A rare cause of childhood renal cysts: Xp11.2 translocation renal cell carcinoma.

PubMed

Taşkınlar, Hakan; Avlan, Dinçer; Çıtak, Çağlar; Polat, Ayşe; Naycı, Ali

2015-01-01

Pediatric renal cysts are rare, usually asymptomatic and incidentally detected in children. Cyst associated renal cell carcinoma (RCC) or cystic RCC is extremely rare in children. Bosniak classification system has been accepted for the management of cystic renal masses. Xp11.2 translocation RCC is a recently classified distinct subtype and usually affects children and adolescents. We report the case of a 10-year-old girl with Xp11.2 translocation RCC from a cyst of the right kidney.

[Research advances in Xp11.2 translocation renal cell carcinoma].

PubMed

Huang, Jian-Hua; Zhou, Fang-Jian

2008-09-01

Xp11.2 translocation renal cell carcinoma (RCC) is a newly identified category of RCC described in the 2004 WHO Classification of Kidney Tumors. Although the incidence is very rare, it accounts about one third of pediatric RCCs. It is different from other RCCs in clinical manifestations, histopathologic features, biological behaviour and prognosis. At present, Xp11.2 translocation RCC has seldom been reported. This review analyzed recent researches on Xp11.2 translocation RCC, described its classification and summarized the characteristics of epidemiology, clinical manifestations, histopathology, diagnosis, treatment and prognosis.

Evaluation Metrics for the Paragon XP/S-15

NASA Technical Reports Server (NTRS)

Traversat, Bernard; McNab, David; Nitzberg, Bill; Fineberg, Sam; Blaylock, Bruce T. (Technical Monitor)

1993-01-01

On February 17th 1993, the Numerical Aerodynamic Simulation (NAS) facility located at the NASA Ames Research Center installed a 224 node Intel Paragon XP/S-15 system. After its installation, the Paragon was found to be in a very immature state and was unable to support a NAS users' workload, composed of a wide range of development and production activities. As a first step towards addressing this problem, we implemented a set of metrics to objectively monitor the system as operating system and hardware upgrades were installed. The metrics were designed to measure four aspects of the system that we consider essential to support our workload: availability, utilization, functionality, and performance. This report presents the metrics collected from February 1993 to August 1993. Since its installation, the Paragon availability has improved from a low of 15% uptime to a high of 80%, while its utilization has remained low. Functionality and performance have improved from merely running one of the NAS Parallel Benchmarks to running all of them faster (between 1 and 2 times) than on the iPSC/860. In spite of the progress accomplished, fundamental limitations of the Paragon operating system are restricting the Paragon from supporting the NAS workload. The maximum operating system message passing (NORMA IPC) bandwidth was measured at 11 Mbytes/s, well below the peak hardware bandwidth (175 Mbytes/s), limiting overall virtual memory and Unix services (i.e. Disk and HiPPI I/O) performance. The high NX application message passing latency (184 microns), three times than on the iPSC/860, was found to significantly degrade performance of applications relying on small message sizes. The amount of memory available for an application was found to be approximately 10 Mbytes per node, indicating that the OS is taking more space than anticipated (6 Mbytes per node).

Xp11.2 translocation renal carcinoma with placental metastasis: a case report.

PubMed

Bovio, Ian M; Allan, Robert W; Oliai, Bahram R; Hampton, Troy; Rush, Demaretta S

2011-02-01

Renal cell carcinomas with sporadic Xp11.2 translocations are uncommon malignancies in children and young adults associated with several different reciprocal translocations involving the TFE3 gene located on chromosome Xp11.2. Placental metastases are extremely rare, with only a handful of cases reported. This study reports the case of a 20-year-old woman with an Xp11.2 translocation renal carcinoma that metastasized to the placenta. This is the first reported case of a renal cell carcinoma metastatic to the placenta and highlights the aggressive behavior of Xp11 translocation renal cell carcinomas.

A rare cause of childhood renal cysts: Xp11.2 translocation renal cell carcinoma

PubMed Central

Taşkınlar, Hakan; Avlan, Dinçer; Çıtak, Çağlar; Polat, Ayşe; Naycı, Ali

2015-01-01

Pediatric renal cysts are rare, usually asymptomatic and incidentally detected in children. Cyst associated renal cell carcinoma (RCC) or cystic RCC is extremely rare in children. Bosniak classification system has been accepted for the management of cystic renal masses. Xp11.2 translocation RCC is a recently classified distinct subtype and usually affects children and adolescents. We report the case of a 10-year-old girl with Xp11.2 translocation RCC from a cyst of the right kidney. PMID:25624966

Chance, destiny, and the inner workings of ClpXP.

PubMed

Russell, Rick; Matouschek, Andreas

2014-07-31

AAA+ proteases are responsible for protein degradation in all branches of life. Using single-molecule and ensemble assays, Cordova et al. investigate how the bacterial protease ClpXP steps through a substrate's polypeptide chain and construct a quantitative kinetic model that recapitulates the interplay between stochastic and deterministic behaviors of ClpXP. Copyright © 2014 Elsevier Inc. All rights reserved.

TFE3-Fusion Variant Analysis Defines Specific Clinicopathologic Associations Among Xp11 Translocation Cancers

PubMed Central

Argani, Pedram; Zhong, Minghao; Reuter, Victor E.; Fallon, John T.; Epstein, Jonathan I.; Netto, George J.; Antonescu, Cristina R.

2016-01-01

Xp11 translocation cancers include Xp11 translocation renal cell carcinoma (RCC), Xp11 translocation perivascular epithelioid cell tumor (PEComa), and melanotic Xp11 translocation renal cancer. In Xp11 translocation cancers, oncogenic activation of TFE3 is driven by the fusion of TFE3 with a number of different gene partners, however, the impact of individual fusion variant on specific clinicopathologic features of Xp11 translocation cancers has not been well defined. In this study, we analyze 60 Xp11 translocation cancers by fluorescence in situ hybridization (FISH) using custom BAC probes to establish their TFE3 fusion gene partner. In 5 cases RNA sequencing (RNA-seq) was also used to further characterize the fusion transcripts. The 60 Xp11 translocation cancers included 47 Xp11 translocation RCC, 8 Xp11 translocation PEComas, and 5 melanotic Xp11 translocation renal cancers. A fusion partner was identified in 53/60 (88%) cases, including 18 SFPQ (PSF), 16 PRCC, 12 ASPSCR1 (ASPL), 6 NONO, and 1 DVL2. We provide the first morphologic description of the NONO-TFE3 RCC, which frequently demonstrates sub-nuclear vacuoles leading to distinctive suprabasal nuclear palisading. Similar sub-nuclear vacuolization was also characteristic of SFPQ-TFE3 RCC, creating overlapping features with clear cell papillary RCC. We also describe the first RCC with a DVL2-TFE3 gene fusion, in addition to an extrarenal pigmented PEComa with a NONO-TFE3 gene fusion. Furthermore, among neoplasms with the SFPQ-TFE3, NONO-TFE3, DVL2-TFE3 and ASPL-TFE3 gene fusions, the RCC are almost always PAX8-positive, cathepsin K-negative by immunohistochemistry, whereas the mesenchymal counterparts (Xp11 translocation PEComas, melanotic Xp11 translocation renal cancers, and alveolar soft part sarcoma) are PAX8-negative, cathepsin K-positive. These findings support the concept that despite an identical gene fusion, the RCCs are distinct from the corresponding mesenchymal neoplasms, perhaps due to the

Xp11.2 translocation renal cell carcinoma with PSF-TFE3 rearrangement.

PubMed

Zhong, Minghao; Weisman, Paul; Zhu, Bing; Brassesco, Maria; Yang, Youfeng; Linehan, W Marston; Merino, Maria J; Zhang, David; Rohan, Stephen; Cai, Dongming; Yang, Ximing

2013-06-01

Xp11.2 translocation renal cell carcinoma (Xp11.2 RCC) is a subtype of RCC characterized by translocations involving a breakpoint at the TFE3 gene (Xp11.2). Moderate to strong nuclear TFE3 immunoreactivity has been recognized as a specific diagnostic marker for this type of tumor. However, exclusive cytoplasmic localization of a TFE3 fusion protein was reported in UOK 145 cells, a cell line derived from an Xp11.2 RCC harboring the PSF-TFE3 translocation. If reproducible using immunohistochemistry (IHC), this finding would have important implications for pathologists in the diagnosis of Xp11.2 RCC, calling into question the specificity of nuclear immunoreactivity for TFE3 in these tumors. The purpose of this study was to determine whether the above-noted cytoplasmic localization of the TFE3 fusion protein could be reproduced using IHC. UOK 145 cells and fresh frozen tissue from 2 clinical cases of Xp11.2 RCC found to harbor the PSF-TFE3 gene rearrangement (by cytogenetic testing) were collected. All samples were subjected to histopathologic evaluation by board-certified pathologists, TFE3 IHC, reverse transcription polymerase chain reaction, and Sanger sequencing analysis. A strong nuclear TFE3 immunoreactivity was demonstrated in all samples including the UOK 145 cell line. No cytoplasmic immunoreactivity was seen. Reverse transcription polymerase chain reaction and Sanger sequencing confirmed the previously reported PSF-TFE3 gene fusion between exon 9 of PSF and exon 6 of TFE3 in the UOK 145 cell line and in one of 2 clinical cases of Xp11.2 RCC. A novel PSF-TFE3 gene fusion between exon 9 of PSF and exon 5 of TFE3 was detected in the second clinical case of Xp11.2 RCC.

[A case of xp11.2 translocation renal cell carcinoma].

PubMed

Horie, Kengo; Kikuchi, Mina; Miwa, Kosei; Minamidate, Yuzuru; Yokoi, Shigeaki; Nakano, Masahiro; Deguchi, Takashi; Ehara, Hidetoshi; Asano, Nami; Hirose, Yoshinobu

2011-03-01

Xp11.2/TFE3 translocation renal cell carcinoma (RCC), a recently classified distinct subtype, is a rare tumor that usually affects children and adolescents. The morphology and biological behavior are not widely recognized, Xp11.2 translocation RCC is suggestive of early metastases despite the small tumor size. The definitive diagnosis requires the evidence of several different reciprocal translocations involving the TFE3 gene located on chromosome Xp11.2. Here, we present a case of Xp11.2 translocation RCC in an 18-yearold male. He was referred to our hospital because of a right renal tumor with macroscopic hematuria and right flank colic. The radiographic evaluation including magnetic resonance imaging (MRI) suggested it to be a typical papillary renal cell carcinoma or benign renal tumor. He underwent laparoscopic nephrectomy against the repeat symptom in spite of small tumor (3.5 cm in diameter). The immunohistochemical study revealed nuclear staining for TFE3 protein in the cancer cells. The urologic and radiologic outcomes were satisfactory after more than 1 year of follow-up.

Modeling of a lot scale rainwater tank system in XP-SWMM: a case study in Western Sydney, Australia.

PubMed

van der Sterren, Marlène; Rahman, Ataur; Ryan, Garry

2014-08-01

Lot scale rainwater tank system modeling is often used in sustainable urban storm water management, particularly to estimate the reduction in the storm water run-off and pollutant wash-off at the lot scale. These rainwater tank models often cannot be adequately calibrated and validated due to limited availability of observed rainwater tank quantity and quality data. This paper presents calibration and validation of a lot scale rainwater tank system model using XP-SWMM utilizing data collected from two rainwater tank systems located in Western Sydney, Australia. The modeling considers run-off peak and volume in and out of the rainwater tank system and also a number of water quality parameters (Total Phosphorus (TP), Total Nitrogen (TN) and Total Solids (TS)). It has been found that XP-SWMM can be used successfully to develop a lot scale rainwater system model within an acceptable error margin. It has been shown that TP and TS can be predicted more accurately than TN using the developed model. In addition, it was found that a significant reduction in storm water run-off discharge can be achieved as a result of the rainwater tank up to about one year average recurrence interval rainfall event. The model parameter set assembled in this study can be used for developing lot scale rainwater tank system models at other locations in the Western Sydney region and in other parts of Australia with necessary adjustments for the local site characteristics. Copyright © 2014 Elsevier Ltd. All rights reserved.

A family with X-linked optic atrophy linked to the OPA2 locus Xp11.4-Xp11.2.

PubMed

Katz, Bradley J; Zhao, Yu; Warner, Judith E A; Tong, Zongzhong; Yang, Zhenglin; Zhang, Kang

2006-10-15

Autosomal dominant optic atrophy (ADOA) is the most common inherited optic atrophy. Clinical features of ADOA include a slowly progressive bilateral loss of visual acuity, constriction of peripheral visual fields, central scotomas, and color vision abnormalities. Although ADOA is the most commonly inherited optic atrophy, autosomal recessive, X-linked, mitochondrial, and sporadic forms have also been reported. Four families with X-linked optic atrophy (XLOA) were previously described. One family was subsequently linked to Xp11.4-Xp11.2 (OPA2). This investigation studied one multi-generation family with an apparently X-linked form of optic atrophy and compared their clinical characteristics with those of the previously described families, and determined whether this family was linked to the same genetic locus. Fifteen individuals in a three-generation Idaho family underwent complete eye examination, color vision testing, automated perimetry, and fundus photography. Polymorphic markers were used to genotype each individual and to determine linkage. Visual acuities ranged from 20/30 to 20/100. All affected subjects had significant optic nerve pallor. Obligate female carriers were clinically unaffected. Preliminary linkage analysis (LOD score = 1.8) revealed that the disease gene localized to the OPA2 locus on Xp11.4-Xp11.2. Four forms of inherited optic neuropathy, ADOA, autosomal recessive optic atrophy (Costeff Syndrome), Leber hereditary optic neuropathy, and Charcot-Marie-Tooth disease with optic atrophy, are associated with mitochondrial dysfunction. Future identification of the XLOA gene will reveal whether this form of optic atrophy is also associated with a mitochondrial defect. Identification of the XLOA gene will advance our understanding of the inherited optic neuropathies and perhaps suggest treatments for these diseases. An improved understanding of inherited optic neuropathies may in turn advance our understanding of acquired optic nerve diseases, such

Magnetic properties of x(Fe2O3).(100-x)[P2O5.Li2O] and x(Fe2O3).(100-x)[P2O5.CaO] glass systems

NASA Astrophysics Data System (ADS)

Andronache, Constantin; Racolta, Dania; Ardelean, Gheorghe

2017-12-01

Magnetic properties of x(Fe2O3).(100-x)[P2O5 .Li2O] and x(Fe2O3).(100-x)[P2O5 .CaO] with 0 < x ≤ 50 mol % were investigated using magnetic susceptibility measurements. The both glass systems were prepared in the same condition. The valence states and the distribution of iron ions in the glass matrix depend on the Fe2O3 content. For the P2O5.CaO glass matrix with x≤35mol%, the data revealed iron ions as isolated or participating in dipole-dipole interaction. For x > 35 mol% an antiferromagnetic coupling is observed. For the P2O5.Li2O glass matrix, the iron ions behave magnetically similarly as in other oxide glasses, but concentration of Fe2O3 over which magnetic superexchange interactions occur is lower. The absolute magnitude of θp values increases when content of Fe2O3 are increased. If the content of the magnetic ions is increased in the glass, the exchange integral increased and as a result the magnitude of the θP increases.

