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.

  5. 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.

  6. 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).

  7. 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. PMID:26292645

  8. Evaluation of earthquake parameters used in the Indonesian Tsunami Early Warning System

    NASA Astrophysics Data System (ADS)

    Madlazim; Prastowo, Tjipto

    2016-02-01

    Twenty-two of a total of 30 earthquake events reported by the Indonesian Agency for Geophysics, Climatology and Meteorology during the time period 2007-2010 were falsely issued as tsunamigenic by the Indonesian Tsunami Early Warning System (Ina-TEWS). These 30 earthquakes were of different magnitudes and occurred in different locations. This study aimed to evaluate the performance of the Ina-TEWS using common earthquake parameters, including the earthquake magnitude, origin time, depth, and epicenter. In total, 298 datasets assessed by the Ina-TEWS and the global centroid moment tensor (CMT) method were assessed. The global CMT method is considered by almost all seismologists to be a reference for the determination of these parameters as they have been proved to be accurate. It was found that the earthquake magnitude, origin time, and depth provided by the Ina-TEWS were significantly different from those given in the global CMT catalog, whereas the latitude and longitude positions of the events provided by both tsunami assessment systems were coincident. The performance of the Ina-TEWS, particularly in terms of accuracy, remains questionable and needs to be improved.

  9. Improved centroid moment tensor analyses in the NIED AQUA (Accurate and QUick Analysis system for source parameters)

    NASA Astrophysics Data System (ADS)

    Kimura, H.; Asano, Y.; Matsumoto, T.

    2012-12-01

    The rapid determination of hypocentral parameters and their transmission to the public are valuable components of disaster mitigation. We have operated an automatic system for this purpose—termed the Accurate and QUick Analysis system for source parameters (AQUA)—since 2005 (Matsumura et al., 2006). In this system, the initial hypocenter, the moment tensor (MT), and the centroid moment tensor (CMT) solutions are automatically determined and posted on the NIED Hi-net Web site (www.hinet.bosai.go.jp). This paper describes improvements made to the AQUA to overcome limitations that became apparent after the 2011 Tohoku Earthquake (05:46:17, March 11, 2011 in UTC). The improvements included the processing of NIED F-net velocity-type strong motion records, because NIED F-net broadband seismographs are saturated for great earthquakes such as the 2011 Tohoku Earthquake. These velocity-type strong motion seismographs provide unsaturated records not only for the 2011 Tohoku Earthquake, but also for recording stations located close to the epicenters of M>7 earthquakes. We used 0.005-0.020 Hz records for M>7.5 earthquakes, in contrast to the 0.01-0.05 Hz records employed in the original system. The initial hypocenters determined based on arrival times picked by using seismograms recorded by NIED Hi-net stations can have large errors in terms of magnitude and hypocenter location, especially for great earthquakes or earthquakes located far from the onland Hi-net network. The size of the 2011 Tohoku Earthquake was initially underestimated in the AQUA to be around M5 at the initial stage of rupture. Numerous aftershocks occurred at the outer rise east of the Japan trench, where a great earthquake is anticipated to occur. Hence, we modified the system to repeat the MT analyses assuming a larger size, for all earthquakes for which the magnitude was initially underestimated. We also broadened the search range of centroid depth for earthquakes located far from the onland Hi

  10. Systemic inflammation disrupts oligodendrocyte gap junctions and induces ER stress in a model of CNS manifestations of X-linked Charcot-Marie-Tooth disease.

    PubMed

    Olympiou, Margarita; Sargiannidou, Irene; Markoullis, Kyriaki; Karaiskos, Christos; Kagiava, Alexia; Kyriakoudi, Styliana; Abrams, Charles K; Kleopa, Kleopas A

    2016-01-01

    X-linked Charcot-Marie-Tooth disease (CMT1X) is a common form of inherited neuropathy resulting from different mutations affecting the gap junction (GJ) protein connexin32 (Cx32). A subset of CMT1X patients may additionally present with acute fulminant CNS dysfunction, typically triggered by conditions of systemic inflammation and metabolic stress. To clarify the underlying mechanisms of CNS phenotypes in CMT1X we studied a mouse model of systemic inflammation induced by lipopolysaccharide (LPS) injection to compare wild type (WT), connexin32 (Cx32) knockout (KO), and KO T55I mice expressing the T55I Cx32 mutation associated with CNS phenotypes. Following a single intraperitoneal LPS or saline (controls) injection at the age of 40-60 days systemic inflammatory response was documented by elevated TNF-α and IL-6 levels in peripheral blood and mice were evaluated 1 week after injection. Behavioral analysis showed graded impairment of motor performance in LPS treated mice, worse in KO T55I than in Cx32 KO and in Cx32 KO worse than WT. Iba1 immunostaining revealed widespread inflammation in LPS treated mice with diffusely activated microglia throughout the CNS. Immunostaining for the remaining major oligodendrocyte connexin Cx47 and for its astrocytic partner Cx43 revealed widely reduced expression of Cx43 and loss of Cx47 GJs in oligodendrocytes. Real-time PCR and immunoblot analysis indicated primarily a down regulation of Cx43 expression with secondary loss of Cx47 membrane localization. Inflammatory changes and connexin alterations were most severe in the KO T55I group. To examine why the presence of the T55I mutant exacerbates pathology even more than in Cx32 KO mice, we analyzed the expression of ER-stress markers BiP, Fas and CHOP by immunostaining, immunoblot and Real-time PCR. All markers were increased in LPS treated KO T55I mice more than in other genotypes. In conclusion, LPS induced neuroinflammation causes disruption of the main astrocyte

  11. 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.

  12. Microbial Enhanced Oil Recovery in Fractional-Wet Systems: A Pore-Scale Investigation

    SciTech Connect

    Armstrong, Ryan T.; Wildenschild, Dorthe

    2012-10-24

    Microbial enhanced oil recovery (MEOR) is a technology that could potentially increase the tertiary recovery of oil from mature oil formations. However, the efficacy of this technology in fractional-wet systems is unknown, and the mechanisms involved in oil mobilization therefore need further investigation. Our MEOR strategy consists of the injection of ex situ produced metabolic byproducts produced by Bacillus mojavensis JF-2 (which lower interfacial tension (IFT) via biosurfactant production) into fractional-wet cores containing residual oil. Two different MEOR flooding solutions were tested; one solution contained both microbes and metabolic byproducts while the other contained only the metabolic byproducts. The columns were imaged with X-ray computed microtomography (CMT) after water flooding, and after MEOR, which allowed for the evaluation of the pore-scale processes taking place during MEOR. Results indicate that the larger residual oil blobs and residual oil held under relatively low capillary pressures were the main fractions recovered during MEOR. Residual oil saturation, interfacial curvatures, and oil blob sizes were measured from the CMT images and used to develop a conceptual model for MEOR in fractional-wet systems. Overall, results indicate that MEOR was effective at recovering oil from fractional-wet systems with reported additional oil recovered (AOR) values between 44 and 80%; the highest AOR values were observed in the most oil-wet system.

  13. Comparison of intravitreal bevacizumab with macular photocoagulation for treatment of diabetic macular edema: a systemic review and Meta-analysis

    PubMed Central

    Liu, Xiang-Dong; Zhou, Xiao-Dong; Wang, Zhi; Shen, Hong-Jie

    2014-01-01

    AIM To further evaluate the efficacy and safety of intravitreal bevacizumab (IVB) versus macular photocoagulation (MPC) in treatment of diabetic macular edema (DME) by Meta-analysis. METHODS Pertinent publications were identified through systemic searches of PubMed, Medline, EMBASE, and the Cochrane Controlled Trials Register up to 30 November, 2013. Changes in central macular thickness (CMT) in µm and best-corrected visual acuity (BCVA) in logMAR equivalents were extracted at 1, 3, 6, 12 and 24mo after initial treatment, and a Meta-analysis was carried out to compare results between groups receiving IVB and MPC. RESULTS Five randomized controlled trial (RCTs) and one high-quality comparative study were identified and included. Our Meta-analysis revealed that both IVB and MPC resulted in the improvements of CMT and BCVA in eyes with DME at 1mo after initial treatment, with IVB being significantly superior to MPC (P=0.01 and 0.02, respectively). The improvements of both measure outcomes at 3, 6, 12 and 24mo after treatment did not vary significantly between the IVB groups and MPC groups (CMT at 3mo, P=0.85; at 6mo, P=0.29; at 12mo, P=0.56; at 24mo, P=0.71; BCVA at 3mo, P=0.31; at 6mo, P= 0.30; at 12mo, P=0.23; at 24mo, P=0.52). However, the number of observed adverse events was low in all studies. CONCLUSION Current evidence shows IVB treatment trends to be more effective in improvements of macular edema and vision in eyes with DME at an earlier follow up (1mo) compared with MPC. At other time, both interventions have comparable efficacy without statistical significances. PMID:25540764

  14. Subclinical mastitis in dairy cows in Swiss organic and conventional production systems.

    PubMed

    Roesch, Markus; G Doherr, Marcus; Schären, Walter; Schällibaum, Melchior; Blum, Jürg W

    2007-02-01

    The objective was to compare the prevalence of subclinical mastitis (SM) and of udder pathogens in 60 Swiss organic (OP) and 60 conventional production systems (CP). Cows (n=970) were studied for SM prevalence and udder pathogens at median 31 d and 102 d post partum. Cows showing a >or=1+ positive California Mastitis Test (CMT) in at least one quarter were considered to have SM. Cow-level prevalences of SM for visits at 31 d and 102 d post partum (39% and 40% in OP and 34% and 35% in CP) were similar, but quarter-level prevalences of SM were higher (P<0.02) in OP than CP (15% and 18% in OP and 12% and 15% in CP). Median somatic cell counts in milk at 31 d post partum were higher (P<0.05) in OP than CP cows (43000 and 28000 cells/ml, respectively), but were similar at 102 d post partum in OP and CP cows (45000 and 38000 cells/ml, respectively). In milk samples from quarters showing a CMT reaction >or=2+ the prevalences of coagulase negative staphylococci were lower (P<0.05) at 102 d post partum, whereas prevalences of non-agalactiae streptococci were higher (P<0.05) in OP than in CP cows at 31 d and 102 d post partum. In conclusion, under Swiss conditions, subclinical mastitis is a greater problem in organic than in conventional production systems, but differences are not marked. PMID:16978453

  15. 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. PMID:25012786

  16. [Phenotypes of Charcot-Marie-Tooth Syndrome and Differential Diagnosis Focused in Inflammatory Neuropathies].