Effect of ProTaper Gold, Self-Adjusting File, and XP-endo Shaper Instruments on Dentinal Microcrack Formation: A Micro-computed Tomographic Study.

PubMed

Bayram, H Melike; Bayram, Emre; Ocak, Mert; Uygun, Ahmet Demirhan; Celik, Hakan Hamdi

2017-07-01

The aim of the present study was to evaluate the frequency of dentinal microcracks observed after root canal preparation with ProTaper Universal (PTU; Dentsply Tulsa Dental Specialties, Tulsa, OK), ProTaper Gold (PTG; Dentsply Tulsa Dental Specialties), Self-Adjusting File (SAF; ReDent Nova, Ra'anana, Israel), and XP-endo Shaper (XP; FKG Dentaire, La Chaux-de-Fonds, Switzerland) instruments using micro-computed tomographic (CT) analysis. Forty extracted human mandibular premolars having single-canal and straight root were randomly assigned to 4 experimental groups (n = 10) according to the different nickel-titanium systems used for root canal preparation: PTU, PTG, SAF, and XP. In the SAF and XP groups, the canals were first prepared with a K-file until #25 at the working length, and then the SAF or XP files were used. The specimens were scanned using high-resolution micro-computed tomographic imaging before and after root canal preparation. Afterward, preoperative and postoperative cross-sectional images of the teeth were screened to identify the presence of dentinal defects. For each group, the number of microcracks was determined as a percentage rate. The McNemar test was used to determine significant differences before and after instrumentation. The level of significance was set at P ≤ .05. The PTU system significantly increased the percentage rate of microcracks compared with preoperative specimens (P < .05). No new dentinal microcracks were observed in the PTG, SAF, or XP groups. Root canal preparations with the PTG, SAF, and XP systems did not induce the formation of new dentinal microcracks on straight root canals of mandibular premolars. Copyright © 2017 American Association of Endodontists. Published by Elsevier Inc. All rights reserved.

Xp11.2 Translocation Renal Cell Carcinoma Diagnosed by Immunohistochemistry and Cytogenetics.

PubMed

Dey, Biswajit; Badhe, Bhawana; Govindarajan, Krishna Kumar; Ramesh, Ranjith Arumbakkam

2016-01-01

Xp11.2 translocation renal cell carcinomas (TRCCs) are a group of neoplasms with distinct clinical, histopathological appearance, immunohistochemical, and cytogenetic profile. We report a case of Xp11.2 translocation TRCC in an 11-year-old male diagnosed based on immunohistochemistry and fluorescence in situ hybridization.

Xp11.2 Translocation Renal Cell Carcinoma Diagnosed by Immunohistochemistry and Cytogenetics

PubMed Central

Dey, Biswajit; Badhe, Bhawana; Govindarajan, Krishna Kumar; Ramesh, Ranjith Arumbakkam

2016-01-01

Xp11.2 translocation renal cell carcinomas (TRCCs) are a group of neoplasms with distinct clinical, histopathological appearance, immunohistochemical, and cytogenetic profile. We report a case of Xp11.2 translocation TRCC in an 11-year-old male diagnosed based on immunohistochemistry and fluorescence in situ hybridization. PMID:27365924

Dynamic Computed Tomographic Features of Adult Renal Cell Carcinoma Associated With Xp11.2 Translocation/TFE3 Gene Fusions: Comparison With Clear Cell Renal Cell Carcinoma.

PubMed

He, Jian; Gan, Weidong; Liu, Song; Zhou, Kefeng; Zhang, Gutian; Guo, Hongqian; Zhu, Bin

2015-01-01

To investigate the dynamic contrast-enhanced computed tomography (CT) characteristics of renal cell carcinoma associated with Xp11.2 translocation and TFE gene fusion (Xp11.2 RCC) by comparison with clear cell renal cell carcinoma (CCRCC). Dynamic contrast-enhanced CT images and clinical and pathological records of 20 adult patients with Xp11.2 RCC confirmed by TFE3 immunohistochemical and fluorescence in situ hybridization assay were retrospectively analyzed and compared with the findings of 21 contemporary CCRCCs. Renal cell carcinoma associated with Xp11.2 translocation and TFE gene fusions often occurred in young (30.6 ± 8.6 years) patients with hematuria (9/20). They presented as well-defined (17/20) cystic-solid (17/20) mass with hemorrhage (8/20) and circular/rim calcifications (6/20). Dynamic contrast-enhanced CT showed heterogeneous moderate prolonged enhancement. A tumor-to-cortex attenuation ratio in corticomedullary phase less than 0.62 gave a sensitivity of 90.0% and a specificity of 92.9% in differentiating Xp11.2 RCC from CCRCC (area under the receiver operating characteristic curve = 0.957, P < 0.001). Computed tomographic characteristics and dynamic contrast-enhanced patterns and index can differentiate Xp11.2 RCC from CCRCC.

Validation of a TFE3 break-apart FISH assay for Xp11.2 translocation renal cell carcinomas.

PubMed

Mosquera, Juan-Miguel; Dal Cin, Paola; Mertz, Kirsten D; Perner, Sven; Davis, Ian J; Fisher, David E; Rubin, Mark A; Hirsch, Michelle S

2011-09-01

Renal cell carcinomas (RCCs) with an Xp11.2 translocation predominantly affect young patients, and can present at an advanced stage. However, more cases in older patients and incidentally detected cancers at earlier stages are also being identified. As the histology of Xp11.2 RCCs overlaps with clear cell and papillary RCCs, it is not infrequent that Xp11.2 RCCs are overlooked and misdiagnosed. The objective of this study was to validate the use of fluorescence in-situ hybridization (FISH) for identifying Xp11.2 RCCs. One hundred fifty-eight consecutive, unselected renal tumors were evaluated in tissue microarrays, including 109 clear cell RCCs, 20 papillary RCCs, 3 RCCs with mixed papillary and clear cell features, 1 Xp11.2 translocation RCC, 8 chromophobe RCCs, 10 oncocytomas, and 7 angiomyolipomas. FISH evaluation was performed blinded to karyotype data, available in about two-thirds of cases. Furthermore, conventional sections of 4 Xp11.2 RCCs, 4 RCCs with mixed papillary and clear cell features, and 4 cases of alveolar soft part sarcoma (the latter for control purposes) were also assessed by FISH. Break-apart signals were homogeneously identified throughout tumor cells in 2 cases from the tissue microarrays including 1 known Xp11.2 RCC and 1 misdiagnosed Xp11.2 RCC. All conventional sections from the Xp11.2 RCC and alveolar soft part sarcoma cases were positive for the TFE3 rearrangement by FISH. All remaining cases were negative. Our study shows the clinical application of FISH in formalin-fixed, paraffin-embedded tissue for detection of Xp11.2 translocation RCCs and other tumors with this genetic aberration.

Cyclic and Torsional Fatigue Resistance of XP-endo Shaper and TRUShape Instruments.

PubMed

Silva, Emmanuel João Nogueira Leal; Vieira, Victor Talarico Leal; Belladonna, Felipe Gonçalves; Zuolo, Arthur de Siqueira; Antunes, Henrique Dos Santos; Cavalcante, Daniele Moreira; Elias, Carlos Nelson; De-Deus, Gustavo

2018-01-01

The purpose of this study was to evaluate the cyclic and torsional fatigue resistance of the XP-endo Shaper (FKG Dentaire, La Chaux-de-Fonds, Switzerland) and TRUShape (Dentsply Tulsa Dental Specialties, Tulsa, OK) instruments. Twenty XP-endo Shaper (30/0.01) instruments and 20 TRUShape (30/0.06v) instruments were used. Cyclic fatigue resistance was tested by measuring the number of cycles and time to fracture in an artificial stainless steel canal with a 60° angle and a 5-mm radius of curvature (n = 10). The torque and angle of rotation at failure of new instruments (n = 10) were measured according to ISO 3630-1. The fracture surface of all fragments was examined with a scanning electron microscope. Results were statistically analyzed using the Student t test at a significance level of P < .05. The XP-endo Shaper instruments showed a significantly longer number of cycles to fracture and time to failure in seconds than the TRUShape instruments (P < .05). The XP-endo Shaper also presented a lower maximum torque load (P < .05) but a significantly higher angular rotation to fracture than TRUShape (P < .05). The XP-endo Shaper instruments showed a higher cyclic fatigue resistance and angle of rotation to fracture but lower torque to failure than TRUShape instruments. Copyright © 2017 American Association of Endodontists. Published by Elsevier Inc. All rights reserved.

Renal cell carcinoma associated with Xp11.2 translocations, report of a case.

PubMed

Jing, Hongbiao; Tai, Yanhong; Xu, Dazhou; Yang, Fan; Geng, Ming

2010-07-01

Renal cell carcinomas (RCCs) associated with Xp11.2 translocations (Xp11.2 translocation RCCs) are rare and occur predominantly in children and adolescents. A case of such tumor in a 12-year boy is reported. Grossly the cut surface of the ill-defined mass was polychromatic, containing areas of hemorrhage and necrosis. Microscopically, the tumor was composed of epithelioid cells with clear to weakly eosinophilic cytoplasm arranged in nested, alveolar, and pseudopapillary formations. Immunohistochemically, the neoplastic cells were positive for transcription factor E3 and CD10. We concluded that this case was an Xp11.2 translocation RCC. Copyright 2010 Elsevier Inc. All rights reserved.

XP11.2 translocation renal cell carcinoma: clinical experience of Taipei Veterans General Hospital.

PubMed

Hung, Chia-Chen; Pan, Chin-Chen; Lin, Chih-Chieh; Lin, Alex T L; Chen, Kuang-Kuo; Chang, Yen-Hwa

2011-11-01

Xp11.2 translocation renal cell carcinoma (RCC), a recently recognized distinct subtype of RCC, is characterized by various translocations, all involving the TFE3 transcription factor gene. These rare cancers occur predominantly in children and young adults and comprise about one-third of pediatric RCCs. In the present study, we review the clinical course of Xp11.2 translocation renal cell carcinoma in our institution. We identified eight cases with Xp11.2 translocation RCC between 2007 and 2010 from the pathological archives of the Taipei Veterans General Hospital. We retrospectively analyzed the patients' characteristics, clinical manifestations, and specific pathological features for definitive diagnosis, surgical and systemic treatment and clinical outcome of these rare cancers. Patients were aged 20 years to 49 years (mean age 28 years) with female predominance (6 females, 2 males). One patient presented with asymptomatic renal mass detected incidentally during abdominal sonography. Four patients complained of flank or abdominal pain, and the other three complained of gross hematuria at initial presentation. The mean tumor size was 9.2 cm (range, 4 cm-17 cm). Seven patients underwent radical nephrectomy for the primary tumor, while one presented with multiple metastases. All cases were confirmed by TFE3 immunohistochemistry, a sensitive and specific marker of tumors with TFE3 gene fusion, which showed positive nuclear staining. Three patients presented initially with metastatic diseases, and another three patients progressed to lung, liver and bone metastases at eight, seven and nine months postoperatively. Although RT-PCR and DNA sequencing are the final diagnoses of the molecular identity of Xp11.2 translocation RCC, experienced pathologists could confirm the histologic diagnosis based on the distinctive morphologic features with positive TFE3 immunochemical nuclear stain. Surgical resection is the only treatment. The role of systemic therapy for local

Clinicopathologic Characteristics and Prognosis of Xp11.2 Translocation Renal Cell Carcinoma: Multicenter, Propensity Score Matching Analysis.

PubMed

Choo, Min Soo; Jeong, Chang Wook; Song, Cheryn; Jeon, Hwang Gyun; Seo, Seong Il; Hong, Sung Kyu; Byun, Seok-Soo; Chung, Jin Soo; Hong, Sung-Hoo; Hwang, Eu Chang; Kim, Hyeon Hoe; Kwak, Cheol

2017-10-01

We evaluated the clinicopathologic characteristics and prognosis of Xp11.2 translocation (Xp11.2t) renal cell carcinoma (RCC) from a multicenter study and compare them with clear-cell RCC using a propensity score matching analysis. Between 2004 and 2013, 8384 consecutive patients from 7 institutions who were diagnosed with RCC were reviewed, and the pathologically confirmed Xp11.2t cases were enrolled. The oncological outcomes of Xp11.2t were compared with those of clear-cell RCC by selecting matched cases using 1:3 propensity score matching methods in a precollected clear-cell RCC data set from our hospital. The patients were divided into 2 subgroups on the basis of age of onset, either before (early) or after (late) 45 years old. Xp11.2t was found in 61 cases, corresponding to 0.72% of RCC cases for the 10 years. The mean age was 38.2 ± 19.4 years, and the mean tumor size was 6.2 ± 3.9 cm. The Xp11.2t cases were at more advanced stages and showed tendencies to involve lymph nodes at diagnosis. After the matching, there were no significant differences in recurrence-free and overall survival compared with clear-cell RCC. The age of incidence for Xp11.2t had a bimodal distribution, which was most common in the 30s and smaller peak in the 60s. Xp11.2t corresponded to a significantly worse prognosis for overall survival in late onset (after 45 years) subgroup (P = .038; hazard ratio, 3.199; 95% confidence interval, 1.065-9.609). This neoplasm has more aggressive clinicopathologic features at diagnosis. In older patients with onset age > 45 years, Xp11.2t showed a significantly worse prognosis than clear-cell RCC. Copyright © 2017 Elsevier Inc. All rights reserved.

Perivascular epithelioid cell tumor (PEComa) of the urinary bladder associated with Xp11 translocation.

PubMed

Russell, Christopher M; Buethe, David D; Dickinson, Shohreh; Sexton, Wade J

2014-01-01

Perivascular epithelioid cell-containing tumors (PEComas) represent a rare family of neoplasms. Their dichotomous phenotypic features, including both myogenic and mylanocytic features, can make a definitive diagnosis difficult. Such tumors have been associated with the overexpression of transcription factor E3 (TFE3). An Xp11 translocation could account for the aberrant activity of TFE3 but has never before been described in affiliation with a PEComa of the urinary bladder. While PEComas of the bladder have exhibited benign clinical courses to date, here we present an intravesical PEComa shown to have an Xp11 translocation and resultant overexpression of TFE3, indicating an aggressive, metastatic nature. No consistent tumor characteristics have proven accurate at identifying aggressive tumors. However, mTOR inhibitors offer a mechanistic management strategy when systemic therapy is warranted.

Dynamic Contrast-Enhanced CT Characterization of Xp11.2 Translocation/TFE3 Gene Fusions versus Papillary Renal Cell Carcinomas.

PubMed

He, Jian; Zhou, Kefeng; Zhu, Bin; Zhang, Gutian; Li, Xiaogong; Guo, Hongqian; Gan, Weidong; Zhou, Zhengyang; Liu, Tian

2015-01-01

To compare the differences of CT characteristics between renal cell carcinomas (RCCs) associated with Xp11.2 translocation/TFE3 gene fusions (Xp11.2 RCCs) and papillary cell renal cell carcinomas (PRCCs). CT images and clinical records of 64 patients (25 Xp11.2 RCCs, 15 type 1 and 24 type 2 PRCCs) were analyzed and compared retrospectively. Xp11.2 RCC more frequently affected young (30.7 ± 8.7 years) women (16/25, 64%) with gross hematuria (12/25, 48%), while PRCC more frequently involved middle-aged (54.8 ± 11.1 years) men (28/39, 71.8%) asymptomatically. Xp11.2 RCC tended to be heterogeneous density with some showing circular calcification. Lesion sizes of Xp11.2 RCC (5.4 ± 2.2 cm) and type 2 PRCC (5.7 ± 2.5 cm) were significantly larger than that of type 1 PRCC (3.8 ± 1.8 cm). Xp11.2 RCC contained more cystic components (22/25, 88%) than type 1 PRCC (all solid) and type 2 PRCC (9/24, 36.0%). Type 1 PRCC (13/15, 86.7%) and Xp11.2 RCC (21/25, 84.0%) showed more clear boundary than type 2 PRCC (12/24, 50.0%). CT features including diameter, boundary, attenuation, nature, and circular calcification of the tumor, combined with demographic information and symptoms, may be useful to differentiate Xp11.2 RCC from different subtypes of PRCC.

Location interval at Xp22.3 for X-linked chondrodysplasia punctata

DOE Office of Scientific and Technical Information (OSTI.GOV)

Sheffield, L.; Hutchison, W.; Holloway, A.

1994-09-01

The literature shows that there is a gene for chondropdysplasia punctata (CDP) located at Xp22.3 from the study of chromosomal rearrangements involving Xp. It is also suspected that there are genes for short stature and mental retardation nearby. Petit has described a family of brachytelephalangic CDP that was due to a submicroscopic interstitial deletion of Xp22.3. Symmetrical (mild) CDP seems to be identical to brachytelephalangic CDP clinically, has variable features of short stature and mental retardation, and has a preponderance of affected males. We describe results using DNA probes from Xp22.3 in 10 patients with radiologically proven symmetrical CDP. Othermore » known genes in this region have a high proportion of deletions as we screened our patients for deletions in DXYS20, MIC2, PABX, DXYS159X, DXS283, DXS285, J502(PCR), DXS31, DXS43 (listed distally to proximally). No deletions were found. We have also studied a fetus with proven CDP who has a X,Y translocation (46,V,t(X;Y)(p22.3;q12)mat). This patient was deleted for all distal probes up to J502(PCR). It is not yet known where the breakpoint lies but it may be just proximal to the CDP gene. The results of the 10 symmetrical CDPs and the X;Y translocation fetus are presented with further definition of the X chromosomal breakpoint in the translocation.« less

MDCT findings of renal cell carcinoma associated with Xp11.2 translocation and TFE3 gene fusion and papillary renal cell carcinoma.

PubMed

Woo, Sungmin; Kim, Sang Youn; Lee, Myoung Seok; Moon, Kyung Chul; Kim, See Hyung; Cho, Jeong Yeon; Kim, Seung Hyup

2015-03-01

OBJECTIVE. The purpose of this study was to compare the MDCT features of renal cell carcinoma (RCC) associated with Xp11.2 translocation and TFE3 gene fusion (Xp11 RCC) and papillary RCC. MATERIALS AND METHODS. The study included 19 and 39 patients with histologically proven Xp11 RCC and papillary RCC, respectively, who underwent multiphase renal MDCT before nephrectomy. CT findings were compared between Xp11 RCC and papillary RCC using the Student t test and chi-square test. Subgroup analyses of small (< 4 cm) renal masses for these features were performed. RESULTS. Patients with Xp11 RCC were younger (p < 0.001), and it was more prevalent in women (p = 0.007). Tumor size was greater in Xp11 RCC (p = 0.004) and more common in cystic change (p < 0.001). Calcification and unenhanced high-attenuating areas were more frequent in Xp11 RCC (p = 0.001 and 0.026, respectively). Xp11 RCCs were more prevalent in lymph node and distant metastasis (p < 0.001 and p = 0.031, respectively). Xp11 RCC and papillary RCC showed no significant difference in epicenter, margin, and venous and collecting duct invasion (p = 0.403-1.000). Although Xp11 RCC and papillary RCC had lower attenuation than the renal cortex on corticomedullary and early excretory phases (p < 0.001), only Xp11 RCCs were hyperattenuating to the cortex on the unenhanced phase (p < 0.001). Xp11 RCCs had significantly higher attenuation compared with papillary RCCs on all phases (p ≤ 0.02). Regarding small masses, cystic change, calcification, and lymph node metastasis were still more frequent in Xp11 RCCs (p ≤ 0.016). CONCLUSION. Greater size, more cystic change, calcification, high-attenuating areas on unenhanced imaging, and lymph node and distant metastasis were helpful for differentiating Xp11 RCC from papillary RCC.

Dynamic Contrast-Enhanced CT Characterization of Xp11.2 Translocation/TFE3 Gene Fusions versus Papillary Renal Cell Carcinomas

PubMed Central

He, Jian; Zhou, Kefeng; Zhu, Bin; Zhang, Gutian; Li, Xiaogong; Guo, Hongqian; Gan, Weidong; Zhou, Zhengyang; Liu, Tian

2015-01-01

Purpose. To compare the differences of CT characteristics between renal cell carcinomas (RCCs) associated with Xp11.2 translocation/TFE3 gene fusions (Xp11.2 RCCs) and papillary cell renal cell carcinomas (PRCCs). Methods. CT images and clinical records of 64 patients (25 Xp11.2 RCCs, 15 type 1 and 24 type 2 PRCCs) were analyzed and compared retrospectively. Results. Xp11.2 RCC more frequently affected young (30.7 ± 8.7 years) women (16/25, 64%) with gross hematuria (12/25, 48%), while PRCC more frequently involved middle-aged (54.8 ± 11.1 years) men (28/39, 71.8%) asymptomatically. Xp11.2 RCC tended to be heterogeneous density with some showing circular calcification. Lesion sizes of Xp11.2 RCC (5.4 ± 2.2 cm) and type 2 PRCC (5.7 ± 2.5 cm) were significantly larger than that of type 1 PRCC (3.8 ± 1.8 cm). Xp11.2 RCC contained more cystic components (22/25, 88%) than type 1 PRCC (all solid) and type 2 PRCC (9/24, 36.0%). Type 1 PRCC (13/15, 86.7%) and Xp11.2 RCC (21/25, 84.0%) showed more clear boundary than type 2 PRCC (12/24, 50.0%). Conclusion. CT features including diameter, boundary, attenuation, nature, and circular calcification of the tumor, combined with demographic information and symptoms, may be useful to differentiate Xp11.2 RCC from different subtypes of PRCC. PMID:26636097

Xp11.2 translocation renal cell carcinoma with TFE3 gene fusion: A case report

PubMed Central

Pan, Xiang; Quan, Jing; Zhao, Liwen; Li, Wenhua; Wei, Benlin; Yang, Shangqi; Lai, Yongqing

2018-01-01

Xp11.2 translocation renal cell carcinoma (RCC) with transcription factor E3 (TFE3) gene fusion is a rare tumor, and the prognosis of this tumor is poorer compared with that of other subtypes of RCC. The patient presented herein was a 70-year-old man who presented with a solid mass sized ~8.2×6.1 cm in the right kidney and underwent radical right nephrectomy. Following pathological and immunohistochemical (IHC) examination and fluorescent in situ hybridization (FISH), the patient was diagnosed with Xp11.2 translocation RCC with TFE3 gene fusion. These tumors are more commonly encountered in children rather than in adults, and adult Xp11.2 translocation RCC is associated with a poorer prognosis compared with its pediatric counterpart. IHC assay and FISH are important diagnostic methods. However, there is currently no established effective treatment for Xp11.2 RCC. PMID:29399348

Xp11.2 translocation renal cell carcinoma with TFE3 gene fusion: A case report.

PubMed

Pan, Xiang; Quan, Jing; Zhao, Liwen; Li, Wenhua; Wei, Benlin; Yang, Shangqi; Lai, Yongqing

2018-01-01

Xp11.2 translocation renal cell carcinoma (RCC) with transcription factor E3 (TFE3) gene fusion is a rare tumor, and the prognosis of this tumor is poorer compared with that of other subtypes of RCC. The patient presented herein was a 70-year-old man who presented with a solid mass sized ~8.2×6.1 cm in the right kidney and underwent radical right nephrectomy. Following pathological and immunohistochemical (IHC) examination and fluorescent in situ hybridization (FISH), the patient was diagnosed with Xp11.2 translocation RCC with TFE3 gene fusion. These tumors are more commonly encountered in children rather than in adults, and adult Xp11.2 translocation RCC is associated with a poorer prognosis compared with its pediatric counterpart. IHC assay and FISH are important diagnostic methods. However, there is currently no established effective treatment for Xp11.2 RCC.

Xp11.2 translocation renal cell carcinoma with multiple bone metastases: A case report.

PubMed

Liu, Jiaju; Su, Zhengming; Li, Yifan; Chen, Duqun; Ni, Liangchao; Mao, Xiangming; Yang, Shangqi; Lai, Yongqing

2016-03-01

Xp11.2 translocation/transcription factor enhancer 3 (TFE3) fusion gene associated with renal cell carcinoma (Xp11.2 translocation RCC) is rare and occurs predominantly in children and adolescents. The current study reports the case of a 14-year-old male with Xp11.2 translocation RCC, who presented with chest pain that had persisted for 1 month. A solid neoplasm was located in the left kidney of the patient. Contrast-enhanced computed tomography revealed the presence of a solid mass in the kidney, with uneven enhancement. Destruction of multiple bones was also observed. The patient was treated with a radical nephrectomy. The pathological examination of the tumor revealed that the tumor cells contained an eosinophilic cytoplasm in the renal interstitial tissue. Immunohistochemistry revealed that the tumor cells expressed P504S, cluster of differentiation 10, pan-cytokeratin, vimentin and TFE3. In conclusion, Xp11.2 translocation RCC is a rare type of kidney cancer. Diagnosing this disease prior to surgery is challenging, and providing a definite diagnosis requires histopathological and immunohistochemical examination, while genetic analysis may also be required.

First reported case of intrachromosomal cryptic inv dup del Xp in a boy with developmental retardation.

PubMed

Dupont, Celine; Lebbar, Aziza; Teinturier, Cecile; Baverel, Françoise; Viot, Geraldine; Le Tessier, Dominique; Le Bozec, Jerome; Cuisset, Laurence; Dupont, Jean-Michel

2007-06-01

We report here on a 6-year-old boy referred to the laboratory for karyotyping and SHOX microdeletion testing. The most significant clinical findings in this boy were small stature, Madelung deformity, facial dysmorphism, mild mental retardation and behavioral problems. R-, G- and RTBG-banding chromosome analysis showed a normal male karyotype. Fine molecular characterization, by FISH, of terminal Xp microdeletion revealed an associated partial duplication. Further refinement of the molecular analysis indicated an inverted duplication of the Xp22.31-Xp22.32 (13.7 Mb) region including the STS, VCX-A and KAL1 genes, associated with a terminal Xp deletion Xp22.33-Xpter (3.6 Mb) encompassing the SHOX and ARSE genes. Such rearrangements have been characterized for other chromosomal pairs, but this is the first reported male patient involving the short arm of the X chromosome. Molecular analysis of the maternal and patient's microsatellite markers showed interchromatid mispairing leading to non-allelic homologous recombination to be the most likely mechanism underlying this rearrangement. This case highlights the importance of clinically driven FISH investigations in order to uncover cryptic micro-rearrangements. Copyright (c) 2007 Wiley-Liss, Inc.

Nephron-sparing surgery in the treatment of pediatric renal cell carcinoma associated with Xp11.2 translocation/TFE3 gene fusions.

PubMed

Liu, Chao; Zhang, Weiping; Song, Hongcheng

2017-09-01

To investigate the safety and efficacy of nephron-sparing surgery (NSS) in the treatment of pediatric Xp11.2 translocation renal cell carcinoma (RCC). Clinical characteristics of 9 RCC children (7 males and 2 females) with Xp11.2 translocation who received NSS between January 1973 and December 2015 were retrospectively analyzed. The mean age was 7.8years (range: 4.5-13.5years). Xp11.2 translocation RCC was found in the left side in 4 patients and right in 5. 3 tumors were located in the upper pole of the kidney, 1 in the middle dorsal, 1 in the middle ventral and 4 in the lower pole. RCC presented with painless gross hematuria in 4 patients, abdominal mass in 1, and as an incidental finding by ultrasound examination in 4 patients. The mean course of hematuria was 3months (range: 1-7months). The mean tumor diameters were 3.7cm (range: 2.2-6.9cm). All the patients received NSS with open transperitoneal approach. The mean operative time and estimated blood loss were 115min and 40ml, respectively. The time of renal pedicle clamping was 19-25min (mean: 21.5min). No complications (such as leakage of urine, prolonged drainage or secondary bleeding) were noted. No patients experienced local recurrence during the mean of 50.1-month follow-up (range: 13-117months). Intravenous urography (IVU) or contrast-enhanced CT was conducted at 6months after surgery which showed favorable kidney function in all patients. Xp11.2 translocation RCC is a predominant pathological but biologically inert type of pediatric RCC. For Xp11.2 translocation RCC sized <4-7cm in diameter and located in one pole, NSS is safe and feasible. Treatment Studies, LEVEL IV. Copyright © 2017 Elsevier Inc. All rights reserved.

Diagnosis of adults Xp11.2 translocation renal cell carcinoma by immunohistochemistry and FISH assays: clinicopathological data from ethnic Chinese population.