    PubMed

    Iijima, Masahiro

    2016-01-01

    Charcot-Marie-Tooth disease (CMT), the most frequent form of inherited neuropathy, is a genetically heterogeneous syndrome of the peripheral nervous system with a rather homologous clinical phenotype (slowly progressive distal weakness and muscle atrophy, skeletal deformities, and areflexia in each limb). CMT1 is the autosomal-dominant demyelinating form, and CMT1A (mostly PMP22 duplication) is the most frequent subtype, followed by CMTX1, HNPP (hereditary neuropathy with liability to pressure palsies), CMT1B, or CMT2. As CMT is characterized by slowly progressive motor and sensory disturbances in each limb, it could be misdiagnosed as chronic inflammatory demyelinating polyneuropathy (CIDP) occasionally. Some points can distinguish demyelinating CMT from CIDP. CMT1 patients do not show the conduction block that is frequent in CIDP. In addition, ultrasonographic findings are useful because CMT1 suggests diffuse enlargement of peripheral nerves, whereas CIDP is characterized by asymmetrical or focal enlargement of peripheral nerves. Some CMT1 cases show favorable responses to immunomodulating therapeutics such as corticosteroids, IVIg, and plasma exchange. Such CIDP-like CMT1 (especially CMT1B or CMT2A) shows moderate to high levels of cerebrospinal fluid protein and infiltrated inflammatory macrophages. PMID:26764297

  17. BIODEGRADATION STUDIES OF CARBOXYMETHYL TARTRONATE

    EPA Science Inventory

    Carboxymethyl tartronate (CMT) was shown to be biodegradable in bench-scale activated sludge reactors. After initial exposure to CMT in continuous flow systems an acclimation period of 14 weeks was necessary before efficient degradation occurred. Once acclimated to CMT the biomas...

  18. Oscillations of relative inclination angles in compact extrasolar planetary systems

    NASA Astrophysics Data System (ADS)

    Becker, Juliette C.; Adams, Fred C.

    2016-01-01

    The Kepler mission has detected dozens of compact planetary systems with more than four transiting planets. This sample provides a collection of close-packed planetary systems with relatively little spread in the inclination angles of the inferred orbits. A large fraction of the observational sample contains limited multiplicity, begging the question whether there is a true diversity of multitransiting systems, or if some systems merely possess high mutual inclinations, allowing them to appear as single-transiting systems in a transit-based survey. This paper begins an exploration of the effectiveness of dynamical mechanisms in exciting orbital inclination within exoplanetary systems of this class. For these tightly packed systems, we determine that the orbital inclination angles are not spread out appreciably through self-excitation. In contrast, the two Kepler multiplanet systems with additional non-transiting planets are susceptible to oscillations of their inclination angles, which means their currently observed configurations could be due to planet-planet interactions alone. We also provide constraints and predictions for the expected transit duration variations for each planet. In these multiplanet compact Kepler systems, oscillations of their inclination angles are remarkably hard to excite; as a result, they tend to remain continually mutually transiting (CMT-stable). We study this issue further by augmenting the planet masses and determining the enhancement factor required for oscillations to move the systems out of transit. The oscillations of inclination found here inform the recently suggested dichotomy in the sample of Solar systems observed by Kepler.

  19. 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. PMID:27410811

  20. 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

  1. PACTEL experiments for the investigation of passive safety injection systems of advanced light water reactors

    SciTech Connect

    Tuunanen, J.; Munther, R.; Vihavainen, J.

    1996-07-01

    An important aspect of Advanced Light Water Reactors (ALWRs) decay heat removal concerns the plant response under Loss of Coolant Accident (LOCA) conditions. In ALWRs, e.g. Westinghouse AP600, pump driven Emergency Core Cooling Systems (ECCS) are replaced by passive safety injection systems, which are gravity driven. It is therefore important that in such accidents, the ALWR coolant system pressure can be controlled to allow gravity fed injection to take place. The safety issue here is whether undesirable system responses could occur in any circumstances. Additionally, it is necessary to prove that the plant always depressurizes sufficiently for the ECCS to operate efficiently. Two experimental series have been carried out with the PACTEL integral test facility on the simulation of passive safety injection systems of ALWRs in accidental conditions. The safety system investigated was a passive core make-up tank (CMT), which was connected to the downcomer of the test facility. This paper starts with a short description of the PACTEL test facility and a summary of the results of the passive safety injection tests on PACTEL. Also included is a summary of the results of the computer simulations of the tests. The second part of the paper consists of a description of the planned third passive safety injection test series and the results of the pre-test simulations of the planned tests.

  2. Analysis of wall heat capacity effects on core makup tank drain-down behavior in ROSA/AP600 experiments

    SciTech Connect

    Kondo, Masaya; Yonomoto, Taisuke; Asaka, Hideaki

    1997-12-01

    The thermal-hydraulic behavior of the core makeup tank (CMT) during scaled integral experiments on the Westinghouse AP600 reactor design was analyzed using the RELAP5/Mod3 (version 5M5) code. The natural circulation rate through the CMT was predicted well, although the prediction of the thermal stratification in the CMT had a problem due to inability to predict multidimensional mixing in the CMT upper regions. The over-scaled CMT metal mass in the experimental facility affected the CMT drain-down behavior in two experiments: (i) a multiple-failure experiment where the system depressurization became extremely slow due to the simulated failure of the ADS valves; and (ii) a relatively-large break experiment where the CMT started draining before thermal stratification developed in the CMT water inventory. In both experiments, the CMT wall became a heat sink and was a large steam condensation site. This had a effect to limit the CMT drain rate. 6 refs., 15 figs.

  3. Analysis of Stress Field in Caribean Continental Plate - Southern Costa Rica zone - from CMT Solutions

    NASA Astrophysics Data System (ADS)

    Zevallos, I.; Quintero, R.; Jimenez, W.

    2007-12-01

    During the of period 1984 2007, 51 earthquakes were registered with magnitudes above 5.1 mb in southern Costa Rica. Depth distribution of hypocenters shows shallow (0 20 km) earthquakes located in continental area; intermediate depth hypocenters (20 60 km) corresponding to subduction of the Coco plate under the Caribean plate; and just one earthquake have focus under 60 km depth. We choose hypocenters shallower than 100 km because our goal is to study intraplate stresses. Centroid Moment Tensor solutions for subduction zone earthquakes have inverse fault mechanism. But, in continental area there are evidences of strike slip and normal fault mechanisms. Direction of main stress (σ3) at the southern continental zone is NE-SW; in the Pacific ocean border region, the main stresses are oriented parallel to the coast line; inside the valley region, mechanisms are predominantly strike slip with σ1 in the near N-S direction. This is a complex stress field, with rotation of main stresses in a short space. σ1 is vertical in some continental areas due to influence of elevated terrain. Stress axis paralell to coast line maybe also due to gravitational body force.

  4. 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

    2016-04-01

    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.

  5. 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.

  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. Cumate-Inducible Gene Expression System for Sphingomonads and Other Alphaproteobacteria

    PubMed Central

    Kaczmarczyk, Andreas; Vorholt, Julia A.

    2013-01-01

    Tunable promoters represent a pivotal genetic tool for a wide range of applications. Here we present such a system for sphingomonads, a phylogenetically diverse group of bacteria that have gained much interest for their potential in bioremediation and their use in industry and for which no dedicated inducible gene expression system has been described so far. A strong, constitutive synthetic promoter was first identified through a genetic screen and subsequently combined with the repressor and the operator sites of the Pseudomonas putida F1 cym/cmt system. The resulting promoter, termed PQ5, responds rapidly to the inducer cumate and shows a maximal induction ratio of 2 to 3 orders of magnitude in the different sphingomonads tested. Moreover, it was also functional in other Alphaproteobacteria, such as the model organisms Caulobacter crescentus, Paracoccus denitrificans, and Methylobacterium extorquens. In the noninduced state, expression from PQ5 is low enough to allow gene depletion analysis, as demonstrated with the essential gene phyP of Sphingomonas sp. strain Fr1. A set of PQ5-based plasmids has been constructed allowing fusions to affinity tags or fluorescent proteins. PMID:23995928

  9. Hereditary motor and sensory neuropathies or Charcot-Marie-Tooth diseases: an update.

    PubMed

    Tazir, Meriem; Hamadouche, Tarik; Nouioua, Sonia; Mathis, Stephane; Vallat, Jean-Michel

    2014-12-15

    Hereditary motor and sensory neuropathies (HMSN) or Charcot-Marie-Tooth (CMT) diseases are the most common degenerative disorders of the peripheral nervous system. However, the frequency of the different subtypes varies within distinct populations. Although more than seventy clinical and genetic forms are known to date, more than 80% of CMT patients in Western countries have genetic abnormalities associated with PMP22, MPZ, MFN2 and GJB1. Given the considerable genetic heterogeneity of CMT, we emphasize the interest of both clinical and pathological specific features such that focused genetic testing could be performed. In this regard, peripheral nerve lesions in GDAP1 mutations (AR CMT1A), such as mitochondrial abnormalities, have been newly demonstrated. Otherwise, while demyelinating autosomal recessive CMT used to be classified as CMT4 (A, B, C …), we propose a simplified classification such as AR CMT1 (A, B, C …), and AR CMT2 for axonal forms. Also, we stress that next generation sequencing techniques, now considered to be the most efficient methods of genetic testing in CMT, will be helpful in molecular diagnosis and research of new genes involved. Finally, while no effective therapy is known to date, ongoing new therapeutic trials such as PXT3003 (a low dose combination of the three already approved drugs baclofen, naltrexone, and D-sorbitol) give hopes for potential curative treatment. PMID:25454638

  10. Dynamically tunable slow light based on plasmon induced transparency in disk resonators coupled MDM waveguide system

    NASA Astrophysics Data System (ADS)

    Han, Xu; Wang, Tao; Li, Xiaoming; Liu, Bo; He, Yu; Tang, Jian

    2015-06-01

    Ultrafast and low-power dynamically tunable single channel and multichannel slow light based on plasmon induced transparencies (PITs) in disk resonators coupled to a metal-dielectric-metal (MDM) waveguide system with a nonlinear optical Kerr medium is investigated both numerically and analytically. A coupled-mode theory (CMT) is introduced to analyze this dynamically tunable single channel slow light structure. Multichannel slow light is realized in this plasmonic waveguide structure based on a bright-dark mode coupling mechanism. In order to reduce the pump intensity and obtain ultrafast response time, the traditional nonlinear Kerr material is replaced by monolayer graphene. It is found that the magnitude of the single PIT window can be controlled between 0.08 and 0.48, while the corresponding group index is controlled between 14.5 and 2.0 by dynamically decreasing pump intensity from 11.7 to 4.4 MW cm-2. Moreover, the phase shift multiplication effect is found in this structure. This work paves a new way towards the realization of highly integrated optical circuits and networks, especially for wavelength-selective, all-optical storage and nonlinear devices.