PubMed

Qu, Yuanyuan; Gu, Chengyuan; Wang, Hongkai; Chang, Kun; Yang, Xiaoqun; Zhou, Xiaoyan; Dai, Bo; Zhu, Yao; Shi, Guohai; Zhang, Hailiang; Ye, Dingwei

2016-02-16

This study aimed to assess the utility of transcription factor E3 (TFE3) break-apart fluorescence in situ hybridization (FISH) assay in diagnosis of Xp11.2 translocation renal cell carcinoma (Xp11.2 RCC) and to compare the clinicopathological features between adult Xp11.2 RCC and non-Xp11.2 RCC. 76 pathologically suspected Xp11.2 RCCs were recruited from our institution. Both TFE3 immunohistochemistry (IHC) and TFE3 FISH assay were performed for the entire cohort. The progression-free survival (PFS) and overall survival (OS) curves were estimated using the Kaplan-Meier method. FISH analysis confirmed 30 Xp11.2 RCCs, including 28 cases with positive TFE3 immunostaining and 2 cases with negative immunostaining. The false-positive and false-negative rates were 6.7% (2/30) and 4.3% (2/46), respectively, for TFE3 IHC compared with FISH assay. Xp11.2 RCC was significantly associated with higher pathological stage and Fuhrman nuclear grade compared with non-Xp11.2 RCC (P < 0.05). The median PFS and OS for TFE3 FISH-positive group were 13.0 months (95% CI, 8.4-17.6 months) and 50.0 months (95% CI, 27.6-72.4 months), respectively, while the median PFS and OS had not been reached for TFE3 FISH-negative group. In conclusion, TFE3 break-apart FISH assay is a highly useful and standard diagnostic method for Xp11.2 RCC. Adult Xp11.2 RCC is clinically aggressive and often presents at advanced stage with poor prognosis.

Sirt1 suppresses RNA synthesis after UV irradiation in combined xeroderma pigmentosum group D/Cockayne syndrome (XP-D/CS) cells.

PubMed

Vélez-Cruz, Renier; Zadorin, Anton S; Coin, Frédéric; Egly, Jean-Marc

2013-01-15

Specific mutations in the XPD subunit of transcription factor IIH result in combined xeroderma pigmentosum (XP)/Cockayne syndrome (CS), a severe DNA repair disorder characterized at the cellular level by a transcriptional arrest following UV irradiation. This transcriptional arrest has always been thought to be the result of faulty transcription-coupled repair. In the present study, we showed that, following UV irradiation, XP-D/CS cells displayed a gross transcriptional dysregulation compared with "pure" XP-D cells or WT cells. Furthermore, global RNA-sequencing analysis showed that XP-D/CS cells repressed the majority of genes after UV, whereas pure XP-D cells did not. By using housekeeping genes as a model, we demonstrated that XP-D/CS cells were unable to reassemble these gene promoters and thus to restart transcription after UV irradiation. Furthermore, we found that the repression of these promoters in XP-D/CS cells was not a simple consequence of deficient repair but rather an active heterochromatinization process mediated by the histone deacetylase Sirt1. Indeed, RNA-sequencing analysis showed that inhibition of and/or silencing of Sirt1 changed the chromatin environment at these promoters and restored the transcription of a large portion of the repressed genes in XP-D/CS cells after UV irradiation. Our work demonstrates that a significant part of the transcriptional arrest displayed by XP-D/CS cells arises as a result of an active repression process and not simply as a result of a DNA repair deficiency. This dysregulation of Sirt1 function that results in transcriptional repression may be the cause of various severe clinical features in patients with XP-D/CS that cannot be explained by a DNA repair defect.

Pseudocapsule of renal cell carcinoma associated with Xp11.2 translocation/TFE3 gene fusion: a clue for tumor enucleation?

PubMed

Cheng, Xiangming; He, Jian; Gan, Weidong; Fan, Xiangshan; Yang, Jun; Zhu, Bin; Guo, Hongqian

2015-01-01

To evaluate the feasibility and efficacy of tumor enucleation (TE) for patients with small renal cell carcinoma (RCC) associated with Xp11.2 translocation/TFE3 gene fusion (Xp11.2 RCC) by analyzing the pseudocapsule characteristics of Xp11.2 RCCs comparing with that of clear cell renal cell carcinoma (ccRCC). From June 2007 to February 2014, 22 patients with Xp11.2 RCC who were diagnosed by fluorescence in-situ hybridization polyclonal (FISH) assay and 32 patients with ccRCC treated in our institution were comparatively studied. 12 patients with ccRCC underwent radical nephrectomy (RN) and 20 received TE. Among 22 patients with Xp11.2 RCC, 19 were treated by RN and 3 by TE (1 by radiofrequency ablation assisted TE). Pseudocapsule and other clinicopathological characteristics of the two subtypes of RCC were compared. Survival of patients treated with different surgical methods was evaluated and compared. Pseudocapsule incidence of Xp11.2 RCC (14/22, 63.6%) was lower than that of ccRCC (32/32, 100%, P<0.001). However, pseudocapsule integrity rate of Xp11.2 RCC (10/14, 71.4%) was comparable with that of ccRCC (23/32, 71.9%, P=1.000). The 5-year overall survival of patients with ccRCC treated with RN and TE was 86% and 81%, respectively (P=0.845). Three patients with small Xp11.2 RCC performed well after TE. Over half Xp11.2 RCC had pseudocapsules, whose integrity rate was comparable to that of ccRCC. Treatment effectives of TE and RN were comparable in ccRCC. A preliminary attempt to treat small Xp11.2 RCC with intact pseudocapsule by using TE produced a favorable treatment outcome.

The multislice CT findings of renal carcinoma associated with XP11.2 translocation/TFE gene fusion and collecting duct carcinoma.

PubMed

Zhu, Qing-Qiang; Wang, Zhong-Qiu; Zhu, Wen-Rong; Chen, Wen-Xin; Wu, Jing-Tao

2013-04-01

Renal cell carcinoma associated with Xp11.2 translocation and TFE gene fusion (Xp11.2/TFE RCC), and collecting duct carcinoma (CDC) are uncommon subtypes of renal cell carcinomas. To investigate the multislice CT (MSCT) characteristics of these two tumor types. Nine patients with Xp11.2/TFE RCC and 10 patients with CDC were studied retrospectively. MSCT was undertaken to investigate differences in tumor characteristics and enhancement patterns. All patients had single tumors centered in the renal medulla. Two patients with each tumor type had lymph node involvement and there was a single case of hepatic metastasis (Xp11.2/TFE RCC). The mean tumor diameter of Xp11.2/TFE RCC tumors was significantly larger than for CDC tumors. Two patients with Xp11.2/TFE RCC had cystic components as did eight patients with CDC (P < 0.05). Calcifications were present in six patients, each with CDC. Clear tumor boundaries were visible in two patients with CDC and in nine with Xp11.2/TFE RCC (P < 0.05). The density of Xp11.2/TFE RCC tumors was greater than that of CDC tumors, normal renal cortex, or medulla on unenhanced CT. Enhancement was higher with Xp11.2/TFE RCC than with CDC tumors during all phases. Xp11.2/TFE RCC enhancement was higher than in the renal medulla during cortical and medullary phase but lower than in normal renal medulla during the delayed phase. CDC tumor enhancement was lower than that for normal renal medulla during all enhanced phases. Both tumor types originated from the renal medulla. Distinguishing features included density on unenhanced CT, enhancement patterns, and capsule signs. Identifying these differences may aid diagnosis.

Pseudocapsule of renal cell carcinoma associated with Xp11.2 translocation/TFE3 gene fusion: a clue for tumor enucleation?

PubMed Central

Cheng, Xiangming; He, Jian; Gan, Weidong; Fan, Xiangshan; Yang, Jun; Zhu, Bin; Guo, Hongqian

2015-01-01

Objectives: To evaluate the feasibility and efficacy of tumor enucleation (TE) for patients with small renal cell carcinoma (RCC) associated with Xp11.2 translocation/TFE3 gene fusion (Xp11.2 RCC) by analyzing the pseudocapsule characteristics of Xp11.2 RCCs comparing with that of clear cell renal cell carcinoma (ccRCC). Methods: From June 2007 to February 2014, 22 patients with Xp11.2 RCC who were diagnosed by fluorescence in-situ hybridization polyclonal (FISH) assay and 32 patients with ccRCC treated in our institution were comparatively studied. 12 patients with ccRCC underwent radical nephrectomy (RN) and 20 received TE. Among 22 patients with Xp11.2 RCC, 19 were treated by RN and 3 by TE (1 by radiofrequency ablation assisted TE). Pseudocapsule and other clinicopathological characteristics of the two subtypes of RCC were compared. Survival of patients treated with different surgical methods was evaluated and compared. Results: Pseudocapsule incidence of Xp11.2 RCC (14/22, 63.6%) was lower than that of ccRCC (32/32, 100%, P<0.001). However, pseudocapsule integrity rate of Xp11.2 RCC (10/14, 71.4%) was comparable with that of ccRCC (23/32, 71.9%, P=1.000). The 5-year overall survival of patients with ccRCC treated with RN and TE was 86% and 81%, respectively (P=0.845). Three patients with small Xp11.2 RCC performed well after TE. Conclusions: Over half Xp11.2 RCC had pseudocapsules, whose integrity rate was comparable to that of ccRCC. Treatment effectives of TE and RN were comparable in ccRCC. A preliminary attempt to treat small Xp11.2 RCC with intact pseudocapsule by using TE produced a favorable treatment outcome. PMID:26191243

Xp11.2 translocation renal cell carcinoma with multiple bone metastases: A case report

PubMed Central

LIU, JIAJU; SU, ZHENGMING; LI, YIFAN; CHEN, DUQUN; NI, LIANGCHAO; MAO, XIANGMING; YANG, SHANGQI; LAI, YONGQING

2016-01-01

Xp11.2 translocation/transcription factor enhancer 3 (TFE3) fusion gene associated with renal cell carcinoma (Xp11.2 translocation RCC) is rare and occurs predominantly in children and adolescents. The current study reports the case of a 14-year-old male with Xp11.2 translocation RCC, who presented with chest pain that had persisted for 1 month. A solid neoplasm was located in the left kidney of the patient. Contrast-enhanced computed tomography revealed the presence of a solid mass in the kidney, with uneven enhancement. Destruction of multiple bones was also observed. The patient was treated with a radical nephrectomy. The pathological examination of the tumor revealed that the tumor cells contained an eosinophilic cytoplasm in the renal interstitial tissue. Immunohistochemistry revealed that the tumor cells expressed P504S, cluster of differentiation 10, pan-cytokeratin, vimentin and TFE3. In conclusion, Xp11.2 translocation RCC is a rare type of kidney cancer. Diagnosing this disease prior to surgery is challenging, and providing a definite diagnosis requires histopathological and immunohistochemical examination, while genetic analysis may also be required. PMID:26998154

PD-L1 expression in Xp11.2 translocation renal cell carcinoma: Indicator of tumor aggressiveness.

PubMed

Chang, Kun; Qu, Yuanyuan; Dai, Bo; Zhao, Jian-Yuan; Gan, Hualei; Shi, Guohai; Zhu, Yiping; Shen, Yijun; Zhu, Yao; Zhang, Hailiang; Ye, Dingwei

2017-05-18

Programmed death ligand-1 (PD-L1), a promising antitumor target, has proven clinical value against many malignancies. However, the PD-L1 content of Xp11.2 translocation renal cell carcinoma (Xp11.2 RCC) and its correlation with clinical outcomes remain unclear. This study aimed to investigate PD-L1 expression in Xp11.2 RCC and to assess its prognostic value. Formalin-fixed paraffin-embedded specimens from 36 adult patients that were histologically confirmed (by fluorescence in situ hybridization) were subjected to immunohistochemical analysis. Of the 36 Xp11.2 RCC patients, 9 (25.0%) had tumors with positive PD-L1 expression and 27 (75.0%) had tumors with negative PD-L1 expression. Positive PD-L1 expression correlated with advanced tumor stage (P = 0.001), regional lymph node metastasis (P < 0.001), and distant metastasis (P < 0.001). A multivariate analysis identified positive PD-L1 expression was an independent adverse prognostic factor for both progression free survival (hazard ratio: 3.7, P = 0.018) and overall survival (hazard ratio: 4.5, P = 0.034). The median PFS and OS for the whole cohort were 13.0 months (95% confidence interval [CI], 9.4-16.6 months) and 36.0 months (95% CI, 23.9-48.1 months), respectively. Our findings suggest that positive PD-L1 expression is indicative of worse clinical outcome in Xp11.2 RCC. Further studies are needed to explore the potential efficacy of targeting PD-L1 in Xp11.2 RCC.

Diagnosis of adults Xp11.2 translocation renal cell carcinoma by immunohistochemistry and FISH assays: clinicopathological data from ethnic Chinese population

PubMed Central

Qu, Yuanyuan; Gu, Chengyuan; Wang, Hongkai; Chang, Kun; Yang, Xiaoqun; Zhou, Xiaoyan; Dai, Bo; Zhu, Yao; Shi, Guohai; Zhang, Hailiang; Ye, Dingwei

2016-01-01

This study aimed to assess the utility of transcription factor E3 (TFE3) break-apart fluorescence in situ hybridization (FISH) assay in diagnosis of Xp11.2 translocation renal cell carcinoma (Xp11.2 RCC) and to compare the clinicopathological features between adult Xp11.2 RCC and non-Xp11.2 RCC. 76 pathologically suspected Xp11.2 RCCs were recruited from our institution. Both TFE3 immunohistochemistry (IHC) and TFE3 FISH assay were performed for the entire cohort. The progression-free survival (PFS) and overall survival (OS) curves were estimated using the Kaplan-Meier method. FISH analysis confirmed 30 Xp11.2 RCCs, including 28 cases with positive TFE3 immunostaining and 2 cases with negative immunostaining. The false-positive and false-negative rates were 6.7% (2/30) and 4.3% (2/46), respectively, for TFE3 IHC compared with FISH assay. Xp11.2 RCC was significantly associated with higher pathological stage and Fuhrman nuclear grade compared with non-Xp11.2 RCC (P < 0.05). The median PFS and OS for TFE3 FISH-positive group were 13.0 months (95% CI, 8.4–17.6 months) and 50.0 months (95% CI, 27.6–72.4 months), respectively, while the median PFS and OS had not been reached for TFE3 FISH-negative group. In conclusion, TFE3 break-apart FISH assay is a highly useful and standard diagnostic method for Xp11.2 RCC. Adult Xp11.2 RCC is clinically aggressive and often presents at advanced stage with poor prognosis. PMID:26880493

Short stature in a mother and daughter with terminal deletion of Xp22.3

DOE Office of Scientific and Technical Information (OSTI.GOV)

Schwinger, E.; Kirschstein, M.; Konermann, T.

1996-05-03

Short stature in females is often caused by homozygosity for the terminal portion of Xp due to monosomy X or a deletion. We report on a mother and daughter with short stature as sole phenotypic abnormality and deletion of bands Xp22.32-p22.33 demonstrated by classic and molecular cytogenetic analysis. In both individuals, the deleted X chromosome was late replicating. Molecular analysis suggested that the deletion is terminal and the breakpoint was localized between the STS and DXS7470 loci in Xp22.32. Chromosome analysis is often done on females with short stature to exclude Ullrich-Turner syndrome. Small deletions, terminal or interstitial, are easilymore » missed by conventional cytogenetic investigation; thus molecular analyses are useful to detect those cases. 8 refs., 3 figs.« less

Nance-Horan syndrome: linkage analysis in 4 families refines localization in Xp22.31-p22.13 region.

PubMed

Toutain, A; Ronce, N; Dessay, B; Robb, L; Francannet, C; Le Merrer, M; Briard, M L; Kaplan, J; Moraine, C

1997-02-01

Nance-Horan syndrome (NHS) is an X-linked disease characterized by severe congenital cataract with microcornea, distinctive dental findings, evocative facial features and mental impairment in some cases. Previous linkage studies have placed the NHS gene in a large region from DXS143 (Xp22.31) to DXS451 (Xp22.13). To refine this localization further, we have performed linkage analysis in four families. As the maximum expected Lod score is reached in each family for several markers in the Xp22.31-p22.13 region and linkage to the rest of the X chromosome can be excluded, our study shows that NHS is a genetically homogeneous condition. An overall maximum two-point Lod score of 9.36 (theta = 0.00) is obtained with two closely linked markers taken together. DXS207 and DXS1053 in Xp22.2. Recombinant haplotypes indicate that the NHS gene lies between DXS85 and DXS1226. Multipoint analysis yield a maximum Lod score of 9.45 with the support interval spanning a 15-cM region that includes DXS16 and DXS1229/365. The deletion map of the Xp22.3-Xp21.3 region suggests that the phenotypic variability of NHS is not related to gross rearrangement of sequences of varying size but rather to allelic mutations in a single gene, presumably located proximal to DXS16 and distal to DXS1226. Comparison with the map position of the mouse Xcat mutation supports the location of the NHS gene between the GRPR and PDHA1 genes in Xp22.2.

Melanotic Xp11 translocation renal cancer: report of a case with a unique intratumoral sarcoid-like reaction.

PubMed

Ritterhouse, Lauren L; Cykowski, Matthew D; Hassell, Lewis A; Slobodov, Gennady; Bane, Barbara L

2014-04-15

Melanotic Xp11 translocation renal cancer is a rare tumor belonging to the family of microphthalmia-associated transcription factor (MiTF)/transcription factor E (TFE) neoplasms. This tumor family also includes alveolar soft part sarcoma, perivascular epithelioid cell neoplasms, Xp11 translocation renal cell carcinoma, and melanoma. To date, six confirmed melanotic Xp11 translocation cancers (five renal, one ovarian) have been reported in the literature. Here, we report the clinical, histologic, immunohistochemical, and molecular features of a unique melanotic Xp11 translocation renal cancer arising in a 34-year-old African-American female. Histologically, the tumor was composed of epithelioid tumor cells arranged in a nested pattern. The cells had clear to eosinophilic granular cytoplasm, vesicular nuclear chromatin, and prominent nucleoli. Multifocal intracytoplasmic deposits of granular brown melanin pigment were identified and confirmed by Fontana-Masson stain. An unusual histologic feature, not previously reported in melanotic Xp11 translocation renal cancer, was a sarcoid-like granulomatous reaction consisting of tight epithelioid granulomas with lymphocytic cuffing, numerous giant cells, and calcifications. Nuclear transcription factor E3 expression was identified by immunohistochemistry and TFE3 rearrangement was confirmed by fluorescence in situ hybridization. Additional immunohistochemical findings included immunoreactivity for HMB45, cathepsin K, and progesterone receptor; negative staining was seen with actin, desmin, cytokeratins, epithelial membrane antigen, CD10, vimentin, and PAX-8. The patient is currently free of disease, two years following initial clinicoradiologic presentation and twenty-two months following partial nephrectomy without additional therapy. This report further expands the spectrum of morphologic and clinical findings previously described in melanotic Xp11 translocation renal cancer, a distinctive tumor showing overlapping

Renal cell carcinoma associated with Xp11.2 translocation/TFE gene fusion: imaging findings in 21 patients.

PubMed

Chen, Xiao; Zhu, Qingqiang; Li, Baoxin; Cui, Wenjing; Zhou, Hao; Duan, Na; Liu, Yongkang; Kundra, Vikas; Wang, Zhongqiu

2017-02-01

To characterize imaging features of renal cell carcinoma (RCC) associated with Xp11.2 translocation/TFE gene fusion. Twenty-one patients with Xp11.2/TFE RCC were retrospectively evaluated. Tumour location, size, density, cystic or solid appearance, calcification, capsule sign, enhancement pattern and metastases were assessed. Fourteen women and seven men were identified with 12 being 25 years old or younger. Tumours were solitary and cystic-solid (76.2 %) masses with a capsule (76.2 %); 90.5 % were located in the medulla. Calcifications and lymph node metastases were each observed in 24 %. On unenhanced CT, tumour attenuation was greater than in normal renal parenchyma (85.7 %). Tumour enhancement was less than in normal renal cortex on all enhanced phases, greater than in normal renal medulla on cortical and medullary phases, but less than in normal renal medulla on delayed phase. On MR, the tumours were isointense on T1WI, heterogeneously hypointense on T2WI and slightly hyperintense on diffusion-weighted imaging. Xp11.2/TFE RCC usually occurs in young women. It is a cystic-solid, hyperdense mass with a capsule. It arises from the renal medulla with enhancement less than in the cortex but greater than in the medulla in all phases except the delayed phase, when it is lower than in the medulla. • Xp11.2/TFE RCC was more prevalent in young women. • On unenhanced CT, Xp11.2/TFE RCC attenuation was greater than in renal parenchyma. • Xp111/2TFE RCC arises primarily from the renal medulla. • Xp11.2/TFE RCC enhancement was less than in the cortex on all phases. • Enhancement was greater than in the medulla in arterial and corticomedullary phase.

Cu3-xP Nanocrystals as a Material Platform for Near-Infrared Plasmonics and Cation Exchange Reactions

PubMed Central

2015-01-01

Synthesis approaches to colloidal Cu3P nanocrystals (NCs) have been recently developed, and their optical absorption features in the near-infrared (NIR) have been interpreted as arising from a localized surface plasmon resonance (LSPR). Our pump–probe measurements on platelet-shaped Cu3-xP NCs corroborate the plasmonic character of this absorption. In accordance with studies on crystal structure analysis of Cu3P dating back to the 1970s, our density functional calculations indicate that this material is substoichiometric in copper, since the energy of formation of Cu vacancies in certain crystallographic sites is negative, that is, they are thermodynamically favored. Also, thermoelectric measurements point to a p-type behavior of the majority carriers from films of Cu3-xP NCs. It is likely that both the LSPR and the p-type character of our Cu3-xP NCs arise from the presence of a large number of Cu vacancies in such NCs. Motivated by the presence of Cu vacancies that facilitate the ion diffusion, we have additionally exploited Cu3-xP NCs as a starting material on which to probe cation exchange reactions. We demonstrate here that Cu3-xP NCs can be easily cation-exchanged to hexagonal wurtzite InP NCs, with preservation of the anion framework (the anion framework in Cu3-xP is very close to that of wurtzite InP). Intermediate steps in this reaction are represented by Cu3-xP/InP heterostructures, as a consequence of the fact that the exchange between Cu+ and In3+ ions starts from the peripheral corners of each NC and gradually evolves toward the center. The feasibility of this transformation makes Cu3-xP NCs an interesting material platform from which to access other metal phosphides by cation exchange. PMID:25960605

Postoperative recurrence of adult renal cell carcinoma associated with Xp11.2 translocation/TFE3 gene fusion.

PubMed

Wang, Zhen; Liu, Ning; Gan, Weidong; Li, Xiaogong; Zhang, Gutian; Li, Dongmei; Guo, Hongqian

2017-08-01

Objective To analyze the postoperative recurrence of renal cell carcinoma associated with Xp11.2 translocation/TFE3 gene fusion (Xp11.2 tRCC). Methods This retrospective study was approved by the institutional review board and performed in accordance with the ethical standards established by the institution. Demographic, clinical, pathological, and follow-up data were compiled for the study cohort. Results During a mean follow-up of 41.3 months (range, 3-104 months), 8 of 34 patients with Xp11.2 tRCC were confirmed to have recurrence. Three of these patients died with poor outcomes due to a vena cava tumor embolus, and one died of distant metastasis 48 months after the initial nephrectomy during which lymph node metastasis was found. Three patients survived after cytoreduction surgery. One patient was diagnosed with lung metastasis 11 months postoperatively. Conclusions The TNM classification provides significant prognostic information for Xp11.2 tRCC. A relatively active surveillance algorithm is recommended, and cytoreduction surgery is an effective approach for recurrent Xp11.2 tRCC. Larger studies are required to more extensively investigate the recurrence of these potentially aggressive tumors.

Postoperative recurrence of adult renal cell carcinoma associated with Xp11.2 translocation/TFE3 gene fusion

PubMed Central

Wang, Zhen; Liu, Ning; Li, Xiaogong; Zhang, Gutian; Li, Dongmei; Guo, Hongqian

2017-01-01

Objective To analyze the postoperative recurrence of renal cell carcinoma associated with Xp11.2 translocation/TFE3 gene fusion (Xp11.2 tRCC). Methods This retrospective study was approved by the institutional review board and performed in accordance with the ethical standards established by the institution. Demographic, clinical, pathological, and follow-up data were compiled for the study cohort. Results During a mean follow-up of 41.3 months (range, 3–104 months), 8 of 34 patients with Xp11.2 tRCC were confirmed to have recurrence. Three of these patients died with poor outcomes due to a vena cava tumor embolus, and one died of distant metastasis 48 months after the initial nephrectomy during which lymph node metastasis was found. Three patients survived after cytoreduction surgery. One patient was diagnosed with lung metastasis 11 months postoperatively. Conclusions The TNM classification provides significant prognostic information for Xp11.2 tRCC. A relatively active surveillance algorithm is recommended, and cytoreduction surgery is an effective approach for recurrent Xp11.2 tRCC. Larger studies are required to more extensively investigate the recurrence of these potentially aggressive tumors. PMID:28587544

Xeroderma Pigmentosum: Low Prevalence of Germline XPA Mutations in a Brazilian XP Population

PubMed Central

Santiago, Karina Miranda; França de Nóbrega, Amanda; Rocha, Rafael Malagoli; Rogatto, Silvia Regina; Achatz, Maria Isabel

2015-01-01

Xeroderma pigmentosum (XP) is a rare autosomal recessive disorder characterized by DNA repair defects that cause photophobia, sunlight-induced cancers, and neurodegeneration. Prevalence of germline mutations in the nucleotide excision repair gene XPA vary significantly in different populations. No Brazilian patients have been reported to carry a germline mutation in this gene. In this study, the germline mutational status of XPA was determined in Brazilian patients exhibiting major clinical features of XP syndrome. The study was conducted on 27 unrelated patients from select Brazilian families. A biallelic inactivating transition mutation c.619C>T (p.Arg207Ter) was identified in only one patient with a history of neurological impairment and mild skin abnormalities. These findings suggest that XP syndrome is rarely associated with inherited disease-causing XPA mutations in the Brazilian population. Additionally, this report demonstrates the effectiveness of genotype-phenotype correlation as a valuable tool to guide direct genetic screening. PMID:25913378

Xeroderma pigmentosum: low prevalence of germline XPA mutations in a Brazilian XP population.

PubMed

Santiago, Karina Miranda; França de Nóbrega, Amanda; Rocha, Rafael Malagoli; Rogatto, Silvia Regina; Achatz, Maria Isabel

2015-04-22

Xeroderma pigmentosum (XP) is a rare autosomal recessive disorder characterized by DNA repair defects that cause photophobia, sunlight-induced cancers, and neurodegeneration. Prevalence of germline mutations in the nucleotide excision repair gene XPA vary significantly in different populations. No Brazilian patients have been reported to carry a germline mutation in this gene. In this study, the germline mutational status of XPA was determined in Brazilian patients exhibiting major clinical features of XP syndrome. The study was conducted on 27 unrelated patients from select Brazilian families. A biallelic inactivating transition mutation c.619C>T (p.Arg207Ter) was identified in only one patient with a history of neurological impairment and mild skin abnormalities. These findings suggest that XP syndrome is rarely associated with inherited disease-causing XPA mutations in the Brazilian population. Additionally, this report demonstrates the effectiveness of genotype-phenotype correlation as a valuable tool to guide direct genetic screening.

Rapidly progressing renal cell carcinoma associated with Xp11.2 translocations: a case report

PubMed Central

2012-01-01

Introduction Renal cell carcinoma associated with Xp11.2 translocations is frequently reported in children, but adult-onset is rare. Here, the case of an adult male who developed a renal cell carcinoma associated with Xp11.2 translocations is presented. Case presentation A 38-year-old Asian man presented with left back pain and macroscopic hematuria. Computed tomography revealed a left renal tumor (T3N2M0), and a left radical nephrectomy was performed. Hematoxylin-eosin staining revealed papillary architecture and clear or eosinophilic cytoplasm, and the diagnosis of renal cell carcinoma associated with Xp11.2 translocations/TFE3 gene fusion was made by the immunohistochemical determination of transcription factor E3 protein. In spite of adjuvant therapy with α-interferon, a recurrent tumor was found in his left lung by computed tomography three months after the nephrectomy. Interleukin-2, tyrosine kinase inhibitors and mammalian target of rapamycin inhibitors showed no effect on tumor progression. Conclusions Renal cell carcinomas associated with Xp11.2 translocations have an aggressive clinical course in adults. Strict diagnosis using the immunohistochemistry of transcription factor E3 protein is important to predict the prognosis of such patients and new strategies need to be determined to treat patients with these tumors PMID:22738297

Rapidly progressing renal cell carcinoma associated with Xp11.2 translocations: a case report.

PubMed

Morii, Akihiro; Fujiuchi, Yasuyoshi; Nomoto, Kazuhiro; Komiya, Akira; Fuse, Hideki

2012-06-27

Renal cell carcinoma associated with Xp11.2 translocations is frequently reported in children, but adult-onset is rare. Here, the case of an adult male who developed a renal cell carcinoma associated with Xp11.2 translocations is presented. A 38-year-old Asian man presented with left back pain and macroscopic hematuria. Computed tomography revealed a left renal tumor (T3N2M0), and a left radical nephrectomy was performed. Hematoxylin-eosin staining revealed papillary architecture and clear or eosinophilic cytoplasm, and the diagnosis of renal cell carcinoma associated with Xp11.2 translocations/TFE3 gene fusion was made by the immunohistochemical determination of transcription factor E3 protein. In spite of adjuvant therapy with α-interferon, a recurrent tumor was found in his left lung by computed tomography three months after the nephrectomy. Interleukin-2, tyrosine kinase inhibitors and mammalian target of rapamycin inhibitors showed no effect on tumor progression. Renal cell carcinomas associated with Xp11.2 translocations have an aggressive clinical course in adults. Strict diagnosis using the immunohistochemistry of transcription factor E3 protein is important to predict the prognosis of such patients and new strategies need to be determined to treat patients with these tumors.