  11. 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. PMID:25574749

  12. Mutation of SIMPLE in Charcot-Marie-Tooth 1C alters production of exosomes.

    PubMed

    Zhu, Hong; Guariglia, Sara; Yu, Raymond Y L; Li, Wenjing; Brancho, Deborah; Peinado, Hector; Lyden, David; Salzer, James; Bennett, Craig; Chow, Chi-Wing

    2013-06-01

    Charcot-Marie-Tooth (CMT) disease is an inherited neurological disorder. Mutations in the small integral membrane protein of the lysosome/late endosome (SIMPLE) account for the rare autosomal-dominant demyelination in CMT1C patients. Understanding the molecular basis of CMT1C pathogenesis is impeded, in part, by perplexity about the role of SIMPLE, which is expressed in multiple cell types. Here we show that SIMPLE resides within the intraluminal vesicles of multivesicular bodies (MVBs) and inside exosomes, which are nanovesicles secreted extracellularly. Targeting of SIMPLE to exosomes is modulated by positive and negative regulatory motifs. We also find that expression of SIMPLE increases the number of exosomes and secretion of exosome proteins. We engineer a point mutation on the SIMPLE allele and generate a physiological mouse model that expresses CMT1C-mutated SIMPLE at the endogenous level. We find that CMT1C mouse primary embryonic fibroblasts show decreased number of exosomes and reduced secretion of exosome proteins, in part due to improper formation of MVBs. CMT1C patient B cells and CMT1C mouse primary Schwann cells show similar defects. Together the data indicate that SIMPLE regulates the production of exosomes by modulating the formation of MVBs. Dysregulated endosomal trafficking and changes in the landscape of exosome-mediated intercellular communications may place an overwhelming burden on the nervous system and account for CMT1C molecular pathogenesis. PMID:23576546

  13. Charcot-Marie-Tooth neuropathy due to a novel EGR2 gene mutation with mild phenotype--usefulness of human mapping chip linkage analysis in a Czech family.

    PubMed

    Safka Brožková, Dana; Nevšímalová, Soňa; Mazanec, Radim; Rautenstrauss, Bernd; Seeman, Pavel

    2012-08-01

    Charcot-Marie-Tooth neuropathies (CMT) are a group of clinically and genetically heterogeneous disorders of the peripheral nervous system. Selection of candidate disease genes for mutation analysis is sometimes difficult since more than 40 genes and loci are known to be associated with CMT neuropathies. Hence a Czech family Cz-CMT with demyelinating type of autosomal dominant CMT disease was investigated by genome-wide linkage analysis by means of single-nucleotide polymorphism (SNP) arrays. Among 35 regions with linkage, five carried known CMT genes. In the final result a novel early growth response 2 - missense mutation c.1235 A>G, p.Glu412Gly was found. Surprisingly, the more severely affected proband carried an additional heterozygous myelin protein zero variant p.Asp246Asn detected previously, which may modify the phenotype. However, this MPZ variant is benign in heterozygous state alone, because it is also carried by the patient's healthy father. PMID:22546699

  14. 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

  15. 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.

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

    DOE PAGESBeta

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

    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.

    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. PMID:26208662

  18. 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. PMID:23250879

  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. Non-antibacterial tetracycline formulations: host-modulators in the treatment of periodontitis and relevant systemic diseases.

    PubMed

    Golub, Lorne M; Elburki, Muna S; Walker, Clay; Ryan, Maria; Sorsa, Timo; Tenenbaum, Howard; Goldberg, Michael; Wolff, Mark; Gu, Ying

    2016-06-01

    Traditionally, the dental profession has primarily treated periodontitis using a mechanical/surgical, rather than a pharmaceutical, approach. However, based on experiments several decades ago which demonstrated that tetracyclines, unexpectedly, inhibit collagen- and bone-destructive mammalian-derived enzymes (e.g. the collagenases), and through non-antibiotic mechanisms, the concept of host-modulation therapy (HMT) was developed. Accordingly, two drug-development strategies evolved: (i) the development of non-antimicrobial formulations of doxycycline; and (ii) the chemical modification of tetracyclines to eliminate their antibiotic activity but retain (or even enhance) their anti-collagenase properties. Regarding the latter, these chemically modified tetracyclines (CMTs) showed efficacy in vitro, in animal models of periodontal (and relevant systemic) disease, and in preliminary clinical trials on patients with Kaposi's sarcoma (however, at the high doses used, photosensitivity was a significant side-effect). In the first strategy, subantimicrobial-dose doxycycline (SDD) demonstrated safety and efficacy in human clinical trials and was approved by the U S Food and Drug Administration (U S FDA) and in other countries for the treatment of periodontitis (20 mg, twice daily, i.e. once every 12 hours) adjunctive to scaling and root planing, and for chronic inflammatory skin diseases (40-mg sustained-release 'beads'). SDD also showed efficacy in patients with systemic diseases relevant to periodontitis, including diabetes mellitus and arthritis, and in postmenopausal women with local and systemic bone loss. Importantly, long-term administration of SDD, of up to 2 years, in clinical trials did not produce antibiotic side-effects. SDD (and in the future, new HMTs, such as low-dose CMT-3, resolvins and chemically modified curcumins) may shift the paradigm of periodontal therapy from a predominantly surgical approach to the greater use of medicinal/pharmacologic strategies

  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. A Strong Stress Shadow Effect of the 2004 M=9.2 Sumatra-Andaman Earthquake on the Andaman Sea Transform-Rift System 250 km Away

    NASA Astrophysics Data System (ADS)

    Sevilgen, V.; Stein, R. S.

    2010-12-01

    The 26 December 2004 earthquake ruptured a 1,300-km section of the Sunda megathrust. A transform-rift back-arc system accommodates most of the trench-parallel component of the highly oblique subduction. We used the NEIC earthquake catalog at its M≥4.7 completeness level since 1999, and at M≥4.8 since 1975, to examine the seismicity rate along the transform-rift system. We also combined teleseismic double-difference earthquake relocations from Pesicek et al (JGR, 2010) with Global CMT mechanisms, to more accurately associate focal mechanisms with their fault systems. We find a strong drop in seismicity rate along the Andaman Sea transform system east of the northern end of the 2004 rupture zone. This occurs immediately following the Sumatra-Andaman mainshock and persists to this day. The rate drop is associated with strike-slip mechanisms only; along the portions of the rift system with normal-faulting mechanisms, the seismicity rate increased. We calculate that the Sagaing-West Andaman transform in this region was subjected to a static Coulomb stress drop of 0.25 bar (for an assumed fault friction of 0.4), whereas the rift segments sustained stress increases greater than 1 bar. Both of these calculations are in accord with the observations. Because of the large distance between the megathrust source and the back-arc receiver faults, the imparted stresses are insensitive to the unknown details of the megathrust slip and geometry; because the 2004 slip is so large, the imparted stresses are nevertheless substantial 200-300 km east of the trench, where the seismicity rate changes are observed. Thus, the seismicity shutdown associated with the 2004 earthquake stress shadow furnishes an important test of the static Coulomb stress triggering hypothesis.

  4. System requirements. [Space systems

    SciTech Connect

    Austin, R.E.

    1982-06-01

    Requirements of future space systems, including large space systems, that operate beyond the space shuttle are discussed. Typical functions required of propulsion systems in this operational regime include payload placement, retrieval, observation, servicing, space debris control and support to large space systems. These functional requirements are discussed in conjunction with two classes of propulsion systems: (1) primary or orbit transfer vehicle (OTV) and (2) secondary or systems that generally operate within or relatively near an operational base orbit. Three propulsion system types are described in relation to these requirements: cryogenic OTV, teleoperator maneuvering system and a solar electric OTV.

  5. ATLAS software configuration and build tool optimisation

    NASA Astrophysics Data System (ADS)

    Rybkin, Grigory; Atlas Collaboration

    2014-06-01

    ATLAS software code base is over 6 million lines organised in about 2000 packages. It makes use of some 100 external software packages, is developed by more than 400 developers and used by more than 2500 physicists from over 200 universities and laboratories in 6 continents. To meet the challenge of configuration and building of this software, the Configuration Management Tool (CMT) is used. CMT expects each package to describe its build targets, build and environment setup parameters, dependencies on other packages in a text file called requirements, and each project (group of packages) to describe its policies and dependencies on other projects in a text project file. Based on the effective set of configuration parameters read from the requirements files of dependent packages and project files, CMT commands build the packages, generate the environment for their use, or query the packages. The main focus was on build time performance that was optimised within several approaches: reduction of the number of reads of requirements files that are now read once per package by a CMT build command that generates cached requirements files for subsequent CMT build commands; introduction of more fine-grained build parallelism at package task level, i.e., dependent applications and libraries are compiled in parallel; code optimisation of CMT commands used for build; introduction of package level build parallelism, i. e., parallelise the build of independent packages. By default, CMT launches NUMBER-OF-PROCESSORS build commands in parallel. The other focus was on CMT commands optimisation in general that made them approximately 2 times faster. CMT can generate a cached requirements file for the environment setup command, which is especially useful for deployment on distributed file systems like AFS or CERN VMFS. The use of parallelism, caching and code optimisation significantly-by several times-reduced software build time, environment setup time, increased the efficiency of

  6. 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

  7. The G-FAST Geodetic Earthquake Early Warning System: Operational Performance and Synthetic Testing

    NASA Astrophysics Data System (ADS)

    Crowell, B. W.; Schmidt, D. A.; Bodin, P.; Vidale, J. E.; Melbourne, T. I.; Santillan, V. M.

    2015-12-01

    The G-FAST (Geodetic First Approximation of Size and TIming) earthquake early warning module is part of a joint seismic and geodetic earthquake early warning system currently under development at the Pacific Northwest Seismic Network (PNSN). Our two-stage approach to earthquake early warning includes: (1) initial detection and characterization from PNSN strong-motion and broadband data with the ElarmS package within ShakeAlert, and then (2) modeling of GPS data from the Pacific Northwest Geodetic Array (PANGA). The two geodetic modeling modules are (1) a fast peak-ground-displacement magnitude and depth estimate and (2) a CMT-based finite fault inversion that utilizes coseismic offsets to compute earthquake extent, slip and magnitude. The seismic and geodetic source estimates are then combined in a decision module currently under development. In this presentation, we first report on the operational performance during the first several months that G-FAST has been live with respect to magnitude estimates, timing information, and stability. Secondly, we report on the performance of the G-FAST test system using simulated displacements from plausible Cascadian earthquake scenarios. The test system permits us to: (1) replay segments of actual seismic waveform data recorded from the PNSN and neighboring networks to investigate both earthquakes and noise conditions, and (2) broadcast synthetic data into the system to simulate signals we anticipate from earthquakes for which we have no actual ground motion recordings. The test system lets us also simulate various error conditions (latent and/or out-of-sequence data, telemetry drop-outs, etc.) in order to explore how best to mitigate them. For example, we show for a replay of the 2001 M6.8 Nisqually earthquake that telemetry drop-outs create the largest variability and biases in magnitude and depth estimates whereas latency only causes some variability towards the beginning of the recordings before quickly stabilizing

  8. Characterizing 3D Structure of Convective Momentum Transport Associated with the MJO Based on Contemporary Reanalyses

    NASA Astrophysics Data System (ADS)

    Oh, J.; Jiang, X.; Waliser, D. E.; Moncrieff, M. W.; Johnson, R. H.

    2013-12-01

    As one of the most prominent tropical atmospheric variability modes, the Madden-Julian Oscillation (MJO) exerts profound influences on global weather and climate, and serves as a critical predictability source for extend-range forecast. While credible representation of the MJO still represents a great challenge for current general circulation models (GCMs), previous studies on the vertical structure of the MJO have largely focused on collective impacts from multi-scale convective systems on thermodynamic properties of the MJO. Most recently, limited observational studies and idealized modeling work suggested that convective momentum transport (CMT) could also play an important role in interpreting the observed MJO features. In this study, the 3D CMT structure associated with the MJO is examined by analyzing model output from three recent high-quality reanalysis systems, including NOAA's Climate Forecast System Reanalysis (CFSR), NASA's Modern Era Retrospective-analysis for Research and Applications (MERRA), and ECMWF-the Year of Tropical Convection (YOTC) reanalysis. Consistent with previous cloud-resolving model study, a well-organized three-layer vertical structure in the CMT associated with the MJO is also discerned based on reanalyses. The result suggests that CMT tends to intensify the MJO circulation, particularly in the lower troposphere. Relative roles of meso-scale systems (MCS) and synoptic waves in contributing the total CMT profiles of the MJO will also be explored. Differences in CMT profiles in these several reanalysis models will be discussed.

  9. Rapid calculation of a Centroid Moment Tensor and waveheight predictions around the north Pacific for the 2011 off the Pacific coast of Tohoku Earthquake

    NASA Astrophysics Data System (ADS)

    Polet, Jascha; Thio, Hong Kie

    2011-07-01

    We present the results of a near real-time determination of a Centroid Moment Tensor for the 2011 Tohoku quake and the subsequent rapid prediction of Pacific coast tsunami waveheights based on these CMT parameters. Initial manual CMT results for this event were obtained within 23 minutes of origin time and fully automatic results were distributed by E-mail within 33 minutes. The mechanism, depth and moment magnitude were all well constrained, as was indicated by a bootstrapping analysis. Using an existing library of tsunami Green's functions, we computed predicted waveheights in the north Pacific for several scenarios of the Tohoku earthquake that are consistent with the CMT solution. Overall, these predicted waveheights correspond well with preliminary observations around the Pacific Rim. The predictions for North America were sent out three and a half hours after the origin time of the earthquake, but this system has the potential to provide these predictions within minutes after receiving the CMT solution.

  10. 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

  11. 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

  12. Validity of clinical use of the clock-drawing test in Thai elderly patients with memory problems.

    PubMed

    Jitapunkul, S; Worakul, P; Kiatprakoth, J

    2000-04-01

    Forty eight elderly Thai patients, 12 demented, 36 non-demented who consecutively attended a geriatric clinic, at Chulalongkorn University Hospital, with a memory problem were recruited in the study which aimed to determine the validity of the clock-drawing test (CDT) and Chula mental test (CMT). Subjects took the Chula Mental Test (CMT) and were asked to draw a clock on a preprinted 12 centimeters circle showing the time of 11:10. Clocks were scored using the Chula Clock-drawing Scoring System (CCSS). Ten subjects voluntarily participated and completed the WAIS test. The best cutoff score of the CMT and the CDT using diagnosis of dementia as a gold standard were 15 and 7 respectively. Sensitivity and specificity for detecting dementia by the CDT were 100 per cent and 94.1 per cent respectively. Sensitivity and specificity for detecting dementia by the CMT were 83.3 per cent 91.7 per cent. The likelihood ratio (95% confidence interval) of the CMT and the CDT were 10 (3.3-30.4) and 17 (4.4-65.2) respectively. There was a good correlation of the CDT score and the CMT score with the WAIS score. Although the CDT was better than the CMT in literate patients, the CDT had limitation of use among illiterate patients but not the CMT. The benefits of simultaneously application on dementia screening was shown. This study showed that the CDT was a valuable, brief method of dementia screening in elderly Thai patients with memory problems. PMID:10808691

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

    PubMed

    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; Burgess, Robert W

    2016-01-01

    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

  14. 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.

  15. Systems autonomy

    NASA Technical Reports Server (NTRS)

    Lum, Henry, Jr.

    1988-01-01

    Information on systems autonomy is given in viewgraph form. Information is given on space systems integration, intelligent autonomous systems, automated systems for in-flight mission operations, the Systems Autonomy Demonstration Project on the Space Station Thermal Control System, the architecture of an autonomous intelligent system, artificial intelligence research issues, machine learning, and real-time image processing.

  16. Immune System

    MedlinePlus

    ... How Can I Help a Friend Who Cuts? Immune System KidsHealth > For Teens > Immune System Print A A ... could put us out of commission. What the Immune System Does The immune (pronounced: ih-MYOON) system, which ...

  17. Stochastic models for plant microtubule self-organization and structure.

    PubMed

    Eren, Ezgi C; Dixit, Ram; Gautam, Natarajan

    2015-12-01

    One of the key enablers of shape and growth in plant cells is the cortical microtubule (CMT) system, which is a polymer array that forms an appropriately-structured scaffolding in each cell. Plant biologists have shown that stochastic dynamics and simple rules of interactions between CMTs can lead to a coaligned CMT array structure. However, the mechanisms and conditions that cause CMT arrays to become organized are not well understood. It is prohibitively time-consuming to use actual plants to study the effect of various genetic mutations and environmental conditions on CMT self-organization. In fact, even computer simulations with multiple replications are not fast enough due to the spatio-temporal complexity of the system. To redress this shortcoming, we develop analytical models and methods for expeditiously computing CMT system metrics that are related to self-organization and array structure. In particular, we formulate a mean-field model to derive sufficient conditions for the organization to occur. We show that growth-prone dynamics itself is sufficient to lead to organization in presence of interactions in the system. In addition, for such systems, we develop predictive methods for estimation of system metrics such as expected average length and number of CMTs over time, using a stochastic fluid-flow model, transient analysis, and approximation algorithms tailored to our problem. We illustrate the effectiveness of our approach through numerical test instances and discuss biological insights. PMID:25700800

  18. Sensitivity and specificity of infrared thermography in detection of subclinical mastitis in dairy cows.

    PubMed

    Polat, B; Colak, A; Cengiz, M; Yanmaz, L E; Oral, H; Bastan, A; Kaya, S; Hayirli, A

    2010-08-01

    The objectives of this experiment were to determine interrelationships among mastitis indicators and evaluate the subclinical mastitis detection ability of infrared thermography (IRT) in comparison with the California Mastitis Test (CMT). Somatic cell count (SCC), CMT, and udder skin surface temperature (USST) data were compiled from 62 Brown Swiss dairy cows (days in milk=117+/-51, milk yield=14.7+/-5.2 kg; mean +/- SD). The CORR, REG, and NLIN procedures of Statistical Analysis System (SAS Institute Inc., Cary, NC) were employed to attain interrelationships among mastitis indicators. The diagnostic merit of IRT as an indirect measure of subclinical mastitis was compared with CMT using the receiver operating characteristics curves. The udder skin surface temperature was positively correlated with the CMT score (r=0.86) and SCC (r=0.73). There was an exponential increase in SCC (SCC, x10(3) cells/mL=22.35 x e(1.31 x CMT score); R(2)=0.98) and a linear increase in USST (USST, degrees C=33.45+1.08 x CMT score; R(2)=0.75) as the CMT score increased. As SCC increased, USST increased logarithmically [USST, degrees C=28.72+0.49 x ln(SCC, x10(3) cells/mL); R(2)=0.72]. The USST for healthy quarters (SCC 400,000 cells/mL; n=135) (mean +/- SE; 33.45+/-0.09 vs. 35.80+/-0.08 degrees C). The sensitivity, specificity, positive likelihood ratio, negative likelihood ratio, positive predictive value, and negative predictive value were 95.6, 93.6, 14.97, 0.05, 95.0, and 93.6, respectively, for IRT and 88.9, 98.9, 83.56, 0.11, 99.2, and 86.1, respectively, for CMT. The area under the receiver operating characteristics curve for IRT and CMT was not different. In conclusion, as a noninvasive and quick tool, IRT can be employed for screening subclinical mastitis via measuring USST, with a high predictive diagnostic ability similar to CMT when microbiological culturing is unavailable. However, the

  19. 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…

  20. 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

  1. Discovery Systems

    NASA Technical Reports Server (NTRS)

    Pell, Barney

    2003-01-01

    A viewgraph presentation on NASA's Discovery Systems Project is given. The topics of discussion include: 1) NASA's Computing Information and Communications Technology Program; 2) Discovery Systems Program; and 3) Ideas for Information Integration Using the Web.

  2. Crystal Systems.

    ERIC Educational Resources Information Center

    Schomaker, Verner; Lingafelter, E. C.

    1985-01-01

    Discusses characteristics of crystal systems, comparing (in table format) crystal systems with lattice types, number of restrictions, nature of the restrictions, and other lattices that can accidently show the same metrical symmetry. (JN)

  3. Concentrator Systems

    NASA Astrophysics Data System (ADS)

    Luque-Heredia, Ignacio; Luque, Antonio

    2015-10-01

    The following sections are included: * Introduction * The early development of CPV * Concentrator solar cells * Optics for photovoltaic concentrators * Photovoltaic concentration modules * Tracking systems for photovoltaic concentration * High-concentration systems * Rating and performance * Cost considerations * Conclusions * References

  4. 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.

  5. 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.

  6. Linked Systems.

    ERIC Educational Resources Information Center

    Association of Research Libraries, Washington, DC.

    Three papers are compiled here for research library directors: (1) "Background: Open Systems Interconnection," in which David F. Bishop provides fundamental background information to explain the concept of the emerging technology of linked systems and open systems interconnection--i.e., an agreed upon standard set of conventions or rules that,…

  7. Expert systems and fuzzy systems

    SciTech Connect

    Negoita, C.