Structure and Barr body formation of an Xp + chromosome with two inactivation centers.

PubMed Central

Daly, R F; Patau, K; Therman, E; Sarto, G E

1977-01-01

A patients with seizures, Von Willebrand disease, and symptoms of Turner syndrome was a chromosomal mosaic. In blood culture (1974), 56% of the cells were 45, X 33% 46, XXp+ and 11% 47,XXp + Xp +; in the skin, no cells with 47 chromosomes were found. Presumably the Xp + chromosome arose through a break in the Q-banded dark region next to the centromere on Xp to which an Xq had been attached. The abnormal X was late-labeling and formed a larger than normal Barr body. Of the chromatin-positive fibroblasts, 18.2% showed bipartite Barr bodies, which agrees with the hypothesis that the X inactivation center lies on the proximal part of the Xq. On the basis of the structure and behavior of the bipartite bodies in the present patient, as compared to those formed by other chromosomes with two presumed inactivation centers, we propose that the dark region next to the centromere of Xp remains active in the inactive X. In cells with 45,X and 46,XY, this region has the same relative size, whereas it is significantly shorter in the active X of three females, including the present patient, with one abnormal X. We propose that this region on the active X reveals different states of activity, as reflected in its length, depending on how many other X chromosomes are in the cell. Images Fig. 1 Fig. 2 Fig. 3 PMID:299980

A case of PSF-TFE3 gene fusion in Xp11.2 renal cell carcinoma with melanotic features.

PubMed

Zhan, He-Qin; Chen, Hong; Wang, Chao-Fu; Zhu, Xiong-Zeng

2015-03-01

Xp11.2 translocation renal cell carcinoma (Xp11.2 RCC) with PSF-TFE3 gene fusion is a rare neoplasm. Only 22 cases of Xp11.2 RCCs with PSF-TFE3 have been reported to date. We describe an additional case of Xp11.2 RCC with PSF-TFE3 showing melanotic features. Microscopically, the histologic features mimic clear cell renal cell carcinoma. However, the dark-brown pigments were identified and could be demonstrated as melanins. Immunohistochemically, the tumor cells were widely positive for CD10, human melanoma black 45, and TFE3 but negative for cytokeratins, vimentin, Melan-A, microphthalmia-associated transcription factor, smooth muscle actin, and S-100 protein. Genetically, we demonstrated PSF-TFE3 fusion between exon 9 of PSF and exon 5 of TFE3. The patient was free of disease with 50 months of follow-up. The prognosis of this type of tumor requires more cases because of limited number of cases and follow-up period. Xp11.2 RCC with PSF-TFE3 inevitably requires differentiation from other kidney neoplasms. Immunohistochemical and molecular genetic analyses are essential for accurate diagnosis. Copyright © 2015 Elsevier Inc. All rights reserved.

Renal cell carcinoma associated with transcription factor E3 expression and Xp11.2 translocation: incidence, characteristics, and prognosis.

PubMed

Klatte, Tobias; Streubel, Berthold; Wrba, Friedrich; Remzi, Mesut; Krammer, Barbara; de Martino, Michela; Waldert, Matthias; Marberger, Michael; Susani, Martin; Haitel, Andrea

2012-05-01

We studied the characteristics and prognosis of renal cell carcinoma (RCC) associated with Xp11.2 translocation and transcription factor E3 (TFE3) expression and determined the need for genetic analysis in routine diagnostics. Of 848 consecutive cases, 75 showed microscopic features suggestive of Xp11.2 translocation RCC or occurred in patients 40 years or younger. Of these cases, 17 (23%) showed strong nuclear TFE3 immunostaining, which was associated with more advanced tumors and inverse prognosis in univariate (P = .032) but not multivariate (P = .404) analysis. With fluorescence in situ hybridization and polymerase chain reaction, only 2 cases showed alterations of the X chromosome and the ASPL-TFE3 gene fusion, respectively. In our laboratory, the predictive value of TFE3 expression for the Xp11.2 translocation was 12%. Strong nuclear TFE3 expression is associated with metastatic spread and a poor prognosis. In our laboratory, TFE3 is not diagnostic for Xp11.2 translocation RCC. Diagnosis of Xp11.2 translocation RCC may be made only genetically.

OMEGAHAB-XP a bioregenerative aquatic life support system designed to be used in Bion-M1 long term space flight

NASA Astrophysics Data System (ADS)

Hilbig, Reinhard; Lebert, Michael

The OmegaHab XP Experiment will be based on the OmegaHab system successfully flown in the context of the FOTON M3 mission. OmegaHab XP -a refurbished OmegaHab for a long term mission -is in general assembled from four parts: an algae compartment, a nutrition com-partment for higher plants and crustaceens, a fish compartment and a filter compartment with biodegradant bacterias. The algae compartment (Euglena gracilis; unicellular, photosynthetic flagellate) will be illuminated with photosynthetic active radiation and will produce oxygen. The photosynthetic process also consumes carbon dioxide and if available ammonia. In addi-tion, nitrate will be taken up by the algae and by this means removed from the system. Via a gas-permeable membrane (gas/ion exchanger) the produced oxygen will be transported in a separate fish compartment. The metabolism of the fish will produce carbon dioxide and nitro-genic components. These components as well as the carbon dioxide will be transported back in the algae compartment and subsequently used by the algae. The transport of the components is enhanced by a counter flow inside the gas/ion exchanger driven by a pump. In addition, a filter system is installed which removes debris as well as ammonia by means of ammonia metabolizing bacteria. The nutrition compartment with higher plants and the crustaceans (e.g. Hyalella azteca; flown successfully aboard shuttles) builds the basis of this multi-trophic sys-tem. Hyalella azteca can reproduce in an adequate amount to replace external fish nutrition for Oreochromis mossambicus in large parts. The fish compartment is divided into two chambers: a hatchery chamber for larval fishes and an chamber for subadult Oreochromis mossambicus. The system is fully automatic and measures and stores all house-keeping data internally. These house-keeping data include light, temperature, acceleration and oxygen as well as many system related parameters. By means of Peltier-elements the system can be

Simultaneous detection of eight avian influenza A virus subtypes by multiplex reverse transcription-PCR using a GeXP analyser.

PubMed

Li, Meng; Xie, Zhixun; Xie, Zhiqin; Liu, Jiabo; Xie, Liji; Deng, Xianwen; Luo, Sisi; Fan, Qing; Huang, Li; Huang, Jiaoling; Zhang, Yanfang; Zeng, Tingting; Wang, Sheng

2018-04-18

Recent studies have demonstrated that at least eight subtypes of avian influenza virus (AIV) can infect humans, including H1, H2, H3, H5, H6, H7, H9 and H10. A GeXP analyser-based multiplex reverse transcription (RT)-PCR (GeXP-multiplex RT-PCR) assay was developed in our recent studies to simultaneously detect these eight AIV subtypes using the haemagglutinin (HA) gene. The assay consists of chimeric primer-based PCR amplification with fluorescent labelling and capillary electrophoresis separation. RNA was extracted from chick embryo allantoic fluid or liquid cultures of viral isolates. In addition, RNA synthesised via in vitro transcription was used to determine the specificity and sensitivity of the assay. After selecting the primer pairs, their concentrations and GeXP-multiplex RT-PCR conditions were optimised. The established GeXP-multiplex RT-PCR assay can detect as few as 100 copies of premixed RNA templates. In the present study, 120 clinical specimens collected from domestic poultry at live bird markets and from wild birds were used to evaluate the performance of the assay. The GeXP-multiplex RT-PCR assay specificity was the same as that of conventional RT-PCR. Thus, the GeXP-multiplex RT-PCR assay is a rapid and relatively high-throughput method for detecting and identifying eight AIV subtypes that may infect humans.

Laparoscopic radiofrequency ablation-assisted enucleation of Xp11.2 translocation renal cell carcinoma: A case report

PubMed Central

XU, LINFENG; YANG, RONG; WANG, WEI; ZHANG, YIFEN; GAN, WEIDONG

2014-01-01

The current study presents a case of Xp11.2 translocation renal cell carcinoma (Xp11.2 RCC) in a 30-year-old female. The patient was referred to The Affiliated Drum Tower Hospital of the Medical College of Nanjing University (Nanjing, Jiangsu, China) due to a right renal tumor without evident symptoms, which was found by a routine physical examination. A computed tomography (CT) scan indicated that the mass exhibited cystic and solid components. The patient underwent laparoscopic radiofrequency ablation-assisted enucleation. Immunohistochemistry revealed intense nuclear staining for transcription factor E3 protein in the cancer cells. The patient was diagnosed with Xp11.2 RCC. The urological and radiological outcomes remained satisfactory after >2.5 years of follow-up. PMID:25120696

Laparoscopic radiofrequency ablation-assisted enucleation of Xp11.2 translocation renal cell carcinoma: A case report.

PubMed

Xu, Linfeng; Yang, Rong; Wang, Wei; Zhang, Yifen; Gan, Weidong

2014-09-01

The current study presents a case of Xp11.2 translocation renal cell carcinoma (Xp11.2 RCC) in a 30-year-old female. The patient was referred to The Affiliated Drum Tower Hospital of the Medical College of Nanjing University (Nanjing, Jiangsu, China) due to a right renal tumor without evident symptoms, which was found by a routine physical examination. A computed tomography (CT) scan indicated that the mass exhibited cystic and solid components. The patient underwent laparoscopic radiofrequency ablation-assisted enucleation. Immunohistochemistry revealed intense nuclear staining for transcription factor E3 protein in the cancer cells. The patient was diagnosed with Xp11.2 RCC. The urological and radiological outcomes remained satisfactory after >2.5 years of follow-up.

[Application of polyclonal break-apart probes in the diagnosis of Xp11.2 translocation renal cell carcinoma].

PubMed

Chen, Xiancheng; Gan, Weidong; Ye, Qing; Yang, Jun; Guo, Hongqian; Li, Dongmei

2014-12-16

To explore the value of self-designed fluorescent in situ hybridization (FISH) polyclonal break-apart probes specific for TFE3 gene in the diagnosis of Xp11.2 translocation renal cell carcinoma. All tissue samples were collected from 2006 to 2013, including Xp11.2 translocation renal cell carcinoma (n = 10), renal clear cell carcinoma (n = 10) and renal papillary cell carcinoma (n = 10). FISH was conducted for paraffin-embedded tumor tissue sections with probes. The types of fluorescence were observed by fluorescent microscopy to determine the existence or non-existence of translocated TFE3 gene. All sections were successfully probed. The split red and green signals within a single nucleus were detected simultaneously in 9 cases of Xp11.2 translocation renal cell carcinoma as diagnosed by traditional pathological and immunohistochemical methods. And it was consistent with the initial diagnosis. Detection of fusion signal in 1/10 and negative FISH result did not conform to the initial diagnosis. The fluorescent types of renal clear cell carcinoma and renal papillary cell carcinoma were all fusion signals. FISH tests were negative for renal clear and papillary cell carcinomas. Xp11.2 translocation renal cell carcinomas diagnosed by traditional pathological and immunohistochemical methods are sometimes misdiagnosed. Detecting the translocation of TFE3 gene with FISH polyclonal break-apart probes is both accurate and reliable for diagnosing Xp11.2 translocation renal cell carcinoma.

Renal cell carcinoma associated with Xp11.2 translocation/TFE3 gene fusion: radiological findings mimicking papillary subtype.

PubMed

Kato, Hiroki; Kanematsu, Masayuki; Yokoi, Shigeaki; Miwa, Kousei; Horie, Kengo; Deguchi, Takashi; Hirose, Yoshinobu

2011-01-01

The authors describe the computed tomography (CT) and magnetic resonance imaging (MRI) findings of an 18-year-old man with renal cell carcinoma (RCC) associated with the Xp11.2 translocation/transcription factor E3 (TFE3) gene fusion (Xp11 translocation carcinoma). The lesion was hyperdense on unenhanced CT, hypovascular on contrast-enhanced studies, hypointense on T2-weighted MR images, and hemosiderin deposition was suspected on phase-shift gradient-echo MR images. Histopathological specimens revealed pathological findings resembling papillary RCC predominantly and exhibited immunoreactivity for TFE3. Because there is often considerable morphological overlap between this carcinoma and papillary RCC, the imaging findings of Xp11 translocation carcinoma may be similar to those of the papillary subtype. Therefore, Xp11 translocation carcinoma should be considered, particularly in young patients when radiologic images demonstrate a renal tumor mimicking the papillary subtype. Copyright © 2010 Wiley-Liss, Inc.

Xp11.2 Translocation renal cell carcinomas have a poorer prognosis than non-Xp11.2 translocation carcinomas in children and young adults: a meta-analysis.

PubMed

Qiu Rao; Bing Guan; Zhou, Xiao-jun

2010-12-01

Renal cell carcinomas (RCCs) in children and adolescents are much rarer than in adults. In this age group, Xp11.2 translocation RCCs were the most common subtype of pediatric RCCs. Information regarding the clinical behavior of pediatric RCCs remains controversial because of their relatively rare incidence. The authors aimed to perform a systematic review and meta-analysis to better define the biological features of pediatric RCCs. Eligible studies were identified through multiple search strategies. Studies were assessed for quality using the Jadad Quality Scale. Data were collected comparing overall survival (OS), disease-free survival (DFS), and stage in patients with TFE3 + pediatric RCCs and TFE3 - RCCs. A total of 4 studies were included for meta-analysis, and pooled odds ratios (ORs) with 95% confidence interval (CI) were calculated. The meta-analysis outcomes showed that TFE3 + pediatric RCCs were significantly associated with poorer outcomes (OS and DFS) and a higher stage (III/IV) than TFE3 - RCCs (pooled ORs for each group: 4.59 [95% CI = 1.46-14.42] for OS; 5.79 [95% CI = 1.85-18.16] for DFS; and 5.89 [95% CI = 2.23-15.52] for stage). This result was also confirmed by OS and DFS curves (P = .005 and P = .001). Xp11.2 translocation carcinomas appear to have a poorer prognosis than non-Xp11.2 translocation carcinomas in children and young adults.

Renal cell carcinoma associated with Xp11.2 translocation/TFE3 gene fusions: clinical experience and literature review.