    1985-01-01

    This book examines the design of the expert computer system and how fuzzy systems can be used to deal with imprecise information. As the author explores the effects of semantic systems on decision support systems, he asserts that the utilization of fuzzy set theory can help an expert system draw from its knowledge base more efficiently and therefore make more accurate and reliable decisions. The book includes realistic status reports in approximate reasoning and knowledge representation that are supported by a ''theory of categories'' mathematical approach. The differences between symbolic and semantic manipulation are outline, and detailed information is given on the actual theory of knowledge-based systems.

  8. 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

  9. 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

  10. [Information systems].

    PubMed

    Rodríguez Maniega, José Antonio; Trío Maseda, Reyes

    2005-03-01

    The arrival of victims of the terrorist attacks of 11 March at the hospital put the efficiency of its information systems to the test. To be most efficient, these systems should be simple and directed, above all, to the follow-up of victims and to providing the necessary information to patients and families. A specific and easy to use system is advisable. PMID:15771852

  11. 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.

  12. 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

  13. A compound heterozygous mutation in HADHB gene causes an axonal Charcot-Marie-tooth disease

    PubMed Central

    2013-01-01

    Background Charcot-Marie-Tooth disease (CMT) is a heterogeneous disorder of the peripheral nervous system. So far, mutations in hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), beta subunit (HADHB) gene exhibit three distinctive phenotypes: severe neonatal presentation with cardiomyopathy, hepatic form with recurrent hypoketotic hypoglycemia, and later-onset axonal sensory neuropathy with episodic myoglobinuria. Methods To identify the causative and characterize clinical features of a Korean family with motor and sensory neuropathies, whole exome study (WES), histopathologic study of distal sural nerve, and lower limb MRIs were performed. Results WES revealed that a compound heterozygous mutation in HADHB is the causative of the present patients. The patients exhibited an early-onset axonal sensorimotor neuropathy without episodic myoglobinuria, and showed typical clinical and electrophysiological features of CMT including predominant distal muscle weakness and atrophy. Histopathologic findings of sural nerve were compatible with an axonal CMT neuropathy. Furthermore, they didn’t exhibit any other symptoms of the previously reported HADHB patients. Conclusions These data implicate that mutation in HADHB gene can also cause early-onset axonal CMT instead of typical manifestations in mitochondrial trifunctional protein (MTP) deficiency. Therefore, this study is the first report of a new subtype of autosomal recessive axonal CMT by a compound heterozygous mutation in HADHB, and will expand the clinical and genetic spectrum of HADHB. PMID:24314034

  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. PMID:26423936

  15. 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. PMID:27025386

  16. Postural stabilization and balance assessment in Charcot–Marie–Tooth 1A subjects

    PubMed Central

    Lencioni, T.; Rabuffetti, M.; Piscosquito, G.; Pareyson, D.; Aiello, A.; Di Sipio, E.; Padua, L.; Stra, F.; Ferrarin, M.

    2014-01-01

    The aim of the present study was to assess postural stabilization skill in adult subjects affected by Charcot–Marie–Tooth disease (CMT) type 1A. For this purpose ground reaction force (GRF) was measured by means of a piezoelectric force platform during the sit-to-stand (STS) movement, until a steady state erect posture was achieved. Specific indexes to quantify Centre of Mass acceleration, both during postural stabilization and during quiet standing, were computed using a mathematical model. Forty-seven CMT1A subjects were recruited for the study, and the control group was formed by forty-one age- and sex-matched healthy subjects. The results show that CMT1A subjects are less stable than controls during the quiet stance. Greater difficulty (high values of Yinf, the final instability rate) to maintain erect posture appears to be mainly associated with plantar-flexor muscle weakness, rather than to damage of the proprioceptive system. The worst performances shown by CMT1A subjects in the stabilization phase (high values of I, the global index of postural stabilization performance) seem to be associated with reduced muscle strength and the loss of large sensory nerve fibres. Distal muscle weakness appears to affect both postural stabilization and quiet erect posture. The presented protocol and the analysis of postural stabilization parameters provide useful information on CMT1A balance disorders. PMID:25082324

  17. Postural stabilization and balance assessment in Charcot-Marie-Tooth 1A subjects.

    PubMed

    Lencioni, T; Rabuffetti, M; Piscosquito, G; Pareyson, D; Aiello, A; Di Sipio, E; Padua, L; Stra, F; Ferrarin, M

    2014-09-01

    The aim of the present study was to assess postural stabilization skill in adult subjects affected by Charcot-Marie-Tooth disease (CMT) type 1A. For this purpose ground reaction force (GRF) was measured by means of a piezoelectric force platform during the sit-to-stand (STS) movement, until a steady state erect posture was achieved. Specific indexes to quantify Centre of Mass acceleration, both during postural stabilization and during quiet standing, were computed using a mathematical model. Forty-seven CMT1A subjects were recruited for the study, and the control group was formed by forty-one age- and sex-matched healthy subjects. The results show that CMT1A subjects are less stable than controls during the quiet stance. Greater difficulty (high values of Yinf, the final instability rate) to maintain erect posture appears to be mainly associated with plantar-flexor muscle weakness, rather than to damage of the proprioceptive system. The worst performances shown by CMT1A subjects in the stabilization phase (high values of I, the global index of postural stabilization performance) seem to be associated with reduced muscle strength and the loss of large sensory nerve fibres. Distal muscle weakness appears to affect both postural stabilization and quiet erect posture. The presented protocol and the analysis of postural stabilization parameters provide useful information on CMT1A balance disorders. PMID:25082324

  18. 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. PMID:25440412

  19. Outbreak of Subclinical Mastitis in a Flock of Dairy Sheep Associated with Burkholderia cepacia Complex Infection

    PubMed Central

    Berriatua, E.; Ziluaga, I.; Miguel-Virto, C.; Uribarren, P.; Juste, R.; Laevens, S.; Vandamme, P.; Govan, J. R. W.

    2001-01-01

    An outbreak of subclinical mastitis in a flock of 620 milking sheep was investigated. Microbiological and epidemiological analyses identified the causative agent as belonging to the Burkholderia cepacia complex (formerly Pseudomonas cepacia). Every ewe in the milking flock was individually tested for subclinical mastitis on two separate occasions, 6 weeks apart, by the California (rapid) mastitis test (CMT). The proportion of CMT-positive ewes was 69 of 393 (17.6%) on the first sampling and 27 of 490 (5.5%) on the second sampling. Pure B. cepacia cultures identified with the API 20 NE system were grown from 64 of 96 (66.7%) CMT-positive ewes and from 1 of 33 (3.0%) CMT-negative ewes. Statistical analysis confirmed the significant association between a positive CMT result and a positive culture result for B. cepacia complex. Additional polyphasic taxonomic analyses of eight isolates showed that seven belonged to B. cepacia genomovar III; the remaining isolate was identified as Burkholderia vietnamiensis (formerly B. cepacia genomovar V). Bacteriological investigation of samples from milking equipment and other environmental sites failed to identify “B. cepacia” in any of the samples taken. To our knowledge, this is the first report of an outbreak of natural infection in animals caused by B. cepacia complex and the first description of B. cepacia complex infection in sheep. PMID:11230416

  20. 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. PMID:18697926

  1. 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

  2. Educational Systems.

    ERIC Educational Resources Information Center

    Archer, Margaret

    1981-01-01

    Presents a general review of research on educational systems, with emphasis on variations in the definition of an educational system, neglected questions, areas of major concern, research needs, traditional and modern stands in the sociology of education, educational politics, and researcher bias. (DB)

  3. Systemic Change.

    ERIC Educational Resources Information Center

    Tosti, Donald T.

    2000-01-01

    Examines three factors contributing to organizational success: use of a systemic model to design and guide the change effort; analysis of interdependencies within the system and of the change potential; and use of communication methods to help ensure that the change will be supported. Presents characteristics of successful change efforts with…

  4. Potential applications of synchrotron computed microtomography to soil science

    SciTech Connect

    Spanne, P.; Jones, K.W. ); Prunty, L.D. ); Anderson, S.H. )

    1993-01-01

    Synchrotron x-ray computed microtomography (CMT) can be used to make non-destructive tomographic sections with spatial resolutions of a few [mu]m. This resolution presents possibilities for study of soil-fluid interactions on a spatial scale hitherto unreachable. Details of a CMT apparatus in operation at the Brookhaven National Synchrotron Light Source X26 beam line are presented and prospects for future investigations of test systems have been made and results for wet and dry samples of glass beads and sand samples are given to show the power of the system.

  5. Potential applications of synchrotron computed microtomography to soil science

    SciTech Connect

    Spanne, P.; Jones, K.W.; Prunty, L.D.; Anderson, S.H.

    1993-01-01

    Synchrotron x-ray computed microtomography (CMT) can be used to make non-destructive tomographic sections with spatial resolutions of a few {mu}m. This resolution presents possibilities for study of soil-fluid interactions on a spatial scale hitherto unreachable. Details of a CMT apparatus in operation at the Brookhaven National Synchrotron Light Source X26 beam line are presented and prospects for future investigations of test systems have been made and results for wet and dry samples of glass beads and sand samples are given to show the power of the system.

  6. 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.

  7. Processing system

    NASA Technical Reports Server (NTRS)

    Hilland, J. E.

    1983-01-01

    To implement the analysis techniques and to provide end-to-end processing, a system was designed with the following capabilities: receive and catalog data from many sources; organize the data on mass storage for rapid access; edit for reasonableness; create new data sets by sorting on parameter, averaging and merging; provide statistical analysis and display tools; and distribute data on demand. Consideration was given to developing a flexible system that could meet immediate workshop needs and respond to future requirements. System architecture and data set details implemented are discussed.

  8. 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.)

  9. System identification

    NASA Astrophysics Data System (ADS)

    Juang, Jer-Nan

    Major issues in system identification are summarized and recent advances are reviewed. Modal testing and system identification used in control theory are examined, and the mathematical relationships and conversions of the models appropriate to modal testing and those appropriate to modern control design methods are discussed. The importance of obtaining input and output matrices in modal testing is emphasized, and the changes that may be needed in modal testing procedures to meet the needs of the control system designer are addressed. Directions for future research are considered.

  10. Saturn Systems.

    PubMed

    U Rehman, Habib; McKee, Nida A; McKee, Michael L

    2016-01-15

    Several ring systems (Saturn systems) have been studied using DFT methods that include dispersion effects. Comparison with X-ray structures are made with three systems, and the agreement is quite good. Binding enthalpies and binding free energies in dichloromethane and toluene have been computed. The effect of an encapsulated lithium cation is accessed by comparing C60 @(C6 H4 )10 and [Li@C60 @(C6 H4 )10 ](+). The [Li@C60 ](+) cation is a much better acceptor than C60 which leads to greater donor-acceptor interactions and larger charge transfer from the ring to [Li@C60 ](+). PMID:26096724

  11. Lymph system

    MedlinePlus

    ... the lymph nodes make more infection-fighting white blood cells, which cause the nodes to swell. The swollen nodes are sometimes felt in the neck, under the arms, and groin. The lymph system includes the: Tonsils Adenoids Spleen Thymus

  12. 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.

  13. [Systemic urticarias].

    PubMed

    Hachulla, E

    2003-05-01

    Systemic urticaria remains a challenge in terms of etiology, investigation and management. Most of cases are urticarial vasculitis consequence of inflammatory injury of capillaries and postcapillary venules in the skin. If hypocomplementemic urticarial vasculitis syndrome is a classical cause, the majority of patients have an underlying systemic disease like systemic lupus erythematosus, Sjögren's syndrome, mixed cryoglobulinemia, Still disease or cancer. Others systemic urticaria have been reported without clearly evidence of vasculitis like in primary or acquired angioedema, hereditary periodic fever syndromes and in some thyroiditis. Diagnosis needs a step to step procedure. Treatment depends the underlying disease. Some patients respond to nonsteroidal antiinflammatory drugs, some other need corticosteroids or immunosuppression. If urticarial vasculitis seems isolated in the absence of chronic obstructive pulmonary disease, antihistamines, nonsteroidal antiinflammatory drugs, colchicine, dapsone or hydroxychloroquine must be first used. PMID:12843810

  14. 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.

  15. 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.

  16. Recommender systems

    NASA Astrophysics Data System (ADS)

    Lü, Linyuan; Medo, Matúš; Yeung, Chi Ho; Zhang, Yi-Cheng; Zhang, Zi-Ke; Zhou, Tao

    2012-10-01

    The ongoing rapid expansion of the Internet greatly increases the necessity of effective recommender systems for filtering the abundant information. Extensive research for recommender systems is conducted by a broad range of communities including social and computer scientists, physicists, and interdisciplinary researchers. Despite substantial theoretical and practical achievements, unification and comparison of different approaches are lacking, which impedes further advances. In this article, we review recent developments in recommender systems and discuss the major challenges. We compare and evaluate available algorithms and examine their roles in the future developments. In addition to algorithms, physical aspects are described to illustrate macroscopic behavior of recommender systems. Potential impacts and future directions are discussed. We emphasize that recommendation has great scientific depth and combines diverse research fields which makes it interesting for physicists as well as interdisciplinary researchers.

  17. A Multi-Scale Interaction Model for Madden-Julian Oscillation

    NASA Astrophysics Data System (ADS)

    Wang, B.; Liu, F.

    2010-12-01

    Madden-Julian oscillation (MJO) is an equatorial, planetary scale circulation system coupled with a multi-scale convective complex. The nature and roles of multi-scale interaction (MSI) on MJO dynamics has not been well understood. Here we formulate a prototype theoretical model to advance our understanding the MSI in MJO. The model integrates three essential elements: a) large scale equatorial wave dynamics driven by boundary layer frictional convergence instability (FCI), b) effects of multi-cloud heating and an instability arising from synoptic system-induced convective momentum transfer (CMT), and c) interaction between the planetary and synoptic systems. We show that the CMT mechanism tends to yield a growing stationary mode with a quadrupole-vortex horizontal structure (enhanced Rossby wave component); whereas the FCI favors a fast eastward-moving mode with a Gill-Pattern structure (enhanced Kelvin wave response). The MSI instability can stem from either FCI or CMT mechanisms or both, depending on the ratio of deep convective versus stratiform/congestus heating. With increasing stratiform/congestus heating, the FCI weakens while the CMT becomes more effective. A growing MSI mode has a mixed horizontal structure of CMT and FCI and prefers slow eastward propagation. The FCI sets the eastward propagation, and CMT plays a vital role in slowing down the propagation speed. These results encourage further observational diagnosis of multi-cloud structure and heating profiles in the MJO convective complex and improvement of models’ capability in reproducing correct partitioning of cloud amounts between deep convective and stratiform/congestus clouds.

  18. 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.

  19. 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. PMID:25072975

  20. 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

  1. 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.

  2. 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. PMID:21701196

  3. 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. PMID:24617751

  4. 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.

  5. Memory systems.

    PubMed

    Wolk, David A; Budson, Andrew E

    2010-08-01

    Converging evidence from patient and neuroimaging studies suggests that memory is a collection of abilities that use different neuroanatomic systems. Neurologic injury may impair one or more of these memory systems. Episodic memory allows us to mentally travel back in time and relive an episode of our life. Episodic memory depends on the hippocampus, other medial temporal lobe structures, the limbic system, and the frontal lobes, as well as several other brain regions. Semantic memory provides our general knowledge about the world and is unconnected to any specific episode of our life. Although semantic memory likely involves much of the neocortex, the inferolateral temporal lobes (particularly the left) are most important. Procedural memory enables us to learn cognitive and behavioral skills and algorithms that operate at an automatic, unconscious level. Damage to the basal ganglia, cerebellum, and supplementary motor area often impair procedural memory. PMID:22810510

  6. 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

  7. 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.

  8. 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

  9. Lindenmayer Systems

    NASA Astrophysics Data System (ADS)

    Honkala, Juha

    The theory of Lindenmayer systems studies free monoid morphisms, free monoid substitutions and their iterations. In this chapter, we discuss similar ideas in a more general framework. Instead of a free monoid, we consider the free semi-algebra S<Σ*> consisting of polynomials with non-commuting variables in Σ and coefficients in a semiring S and we study the iteration of endomorphisms of S<Σ*>. We allow various modes of iteration and we consider various classes of morphisms. Classical L systems are obtained as special cases by taking S to be the Boolean semiring. Our approach also generalizes the theory of algebraic series in noncommuting variables.

  10. 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.

  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. Community Surface Dynamics Modeling System and its CSDMS Modeling Tool to couple models and data (Invited)

    NASA Astrophysics Data System (ADS)

    Syvitski, J. P.; Csdms Scientific; Software Team

    2010-12-01

    CSDMS is the virtual home for a diverse community who foster and promote the modeling of earth surface processes, with emphasis on the movement of fluids, sediment and solutes through landscapes, seascapes and through their sedimentary basins. CSDMS develops, integrates, disseminates & archives software (> 150 models and 3million+ lines of code) that reflects and predicts earth surface processes over a broad range of time and space scales. CSDMS deals with the Earth's surface—the ever-changing, dynamic interface between lithosphere, hydrosphere, cryosphere, and atmosphere. CSDMS employs state-of-the-art architectures, interface standards and frameworks that make it possible to convert stand-alone models into flexible, "plug-and-play" components that can be assembled into larger applications. The CSDMS model-coupling environment offers language interoperability, structured and unstructured grids, and serves as a migration pathway for surface dynamics modelers towards High-Performance Computing (HPC). The CSDMS Modeling Tool is a key product of the overall project, as it allows earth scientists with relatively modest computer coding experience to use the CSDMS modules for earth surface dynamics research and education. The CMT Tool is platform independent. CMT can easily couple models that have followed the CSDMS protocols for model contribution: 1) Open-source license; 2) Available; 3) Vetted; 4) Open-source language; 5) Refactored for componentization; 6) Metadata & test files; 7) Clean and documented using keywords.

  13. Systems Biology

    SciTech Connect

    Wiley, H S.

    2006-06-01

    The biology revolution over the last 50 years has been driven by the ascendancy of molecular biology. This was enthusiastically embraced by most biologists because it took us into increasingly familiar territory. It took mysterious processes, such as the replication of genetic material and assigned them parts that could be readily understood by the human mind. When we think of ''molecular machines'' as being the underlying basis of life, we are using a paradigm derived from everyday experience. However, the price that we paid was a relentless drive towards reductionism and the attendant balkanization of biology. Now along comes ''systems biology'' that promises us a solution to the problem of ''knowing more and more about less and less''. Unlike molecular biology, systems biology appears to be taking us into unfamiliar intellectual territory, such as statistics, mathematics and computer modeling. Not surprisingly, systems biology has met with widespread skepticism and resistance. Why do we need systems biology anyway and how does this new area of research promise to change the face of biology in the next couple of decades?

  14. System Dynamics

    NASA Astrophysics Data System (ADS)

    Morecroft, John

    System dynamics is an approach for thinking about and simulating situations and organisations of all kinds and sizes by visualising how the elements fit together, interact and change over time. This chapter, written by John Morecroft, describes modern system dynamics which retains the fundamentals developed in the 1950s by Jay W. Forrester of the MIT Sloan School of Management. It looks at feedback loops and time delays that affect system behaviour in a non-linear way, and illustrates how dynamic behaviour depends upon feedback loop structures. It also recognises improvements as part of the ongoing process of managing a situation in order to achieve goals. Significantly it recognises the importance of context, and practitioner skills. Feedback systems thinking views problems and solutions as being intertwined. The main concepts and tools: feedback structure and behaviour, causal loop diagrams, dynamics, are practically illustrated in a wide variety of contexts from a hot water shower through to a symphony orchestra and the practical application of the approach is described through several real examples of its use for strategic planning and evaluation.

  15. Metric System.

    ERIC Educational Resources Information Center

    Del Mod System, Dover, DE.

    This autoinstructional unit deals with the identification of units of measure in the metric system and the construction of relevant conversion tables. Students in middle school or in grade ten, taking a General Science course, can handle this learning activity. It is recommended that high, middle or low level achievers can use the program.…

  16. Irrigation System

    NASA Technical Reports Server (NTRS)

    1984-01-01

    Under contract with Marshall Space Flight Center, Midwest Research Institute compiled a Lubrication Handbook intended as a reference source for designers and manufacturers of aerospace hardware and crews responsible for maintenance of such equipment. Engineers of Lindsay Manufacturing Company learned of this handbook through NASA Tech Briefs and used it for supplemental information in redesigning gear boxes for their center pivot agricultural irrigation system.

  17. Cardiovascular system

    MedlinePlus Videos and Cool Tools

    The cardiovascular system is composed of the heart and the network of arteries, veins, and capillaries that transport blood throughout the body. The ... which they are eliminated. Most of the blood is made up of a watery, protein-laden fluid ...

  18. 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.

  19. 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...

  20. 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…

  1. STAR System.

    ERIC Educational Resources Information Center

    Doverspike, James E.