PubMed

He, Jian; Chen, Xiancheng; Gan, Weidong; Zhu, Bin; Fan, Xiangshan; Guo, Hongqian; Jia, Ruipeng

2015-01-01

To analyze the clinicopathological features of renal cell carcinoma (RCC) associated with Xp11.2 translocation/TFE3 gene fusions (Xp11.2 RCC) in our institution. We screened 983 RCC specimens. TFE3 immunohistochemical staining and FISH assay confirmed 22 Xp11.2 RCCs out of 65 suspicious cases. Clinicopathological and treatment outcomes of 22 patients were retrospectively analyzed. In total, 22 patients included 13 females and nine males with a mean age of 27 years. Ten patients showed gross hematuria. Treatments included surgeries, immunotherapy and molecular-targeted therapy. Seven cases were at stage III/IV and four cases had tumor thrombosis or distant metastasis. During a median follow-up of 34 months, 19 patients were alive while three died of distant metastasis. Xp11.2 RCC is rare and FISH proved a useful diagnostic tool. Surgical resection achieved favorable outcome for early disease. Adult patients at advanced stage had poorer outcomes even with postoperative adjuvant therapy.

Renal carcinomas associated with Xp11.2 translocations: are CT findings suggestive of the diagnosis?

PubMed

He, J; Huan, Y; Qiao, Q; Zhang, J; Zhang, J S

2014-01-01

The purpose of the present study was to summarize the computed tomography (CT) features of renal carcinomas associated with Xp11.2 translocations, and determine whether the diagnosis can be reliably deduced from imaging findings. Radiological studies of six patients (aged from 9-29 years) with renal carcinoma associated with Xp11.2 translocations were retrospectively analysed. The tumours varied in size from 3.3-11 cm (mean 5.4 cm). Unenhanced CT and cortical, medullary, and pelvic-phase contrast-enhanced CT imaging was undertaken in all cases. Unenhanced CT revealed that tumours had a relatively increased radiodensity (4/6, ranged from 45-60 HU) and suggested the possibility of diffuse haemorrhage. Three of the six cases showed irregular and boundary calcification of the lesion. Contrast-enhanced CT showed relatively well demarcated tumours with heterogeneous enhancement (6/6). Prolonged enhancement of tumours might be a common sign (6/6) in Xp11.2 translocations. Three out of the six cases were combined with retroperitoneal lymph nodes metastasis. Renal carcinomas associated with Xp11.2 translocations should be considered, particularly in children and young patients, when the lesion has calcification and is hyper-dense on unenhanced CT, and has prolonged enhancement on contrast-enhanced images. Copyright © 2013 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved.

Controlled phase stability of highly Na-active triclinic structure in nanoscale high-voltage Na2-2xCo1+xP2O7 cathode for Na-ion batteries

NASA Astrophysics Data System (ADS)

Song, Hee Jo; Kim, Jae-Chan; Dar, Mushtaq Ahmad; Kim, Dong-Wan

2018-02-01

With the increasing demand for high energy density in energy-storage systems, a high-voltage cathode is essential in rechargeable Li-ion and Na-ion batteries. The operating voltage of a triclinic-polymorph Na2CoP2O7, also known as the rose form, is above 4.0 V (vs. Na/Na+), which is relatively high compared to that of other cathode materials. Thus, it can be employed as a potential high-voltage cathode material in Na-ion batteries. However, it is difficult to synthesize a pure rose phase because of its low phase stability, thus limiting its use in high-voltage applications. Herein, compositional-engineered, rose-phase Na2-2xCo1+xP2O7/C (x = 0, 0.1 and 0.2) nanopowder are prepared using a wet-chemical method. The Na2-2xCo1+xP2O7/C cathode shows high electrochemical reactivity with Na ions at 4.0 V, delivering high capacity and high energy density.

Nucleotide Excision Repair Proteins Rapidly Accumulate but Fail to Persist in Human XP-E (DDB2 Mutant) Cells

PubMed Central

Oh, Kyu-Seon; Imoto, Kyoko; Emmert, Steffen; Tamura, Deborah; DiGiovanna, John J.; Kraemer, Kenneth. H.

2011-01-01

The XP-E DNA damage binding protein (DDB2) is involved in early recognition of global genome DNA damage during DNA nucleotide excision repair (NER). We found that skin fibroblasts from 4 newly reported XP-E patients with numerous skin cancers and DDB2 mutations had slow repair of 6-4 photoproducts (6-4PP) and markedly reduced repair of cyclobutane pyrimidine dimers (CPD). NER proteins (XPC, XPB, XPG, XPA, and XPF) co-localized to CPD and 6-4PP positive regions immediately (< 0.1h) after localized UV irradiation in cells from the XP-E patients and normal controls. While these proteins persist in normal cells, surprisingly, within 0.5h these repair proteins were no longer detectable at the sites of DNA damage in XP-E cells. Our results indicate that DDB2 is not required for the rapid recruitment of NER proteins to sites of UV photoproducts or for partial repair of 6-4PP but is essential for normal persistence of these proteins for CPD photoproduct removal. PMID:21388382

Ultrasonographic Findings of Renal Cell Carcinomas Associated with Xp11.2 Translocation/TFE3 Gene Fusion.

PubMed

Ling, Wenwu; Ma, Xuelei; Luo, Yan; Chen, Linyan; Wang, Huiyao; Wang, Xiaoling; Chen, Ni; Zeng, Hao; Li, Yongzhong; Cai, Diming

2017-01-01

This study was to investigate the features of renal carcinomas associated with Xp11.2 translocations/TFE3 gene fusions (Xp11.2-RCC) on conventional ultrasound (US) and contrast-enhanced ultrasound (CEUS). US and CEUS features of twenty-two cases with histopathologically proven Xp11.2-RCC were retrospectively reviewed. 22 patients (11 males, 11 females) were included in this study, with a mean age of 28.3 ± 20.4 years. Eight tumors (36.3%, 8/22) were in left kidney, and 14 tumors (63.7%, 14/22) were in right kidney. All tumors (100%, 22/22) were mixed echogenicity type. 13 tumors (59.1%, 13/22) presented small dotted calcifications. The boundary of 14 tumors (63.6%, 14/22) was sharp and the other 8 tumors' (36.4%, 8/22) boundary was blurry. By CEUS, in early phase, the solid element of all tumors showed obvious enhancement. In delayed phase, 13 tumors showed hypoenhancement, seven tumors showed isoenhancement, and 2 tumors showed hyperenhancement. There were irregular nonenhancement areas in all tumors inside. By US and CEUS, when children and adolescents were found to have hyperechoic mixed tumor in kidney with sharp margin and calcification, and the tumors showed obvious enhancement and hypoenhancement with irregular nonenhancement areas in the tumor in early phase and delayed phase, respectively, Xp11.2-RCC should be suspected.

Analysis of Extreme Phenotype Bulk Copy Number Variation (XP-CNV) Identified the Association of rp1 with Resistance to Goss's Wilt of Maize.

PubMed

Hu, Ying; Ren, Jie; Peng, Zhao; Umana, Arnoldo A; Le, Ha; Danilova, Tatiana; Fu, Junjie; Wang, Haiyan; Robertson, Alison; Hulbert, Scot H; White, Frank F; Liu, Sanzhen

2018-01-01

Goss's wilt (GW) of maize is caused by the Gram-positive bacterium Clavibacter michiganensis subsp. nebraskensis (Cmn) and has spread in recent years throughout the Great Plains, posing a threat to production. The genetic basis of plant resistance is unknown. Here, a simple method for quantifying disease symptoms was developed and used to select cohorts of highly resistant and highly susceptible lines known as extreme phenotypes (XP). Copy number variation (CNV) analyses using whole genome sequences of bulked XP revealed 141 genes containing CNV between the two XP groups. The CNV genes include the previously identified common rust resistant locus rp1 . Multiple Rp1 accessions with distinct rp1 haplotypes in an otherwise susceptible accession exhibited hypersensitive responses upon inoculation. GW provides an excellent system for the genetic dissection of diseases caused by closely related subspecies of C. michiganesis . Further work will facilitate breeding strategies to control GW and provide needed insight into the resistance mechanism of important related diseases such as bacterial canker of tomato and bacterial ring rot of potato.

Computed tomography and magnetic resonance imaging of adult renal cell carcinoma associated with Xp11.2 translocation.

PubMed

Dang, Trien T; Ziv, Etay; Weinstein, Stefanie; Meng, Maxwell V; Wang, Zhen; Coakley, Fergus V

2012-01-01

This study aimed to report the computed tomography (CT) and magnetic resonance imaging (MRI) findings of renal cell carcinoma associated with Xp11.2 translocation in adults. We retrospectively identified 9 adults with renal cell carcinoma associated with Xp11.2 translocation who underwent baseline cross-sectional imaging with CT (n = 9) or MRI (n = 3). All available clinical, imaging, and histopathological records were reviewed. Mean patient age was 24 years (range, 18-45 years). Eight of 9 cancers demonstrated imaging findings of hemorrhage or necrosis (n = 3), advanced stage disease (n = 2), or both (n = 3) at CT or MRI. The possibility of renal cell carcinoma associated with Xp11.2 translocation should be considered for a renal mass seen in a patient 45 years or younger, which demonstrates hemorrhage or necrosis or advanced stage disease at CT or MRI.

Ultrasonographic Findings of Renal Cell Carcinomas Associated with Xp11.2 Translocation/TFE3 Gene Fusion

PubMed Central

Zeng, Hao

2017-01-01

Objective This study was to investigate the features of renal carcinomas associated with Xp11.2 translocations/TFE3 gene fusions (Xp11.2-RCC) on conventional ultrasound (US) and contrast-enhanced ultrasound (CEUS). Methods US and CEUS features of twenty-two cases with histopathologically proven Xp11.2-RCC were retrospectively reviewed. Results 22 patients (11 males, 11 females) were included in this study, with a mean age of 28.3 ± 20.4 years. Eight tumors (36.3%, 8/22) were in left kidney, and 14 tumors (63.7%, 14/22) were in right kidney. All tumors (100%, 22/22) were mixed echogenicity type. 13 tumors (59.1%, 13/22) presented small dotted calcifications. The boundary of 14 tumors (63.6%, 14/22) was sharp and the other 8 tumors' (36.4%, 8/22) boundary was blurry. By CEUS, in early phase, the solid element of all tumors showed obvious enhancement. In delayed phase, 13 tumors showed hypoenhancement, seven tumors showed isoenhancement, and 2 tumors showed hyperenhancement. There were irregular nonenhancement areas in all tumors inside. Conclusions By US and CEUS, when children and adolescents were found to have hyperechoic mixed tumor in kidney with sharp margin and calcification, and the tumors showed obvious enhancement and hypoenhancement with irregular nonenhancement areas in the tumor in early phase and delayed phase, respectively, Xp11.2-RCC should be suspected. PMID:29333109

A novel Xp22.13 microdeletion in Nance-Horan syndrome.

PubMed

Accogli, Andrea; Traverso, Monica; Madia, Francesca; Bellini, Tommaso; Vari, Maria Stella; Pinto, Francesca; Capra, Valeria

2017-07-03

Nance-Horan syndrome (NHS) is a rare X-linked developmental disorder characterized by congenital cataract, dental anomalies and facial dysmorphisms. Notably, up to 30% of NHS patients have intellectual disability and a few patients have been reported to have congenital cardiac defects. Nance-Horan syndrome is caused by mutations in the NHS gene that is highly expressed in the midbrain, retina, lens, tooth, and is conserved across vertebrate species. Although most pathogenic mutations are nonsense mutations, a few genomic rearrangements involving NHS locus have been reported, suggesting a possible pathogenic role of the flanking genes. Here, we report a microdeletion of 170,6 Kb at Xp22.13 (17.733.948-17.904.576) (GRCh37/hg19), detected by array-based comparative genomic hybridization in an Italian boy with NHS syndrome. The microdeletion harbors the NHS, SCLML1, and RAI2 genes and results in a phenotype consistent with NSH syndrome and developmental delay. We compare our case with the previous Xp22.13 microdeletions and discuss the possible pathogenetic role of the flanking genes. Birth Defects Research 109:866-868, 2017. © 2017 Wiley Periodicals, Inc. © 2017 Wiley Periodicals, Inc.

Usefulness of a break-apart FISH assay in the diagnosis of Xp11.2 translocation renal cell carcinoma.

PubMed

Kim, Soo Hee; Choi, Yoomi; Jeong, Hae Yeon; Lee, Kyoungbun; Chae, Ji Youn; Moon, Kyung Chul

2011-09-01

Xp11.2 translocation renal cell carcinoma (RCC) is a rare subtype of RCC predominantly reported in young patients. It results from gene fusions between the transcription factor E3 (TFE3) gene, which is located on chromosome Xp11.2, and various fusion partners. Recently, a dual color, break-apart fluorescence in situ hybridization (FISH) assay to detect Xp11.2 translocation was reported. We performed this study to evaluate the usefulness of the FISH assay in the diagnosis of Xp11.2 translocation RCC using a commercially available TFE3 break-apart probe. We immunohistochemically analyzed TFE3 nuclear expression in 809 cases of RCCs using 14 tissue microarray blocks and selected nine cases those showed moderate to strong positive nuclear immunoreactivity for TFE3. The extent of TFE3 nuclear expression was variable. The TFE3 FISH assay was performed in these 9 selected cases and 44 negative control cases. Only four out of nine selected cases showed the TFE3 break-apart signal. TFE3 FISH-positive cases mainly showed diffuse and strong TFE3 immunopositivity, but one case revealed focal and moderate TFE3 staining. On the contrary, TFE3 FISH-negative cases mainly revealed focal and moderate TFE3 immunoreactivity, however, one FISH-negative case revealed diffuse and strong TFE3 nuclear immunopositivity. All negative control cases revealed normal TFE3 FISH results. Our results reveal that TFE3 immunohistochemistry can show false-positive results, and that the TFE3 break-apart FISH assay is a useful complementary method for confirming the diagnosis of Xp11.2 translocation RCC.

A case of metastatic Xp11.2 translocation renal cell carcinoma successfully managed by cytoreductive nephrectomy followed by axitinib therapy.

PubMed

Nishimura, Koichi; Takagi, Toshio; Toda, Naohiro; Yamamoto, Tomoko; Kondo, Tsunenori; Ishida, Hideki; Nagashima, Yoji; Tanabe, Kazunari

2017-03-01

Targeted medications for metastatic adult Xp11.2 translocation renal cell carcinoma (RCC) remain to be identified. We herein report a case of metastatic Xp11.2 translocation RCC controlled with cytoreductive nephrectomy (CN) and axitinib therapy. A 57-year-old woman complained of fatigue and low back pain. Imaging studies revealed a right renal tumor, with multiple lung and mediastinal lymph node metastases. Although the patient received 10 mg axitinib therapy for 5 months at the hospital she was initially admitted to, the size of the primary and metastatic lesions was not reduced. Thus, she was referred to the Tokyo Women's Medical University Hospital (Tokyo, Japan) for further treatment, where she underwent CN. On macroscopic examination, almost the entire kidney was replaced by a yellowish brown tumor >80 mm in diameter. Immunohistochemical examination confirmed the diagnosis of Xp11.2 translocation RCC. One month after surgery, axitinib therapy was resumed and the size of the metastatic lesions gradually decreased. These findings suggest that axitinib therapy is effective for adult Xp11.2 translocation RCC.