    The STAR System is a developmental guidance approach to be used with elementary school children in the 5th or 6th grades. Two basic purposes underlie STAR: to increase learning potential and to enhance personal growth and development. STAR refers to 4 basic skills: sensory, thinking, adapting, and revising. Major components of the 4 skills are:…

  2. Tunable edge-mode-based mid-infrared plasmonically induced transparency in the coupling system of coplanar graphene ribbons

    NASA Astrophysics Data System (ADS)

    Li, Hong-Ju; Wang, Ling-Ling; Zhang, Bing-Hua; Zhai, Xiang

    2016-01-01

    The graphene ribbon waveguide with two short parallel, coupled coplanar strips is investigated. Because of the extreme destructive interference of the short strip resonators, an outstanding plasmonically induced transparency (PIT) window with a group time delay up to 0.28 ps is achieved in the mid-infrared region, with an excellent ultraslow-light feature. The PIT window is controlled by varying the coupling distance between resonators and is tuned dynamically by a small change in the chemical potential. Numerical results are confirmed using the coupled-mode theory (CMT). The planar structure will benefit the fabrication of plasmonic circuits for slow light and optical switching.

  3. MMP inhibition in prostate cancer.

    PubMed

    Lokeshwar, B L

    1999-06-30

    Matrix metalloproteinases (MMPs) play a significant role during the development and metastasis of prostate cancer (CaP). CaP cells secrete high levels of MMPs and low levels of endogenous MMP inhibitors (TIMPs), thus creating an excess balance of MMPs. Established CaP cell lines that express high levels of MMPs frequently metastasize to the bone and the lungs. Drugs such as Taxol and alendronate that reduce cell motility and calcium metabolism reduce bony metastasis of xenografted CaP tumors. We tested several synthetic, nontoxic inhibitors of MMPs that can be administered orally, including doxycycline (DC) and chemically modified tetracyclines (CMTs) on CaP cells in vitro and on a rat CaP model in vivo. Among several anti-MMP agents tested, CMT-3 (6-deoxy, 6-demethyl,4-de-dimethylamino tetracycline) showed highest activity against CaP cell invasion and cell proliferation. Micromolar concentration of CMT-3 and DC inhibited both the secretion and activity of MMPs by CaP cells. When tested for in vivo efficacy in the Dunning rat CaP model by daily oral gavage, CMT-3 and DC both reduced the lung metastases (> 50%). CMT-3, but not DC, inhibited tumor incidence (55 +/- 9%) and also reduced the tumor growth rate (27 +/- 9.3%). More significantly, the drugs showed minimum systemic toxicity. Ongoing studies indicate that CMT-3 may inhibit the skeletal metastases of CaP cells and delay the onset of paraplegia due to lumbar metastases. These preclinical studies provide the basis for clinical trials of CMT-3 for the treatment of metastatic disease. PMID:10415736

  4. 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

  5. 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.

  6. Burner systems

    DOEpatents

    Doherty, Brian J.

    1984-07-10

    A burner system particularly useful for downhole deployment includes a tubular combustion chamber unit housed within a tubular coolant jacket assembly. The combustion chamber unit includes a monolithic tube of refractory material whose inner surface defines the combustion zone. A metal reinforcing sleeve surrounds and extends the length of the refractory tube. The inner surface of the coolant jacket assembly and outer surface of the combustion chamber unit are dimensioned so that those surfaces are close to one another in standby condition so that the combustion chamber unit has limited freedom to expand with that expansion being stabilized by the coolant jacket assembly so that compression forces in the refractory tube do not exceed about one-half the safe compressive stress of the material; and the materials of the combustion chamber unit are selected to establish thermal gradient parameters across the combustion chamber unit to maintain the refractory tube in compression during combustion system start up and cool down sequences.

  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. 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...

  9. 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.

  10. 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.

  11. Mine system

    SciTech Connect

    Stoppani, B.R.

    1983-10-04

    A mine system comprises at least one mining machine adapted to haul itself, in a reciprocating manner, along a mineral face, and a control box housing means to control the various electrical elements of the machine(s), the box being located in a mine roadway at one end of the mineral face along which the machine(s) is reciprocating, and the box being electrically connected to a terminal box housed in a body of the machine(s).

  12. 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.

  13. 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.

  14. Systemic amyloidosis.

    PubMed

    Wechalekar, Ashutosh D; Gillmore, Julian D; Hawkins, Philip N

    2016-06-25

    Tissue deposition of protein fibrils causes a group of rare diseases called systemic amyloidoses. This Seminar focuses on changes in their epidemiology, the current approach to diagnosis, and advances in treatment. Systemic light chain (AL) amyloidosis is the most common of these conditions, but wild-type transthyretin cardiac amyloidosis (ATTRwt) is increasingly being diagnosed. Typing of amyloid fibrils, a critical determinant of therapy, has improved with the wide availability of laser capture and mass spectrometry from fixed histological tissue sections. Specific and accurate evaluation of cardiac amyloidosis is now possible using cardiac magnetic resonance imaging and cardiac repurposing of bone scintigraphy tracers. Survival in AL amyloidosis has improved markedly as novel chemotherapy agents have become available, but challenges remain in advanced disease. Early diagnosis, a key to better outcomes, still remains elusive. Broadening the amyloid-specific therapeutic landscape to include RNA inhibitors, fibril formation stabilisers and inhibitors, and immunotherapeutic targeting of amyloid deposits holds promise to transform outcomes in systemic amyloidoses. PMID:26719234

  15. 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.

  16. 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.

  17. Bearing system

    DOEpatents

    Kapich, Davorin D.

    1987-01-01

    A bearing system includes backup bearings for supporting a rotating shaft upon failure of primary bearings. In the preferred embodiment, the backup bearings are rolling element bearings having their rolling elements disposed out of contact with their associated respective inner races during normal functioning of the primary bearings. Displacement detection sensors are provided for detecting displacement of the shaft upon failure of the primary bearings. Upon detection of the failure of the primary bearings, the rolling elements and inner races of the backup bearings are brought into mutual contact by axial displacement of the shaft.

  18. 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.

  19. 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.

  20. Videobasierte Systeme

    NASA Astrophysics Data System (ADS)

    Knoll, Peter

    Videosensoren spielen für Fahrerassistenz systeme eine zentrale Rolle, da sie die Interpretation visueller Informationen (Objektklassifikation) gezielt unterstützen. Im Heckbereich kann die Video sensorik in der einfachsten Variante die ultraschallbasierte Einparkhilfe bei Einpark- und Rangiervorgängen unterstützen. Beim Nachtsichtsystem NightVision wird das mit Infrarotlicht angestrahlte Umfeld vor dem Fahrzeug mit einer Frontkamera aufgenommen und im Fahrzeugcockpit auf einem Display dem Fahrer angezeigt (s. Nachtsichtsysteme). Andere Fahrerassistenzsysteme verarbeiten die Videosignale und generieren daraus gezielt Informationen, die für eigenständige Funktionen (z. B. Spurverlassenswarner) oder aber als Zusatzinformation für andere Funktionen ausgewertet werden (Sensordatenfusion).

  1. 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.

  2. Dataflow systems

    SciTech Connect

    Cohen, A.T.

    1982-01-01

    Dataflow languages are stream-oriented and functional, and assume a different operating environment. Programs in these languages are functions which define output as a function of input, with each program being a composition of subfunctions. There is no notion of a global store and, hence, there are no assignment statements. Control flow is determined by the availability of data (function arguments) and need not be explicitly specified by the programmer. The use and development of dataflow languages are motivated by three considerations. They represent parallelism naturally, particularly as it occurs in networks and distributed systems. They readily support very high level programming and their modularity and lack of side-effects makes their programs amenable to analysis. The paper briefly discusses their advantages in each of these three areas. 32 references.

  3. 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.

  4. 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. PMID:22562485

  5. Intelligent Engine Systems: Bearing System

    NASA Technical Reports Server (NTRS)

    Singh, Arnant P.

    2008-01-01

    The overall requirements necessary for sensing bearing distress and the related criteria to select a particular rotating sensor were established during the phase I. The current phase II efforts performed studies to evaluate the Robustness and Durability Enhancement of the rotating sensors, and to design, and develop the Built-in Telemetry System concepts for an aircraft engine differential sump. A generic test vehicle that can test the proposed bearing diagnostic system was designed, developed, and built. The Timken Company, who also assisted with testing the GE concept of using rotating sensors for the differential bearing diagnostics during previous phase, was selected as a subcontractor to assist General Electric (GE) for the design, and procurement of the test vehicle. A purchase order was prepared to define the different sub-tasks, and deliverables for this task. The University of Akron was selected to provide the necessary support for installing, and integrating the test vehicle with their newly designed test facility capable of simulating the operating environment for the planned testing. The planned testing with good and damaged bearings will be on hold pending further continuation of this effort during next phase.

  6. 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.

  7. [Systemic sclerosis].

    PubMed

    Tamborrini, Giorgio; Distler, Meike; Distler, Oliver

    2008-05-01

    Systemic sclerosis (SSc) is a severe fibrotic multiorgan connective tissue disease. Vascular abnormalities such as fingertip ulcers and Raynaud's syndrome as well as involvement of organs including the lungs, heart, kidney and the gastrointestinal tract are prominent features of the disease. There are currently no disease modifying drugs available that can modify the course of the disease. In this review we will discuss medications that have been found to be effective in improving specific organ involvement due to SSc. For the treatment of gastroesophageal reflux disease (GERD), proton pump inhibitors are effective agents. In the setting of clinically significant gastrointestinal dysmotility, metoclopramide, erythromycin and octreotide may be beneficial. Small bowel bacterial overgrowth should be treated with oral antibiotics. Angiotensin converting enzyme inhibitors are the first-line agents for acute renal crisis. A variety of treatment options are available for Raynaud's phenomenon and include calcium channel blockers, iloprost (i. v.), losartan, fluoxetine and sildenafil. Fingertip ulcers can be prevented by using the endothelin receptor antagonist bosentan. The therapeutic options for treatment of pulmonary hypertension associated with SSc include bosentan, sildenafil and various prostacyclin analogs (eg, epoprostenol, treprostinil, iloprost). Sitaxentan, ambrisentan and new phosphodiesterase-5 inhibitors could be new options for therapy as well. Therapeutic options for interstitial lung fibrosis include cyclophosphamide, however, clinical effects are mild to moderate. Methotrexate has been used to treat skin fibrosis and can be beneficial when arthritis is present. PMID:18552072

  8. 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.

  9. 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…

  10. System safety education focused on system management

    NASA Technical Reports Server (NTRS)

    Grose, V. L.

    1971-01-01

    System safety is defined and characteristics of the system are outlined. Some of the principle characteristics include role of humans in hazard analysis, clear language for input and output, system interdependence, self containment, and parallel analysis of elements.

  11. Focal mechanism determinations of earthquakes along the North Anatolian fault, beneath the Sea of Marmara and the Aegean Sea

    NASA Astrophysics Data System (ADS)

    Nakano, Masaru; Citak, Seckin; Kalafat, Doğan

    2015-09-01

    We determined the centroid moment tensor (CMT) solutions of earthquakes that occurred along the North Anatolian fault (NAF) beneath the Sea of Marmara and the Aegean Sea, using data obtained from Turkey's broad-band seismograph network. The CMT solution of the 2014 Aegean Sea earthquake ( Mw 6.9) represents a strike-slip fault, consistent with the geometry of the 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. 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.

  12. Randomised clinical trial evaluating best-corrected visual acuity and central macular thickness after 532-nm subthreshold laser grid photocoagulation treatment in diabetic macular oedema

    PubMed Central

    Pei-pei, W; Shi-zhou, H; Zhen, T; Lin, L; Ying, L; Jiexiong, O; Wen-bo, Z; Chen-jin, J

    2015-01-01

    Purpose To compare best-corrected visual acuity (BCVA) and central macular thickness (CMT) after 532-nm subthreshold laser grid photocoagulation and threshold laser grid photocoagulation for the treatment of diabetic macular oedema (DME). Patients and methods Twenty-three patients (46 eyes) with binocular DME were enroled in this study. The two eyes of each patient were divided into a subthreshold photocoagulation group and a threshold photocoagulation group. The eyes of the subthreshold group underwent 532-nm patter scan laser system (PASCAL) 50% end point subthreshold laser grid photocoagulation therapy, whereas the threshold photocoagulation group underwent short-pulse grid photocoagulation with a 532-nm PASCAL system. BCVA and CMT were assessed in all patients before treatment, 7 days after treatment, and 1, 3, and 6 months after treatment. Results After grid photocoagulation, the mean BCVA improved in both the subthreshold group, and the threshold group, and the two groups did not differ statistically significantly from each other. Similarly, the macular oedema diminished in both groups after treatment, and the two groups did not differ statistically significantly from each other with regard to CMT. Conclusion Both 532-nm subthreshold laser grid photocoagulation and threshold laser grid photocoagulation can improve the visual acuity and reduce CMT in DME patients. PMID:25697457

  13. Vestibular impairment in Charcot-Marie-Tooth disease type 4C.

    PubMed

    Pérez-Garrigues, Herminio; Sivera, Rafael; Vílchez, Juan Jesús; Espinós, Carmen; Palau, Francesc; Sevilla, Teresa

    2014-07-01

    Charcot-Marie-Tooth disease type 4C (CMT4C) is a hereditary neuropathy with prominent unsteadiness. The objective of the current study is to determine whether the imbalance in CMT4C is caused only by reduced proprioceptive input or if vestibular nerve involvement is an additional factor. We selected 10 CMT4C patients and 10 age-matched and sex-matched controls. We performed a comprehensive evaluation of the vestibular system, including video Head Impulse Test, bithermal caloric test, galvanic stimulation test and skull vibration-induced nystagmus test. None of the patients experienced dizziness, spontaneous or gaze-evoked nystagmus, but all had significant vestibular impairment when tested when compared to controls. Seven had completely unexcitable vestibular systems and abnormal vestibuloocular reflex. There was no correlation between the degree of vestibulopathy and age or clinical severity. Significant vestibular impairment is a consistent finding in CMT4C and is present early in disease evolution. The profound imbalance that is so disabling in these patients may result from a combination of proprioceptive loss and vestibular neuropathy, and this would modify the recommended rehabilitation strategies. PMID:24614092

  14. Charcot-Marie-Tooth disease

    PubMed Central

    Manganelli, Fiore; Nolano, Maria; Pisciotta, Chiara; Provitera, Vincenzo; Fabrizi, Gian M.; Cavallaro, Tiziana; Stancanelli, Annamaria; Caporaso, Giuseppe; Shy, Michael E.

    2015-01-01

    Objective: To evaluate, by skin biopsy, dermal nerve fibers in 31 patients with 3 common Charcot-Marie-Tooth (CMT) genotypes (CMT1A, late-onset CMT1B, and CMTX1), and rarer forms of CMT caused by mutations in RAB7 (CMT2B), TRPV4 (CMT2C), and GDAP1 (AR-CMT2K) genes. Methods: We investigated axonal loss by quantifying Meissner corpuscles and intrapapillary myelinated endings and evaluated morphometric changes in myelinated dermal nerve fibers by measuring fiber caliber, internodal, and nodal gap length. Results: The density of both Meissner corpuscles and intrapapillary myelinated endings was reduced in skin samples from patients with CMT1A and all the other CMT genotypes. Nodal gaps were larger in all the CMT genotypes though widening was greater in CMT1A. Perhaps an altered communication between axons and glia may be a common feature for multiple forms of CMT. Internodal lengths were shorter in all the CMT genotypes, and patients with CMT1A had the shortest internodes of all our patients. The uniformly shortened internodes in all the CMT genotypes suggest that mutations in both myelin and axon genes may developmentally impede internode formation. The extent of internodal shortening and nodal gap widening are likely both important in determining nerve conduction velocities in CMT. Conclusions: This study extends the information gained from skin biopsies on morphologic abnormalities in various forms of CMT and provides insights into potential pathomechanisms of axonal and demyelinating CMT. PMID:26362287

  15. Distinguishing Systemic from Systematic.

    ERIC Educational Resources Information Center

    Carr, Alison A.

    1996-01-01

    Describes the difference between systemic and systematic as they relate to school reform and instructional design. Highlights include a history of systems theory; systems engineering; instructional systems design; systemic versus reductionist thinking; social systems; and systemic change in education, including power relationships. (LRW)

  16. 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.

  17. Systems view of power systems autonomy

    SciTech Connect

    Anderson, J.L.

    1984-08-01

    A space station will involve the formation and sustained operation of an assembly of humans and machines in space for a period of 10-20 years. Technology and mission studies of a permanently manned, evolutionary space station have identified the need for automated and eventually some degree of autonomous systems operation. A space station power system will have a high degree of interaction with other onboard systems which will act as power loads. By examining the evolution of an operational power system from a systems viewpoint through increasing degrees of automation the system and technology requirements are identified for an evolutionary system.

  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. Systems design of long-life systems

    NASA Technical Reports Server (NTRS)

    Miles, R. F., Jr.

    1974-01-01

    A long-life system is defined as a system which cannot be life-tested in its operational environment. Another restriction is that preventive maintenance and repair shall be either impossible or economically disadvantageous. Examples of such systems include planetary spacecraft, communication satellites, undersea telephone cables, and nuclear power plants. The questions discussed are related to the implementation of system functions, approaches to determine the required level of system reliability, and aspects of tradeoffs between requirements and reliability.

  20. Shaker-Related Potassium Channels in the Central Medial Nucleus of the Thalamus Are Important Molecular Targets for Arousal Suppression by Volatile General Anesthetics

    PubMed Central

    Birch, Alexandra M.; Tanaka, Brian S.; Sokolov, Yuri; Goldin, Alan L.; Chandy, K. George; Hall, James E.; Alkire, Michael T.

    2013-01-01

    The molecular targets and neural circuits that underlie general anesthesia are not fully elucidated. Here, we directly demonstrate that Kv1-family (Shaker-related) delayed rectifier K+ channels in the central medial thalamic nucleus (CMT) are important targets for volatile anesthetics. The modulation of Kv1 channels by volatiles is network specific as microinfusion of ShK, a potent inhibitor of Kv1.1, Kv1.3, and Kv1.6 channels, into the CMT awakened sevoflurane-anesthetized rodents. In heterologous expression systems, sevoflurane, isoflurane, and desflurane at subsurgical concentrations potentiated delayed rectifier Kv1 channels at low depolarizing potentials. In mouse thalamic brain slices, sevoflurane inhibited firing frequency and delayed the onset of action potentials in CMT neurons, and ShK-186, a Kv1.3-selective inhibitor, prevented these effects. Our findings demonstrate the exquisite sensitivity of delayed rectifier Kv1 channels to modulation by volatile anesthetics and highlight an arousal suppressing role of Kv1 channels in CMT neurons during the process of anesthesia. PMID:24107962

  1. 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

  2. Replacing the acetyl linkage in aspirin with choline and magnesium moieties reduces the occurrence of gastric mucosal injury.

    PubMed

    Danesh, B J; Nelson, L M; Russell, R I; Docherty, C

    1987-02-01

    The acetyl moiety in aspirin (acetyl salicylic acid: ASA) is considered to play a major part in the pathogenesis of ASA-induced mucosal injury. At equivalent salicylate doses and pH values, the induction of acute gastric mucosal haemorrhagic erosions in rats by ASA and choline magnesium trisalicylate (CMT), a new non-acetylated salicylate, with and without the potentiating damaging effect of taurodeoxycholic acid (TDCA) were compared. Test solutions were administered by per oral intubation to five groups of fasting Sprague-Dawley rats (n = 24). Gastric mucosa were examined after 4 hours and mucosal injury assessed by a lesion-scoring system. The incidence and severity (median lesion scores with quartiles) of the lesions were 83% and 13 (7:20) respectively for ASA (128 mg kg-1) compared with 17% and 0 (0:0) for CMT (128 mg kg-1) (P less than 0.001 and P less than 0.001). TDCA increased mucosal damage to 100% and 29 (20:34) for ASA compared with 30% and 0 (0:4) for CMT (P less than 0.001) and P less than 0.001). Serum salicylate levels (median values of 1.4 for ASA and 1.5 mmol litre-1 for CMT) were not significantly different. It is concluded that replacing the acetyl moiety in ASA with choline and magnesium moieties reduces the ASA-induced mucosal injury, without affecting blood salicylate concentrations. PMID:2979212

  3. 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. PMID:16019110

  4. 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. PMID:26786738

  5. 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. PMID:26599234

  6. Female Reproductive System

    MedlinePlus

    ... How Can I Help a Friend Who Cuts? Female Reproductive System KidsHealth > For Teens > Female Reproductive System Print A ... and female reproductive systems. continue What Is the Female Reproductive System? Most species have two sexes: male and female. ...

  7. Multiple System Atrophy

    MedlinePlus

    ... Enhancing Diversity Find People About NINDS NINDS Multiple System Atrophy Information Page Condensed from Multiple System Atrophy ... Trials Organizations Publicaciones en Español What is Multiple System Atrophy? Multiple system atrophy (MSA) is a progressive ...

  8. 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.

  9. 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.

  10. 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. PMID:27177999