A case of metastatic Xp11.2 translocation renal cell carcinoma successfully managed by cytoreductive nephrectomy followed by axitinib therapy

PubMed Central

Nishimura, Koichi; Takagi, Toshio; Toda, Naohiro; Yamamoto, Tomoko; Kondo, Tsunenori; Ishida, Hideki; Nagashima, Yoji; Tanabe, Kazunari

2017-01-01

Targeted medications for metastatic adult Xp11.2 translocation renal cell carcinoma (RCC) remain to be identified. We herein report a case of metastatic Xp11.2 translocation RCC controlled with cytoreductive nephrectomy (CN) and axitinib therapy. A 57-year-old woman complained of fatigue and low back pain. Imaging studies revealed a right renal tumor, with multiple lung and mediastinal lymph node metastases. Although the patient received 10 mg axitinib therapy for 5 months at the hospital she was initially admitted to, the size of the primary and metastatic lesions was not reduced. Thus, she was referred to the Tokyo Women's Medical University Hospital (Tokyo, Japan) for further treatment, where she underwent CN. On macroscopic examination, almost the entire kidney was replaced by a yellowish brown tumor >80 mm in diameter. Immunohistochemical examination confirmed the diagnosis of Xp11.2 translocation RCC. One month after surgery, axitinib therapy was resumed and the size of the metastatic lesions gradually decreased. These findings suggest that axitinib therapy is effective for adult Xp11.2 translocation RCC. PMID:28451413

Spin and Recovery Characteristics of the Curtiss-Wright XP-87 Airplane

NASA Technical Reports Server (NTRS)

Berman, Theodore

1947-01-01

The spin and recovery characteristics of the Curtiss-Wright XP-87 airplane, as well as the spin-recovery parachute requirements, the control forces that would be encountered in the spin, and the best method for the crew to attempt an emergency escape, are presented in this report. The characteristics were estimated rather than determined by model tests because the XP-87 dimensional and mass characteristics were considered to be noncritical and because data were available from model tests of several similar airplanes. The study indicated that the recovery characteristics of the airplane will be satisfactory for all loadings if the controls are reversed fully and rapidly. The control forces, however, will probably be beyond the capabilities of the pilot unless some additional balance or a booster is used. A 6-foot tail parachute or a 3.5-foot wing-tip parachute with a drag coefficient of 0.7 will be a satisfactory, emergency spin-recovery device for spin demonstrations. If it is necessary for the crew to abandon the spinning airplane, they should leave from the outboard side of the cockpit.

Structural, thermal, magnetic, and electronic transport properties of the LaNi₂(Ge 1-xP x)₂ system

DOE PAGES

Goetsch, R. J.; Anand, V. K.; Pandey, Abhishek; ...

2012-02-29

Polycrystalline samples of LaNi₂(Ge 1-xP x)₂ (x=0,0.25,0.50,0.75,1) were synthesized and their properties investigated by x-ray diffraction (XRD) measurements at room temperature and by heat capacity C p, magnetic susceptibility χ, and electrical resistivity ρ measurements versus temperature T from 1.8 to 350 K. Rietveld refinements of powder XRD patterns confirm that these compounds crystallize in the body-centered-tetragonal ThCr₂Si₂-type structure (space group I4/mmm) with composition-dependent lattice parameters that slightly deviate from Vegard's law. The ρ(T) measurements showed a positive temperature coefficient for all samples from 1.8 to 300 K, indicating that all compositions in this system are metallic. The low-T Cmore » p measurements yield a rather large Sommerfeld electronic specific heat coefficient γ=12.4(2) mJ/mol K² for x=0, reflecting a large density of states at the Fermi energy that is comparable with the largest values found for the AFe₂As₂ class of materials with the same crystal structure. The γ decreases approximately linearly with x to 7.4(1) mJ/mol K² for x=1. The χ measurements show nearly temperature-independent paramagnetic behavior across the entire range of compositions except for LaNi₂Ge₂, where a broad peak is observed at ≈300 K from χ(T) measurements up to 1000 K that may arise from short-range antiferromagnetic correlations in a quasi-two-dimensional magnetic system. High-accuracy Padé approximants representing the Debye lattice heat capacity and Bloch-Grüneisen electron-phonon resistivity functions versus T are presented and are used to analyze our experimental C p(T) and ρ(T) data, respectively, for 1.8K≤T≤300 K. The T dependences of ρ for all samples are well-described over this T range by the Bloch-Grüneisen model, although the observed ρ(300 K) values are larger than calculated from this model. A significant T dependence of the Debye temperature determined from the C p(T) data was

Xp11.2 translocation tumor: a rare cause of gross hematuria.

PubMed

Asaki, Howard E; Moshero, Gianni; Stanton, Melissa L; Humphreys, Mitchell R

2014-02-01

Xp11.2 translocation tumor is a rare but aggressive form of renal cell carcinoma that predominantly occurs in children but also may be found in young adults. Because this type of cancer is diagnosed via histologic and chromosomal analysis, clinicians should consider translocation tumor in the differential diagnosis of patients with renal lesions and gross hematuria.

Effect of Pressure on Some Optical Properties of GaxIn1-xP Semiconductors

NASA Astrophysics Data System (ADS)

Vyas, P.; Gajjar, P.; Jani, A.

2013-06-01

A theoretical procedure is presented for the study of optical properties of ternary alloy GaxIn1-xP. The calculations are based on the pseudopotential formalism in which local potential coupled with the virtual crystal approximation (VCA) is applied to evaluate the effect of pressure on the optical properties like refractive index, electronic polarizability, plasmon energy, dielectric constant and equation of state for gallium concentration x = 0, 0.25, 0.50, 0.75 and 1 of the ternary alloy GaxIn1-xP. To incorporate the screening effect, local field correction functions due to Hartree, Taylor, Ichimaru et al. and Nagy are employed. The refractive index, electronic polarizability and dielectric constant computed for the parent binary compounds GaP and InP are found to be satisfactorily agreeing with the experimental report. It is seen that the refractive index of GaxIn1-xP decreases nonlinearly with the increase in pressure. The results obtained using Hartree's screening functions are not very close to the experimental data as it does not include any exchange and correlation effects. Overall good agreement with the experimental and other theoretical findings confirms the application. The author P. S. Vyas is thankful to UGC, New Delhi, India for providing financial support under minor research project No. F.: 47-651/08(WRO).

[Differential diagnosis between renal cell carcinoma associated with XP11.2 translocation/TFE gene fusion and papillary renal cell carcinoma based on CT and MRI findings].

PubMed

Zhu, Qingqiang; Zhu, Wenrong; Wu, Jingtao; Fu, Jianxiong; Chen, Wenxin; Wang, Zhongqiu

2014-05-20

To comparative study of CT and MRI appearances in renal cell carcinoma associated with XP11.2 translocation/TFE gene fusion (XP11.2 RCC) and papillary renal cell carcinoma (PRCC). 12 patients with XP11.2 RCC and 18 patients with PRCC were retrospectively studied, and the data was analyzed by AVONA and chi-square text. 12 patients with XP11.2 RCC and 18 patients with PRCC, cystic components (2 vs 11, P < 0.05), calcification (0 vs 6, P < 0.05), hemorrhage (9 vs 5, P < 0.05), homogeneous enhancement (10 vs 7, P < 0.05) and had lymph node (3 vs 0) or hepatic metastasis (1vs 0) (P < 0.05). On unenhanced CT, the density of XP11.2 RCC was greater than PRCC, normal renal cortex or medulla (P < 0.05). Their degree of enhancement were less than normal renal cortex on all enhanced phases (P < 0.05). The enhancement degree of XP11.2 RCC was higher than PRCC (on all phases) and renal medulla (on cortical and medullary phase) (P < 0.05), but less than normal renal medulla on the delayed phase (P < 0.05). The enhancement degree of PRCC was lower than renal medulla on all phases (P < 0.05). The XP11.2 RCC was isointense on T1-weighted imaging, hypointense on T2-weighted imaging. The PRCC was isointense or hypointense on T1-weighted imaging, isointense on T2-weighted imaging. The CT and MRI could show imagings features of XP11.2 RCC and PRCC, and these features were helpful in predicting a specific subtype of renal cell carcinoma.

New X-linked mental retardation syndrome with the gene mapped tentatively in Xp22.3

DOE Office of Scientific and Technical Information (OSTI.GOV)

Wittwer, B.; Kircheisen, R.; Leutelt, J.

1996-07-12

X-linked mental retardation (XLMR) is genetically heterogeneous and clinically variable. We describe a new XLMR syndrome of severe mental retardation and multiple congenital anomalies. Two sisters have (with 3 different partners) 3 severely handicapped sons. In 2 cases, oligohydramnios and intrauterine growth retardation were noted. Common anomalies included a square-shaped face, high and broad forehead, frontal bossing, downward slant of palpebral fissures, hypertelorism, epicanthic folds, long philtrum, thin upper lip, and apparently low-set ears. One boy has bilateral microphthalmos and sclerocornea, and his cousin has atrophy of the optic nerve. All 3 patients are blind and have profound statomotor andmore » mental retardation, seizures, and a grossly abnormal electroencephalographic pattern. Additional findings are short stature, delayed bone matuation, hydronephrosis, vesicorenal reflux, cryptorchidism, clinodactyly of the 5th fingers, and transverse palmar creases. The karyotype is normal (46,XY). Segregation analysis showed perfect coinheritance between the clinical phenotype and alleles at several loci in Xp22.3, whereas recombinants were identified with marker loci from Xp22.2-qter. Analysis of multiple informative meioses suggests that the disease locus maps in Xp22.3 distal to DXS16. 9 refs., 5 figs., 2 tabs.« less

Structural and magnetic phase transitions near optimal superconductivity in BaFe 2(As 1-xP x) 2

DOE PAGES

Hu, Ding; Lu, Xingye; Zhang, Wenliang; ...

2015-04-17

In this study, we use nuclear magnetic resonance (NMR), high-resolution x-ray and neutron scattering to study structural and magnetic phase transitions in phosphorus-doped BaFe 2(As 1-xP x) 2. Thus, previous transport, NMR, specific heat, and magnetic penetration depth measurements have provided compelling evidence for the presence of a quantum critical point (QCP) near optimal superconductivity at x = 0.3. However, we show that the tetragonal-to-orthorhombic structural (T s) and paramagnetic to antiferromagnetic (AF, T N) transitions in BaFe 2(As 1-xP x) 2 are always coupled and approach to T N ≈ T s ≥ T c (≈ 29 K) formore » x = 0.29 before vanishing abruptly for x ≥ 0.3. These results suggest that AF order in BaFe 2(As 1-xP x) 2 disappears in a weakly first order fashion near optimal superconductivity, much like the electron-doped iron pnictides with an avoided QCP.« less

Dataset of proinflammatory cytokine and cytokine receptor gene expression in rainbow trout (Oncorhynchus mykiss) measured using a novel GeXP multiplex, RT-PCR assay

USDA-ARS?s Scientific Manuscript database

A GeXP multiplex, RT-PCR assay was developed and optimized that simultaneously measures expression of a suite of immune-relevant genes in rainbow trout (Oncorhynchus mykiss), concentrating on tumor necrosis factor and interleukin-1 ligand/receptor systems and acute phase response genes. The dataset ...

Large inverted repeats within Xp11.2 are present at the breakpoints of isodicentric X chromosomes in Turner syndrome.

PubMed

Scott, Stuart A; Cohen, Ninette; Brandt, Tracy; Warburton, Peter E; Edelmann, Lisa

2010-09-01

Turner syndrome (TS) results from whole or partial monosomy X and is mediated by haploinsufficiency of genes that normally escape X-inactivation. Although a 45,X karyotype is observed in half of all TS cases, the most frequent variant TS karyotype includes the isodicentric X chromosome alone [46,X,idic(X)(p11)] or as a mosaic [46,X,idic(X)(p11)/45,X]. Given the mechanism of idic(X)(p11) rearrangement is poorly understood and breakpoint sequence information is unknown, this study sought to investigate the molecular mechanism of idic(X)(p11) formation by determining their precise breakpoint intervals. Karyotype analysis and fluorescence in situ hybridization mapping of eight idic(X)(p11) cell lines and three unbalanced Xp11.2 translocation lines identified the majority of breakpoints within a 5 Mb region, from approximately 53 to 58 Mb, in Xp11.1-p11.22, clustering into four regions. To further refine the breakpoints, a high-resolution oligonucleotide microarray (average of approximately 350 bp) was designed and array-based comparative genomic hybridization (aCGH) was performed on all 11 idic(X)(p11) and Xp11.2 translocation lines. aCGH analyses identified all breakpoint regions, including an idic(X)(p11) line with two potential breakpoints, one breakpoint shared between two idic(X)(p11) lines and two Xp translocations that shared breakpoints with idic(X)(p11) lines. Four of the breakpoint regions included large inverted repeats composed of repetitive gene clusters and segmental duplications, which corresponded to regions of copy-number variation. These data indicate that the rearrangement sites on Xp11.2 that lead to isodicentric chromosome formation and translocations are probably not random and suggest that the complex repetitive architecture of this region predisposes it to rearrangements, some of which are recurrent.

[MRI findings of renal cell carcinoma associated with Xp11.2 translocations/TFE3 gene fusions].

PubMed

Zhong, Y; Wang, H Y; Chen, X; Guo, A T; Ma, L; Wang, Y W; Ye, H Y

2016-09-06

Objective: To analyze MRI findings of renal cell carcinoma associated with Xp11.2 translocation-TFE gene fusion(Xp11.2 RCC). Methods: MR imaging features of eleven patients with pathologically-proved Xp11.2 RCC were retrospectively analyzed from December 2008 to December 2015. The following MRI features of the lesions were analyzed in the study: location, maximal diameter, signal intensity, hemorrhage, necrosis, cystic change, enhancement features and metastasis. The data was analyzed by using t test. Results: Four men and seven women (mean age, 35.2 years; age range, 15-49 years) were included. Tumors occurred in the right kidney in 5 cases and the left kidney in 6 cases. On T 1 WI tumors showed heterogeneously hypo-intensity and iso-intensity, hyper-intensity in 10 cases, 1 cases, respectively. On T 2 WI tumors showed heterogeneously slight hypo-intensity, heterogeneously slight hyper-intensity and hyper-intensity in 6 cases, 4 cases, 1 case, respectively. On DWI tumors showed hyper-intensity and heterogeneously slight hype-intensity in 2 cases, 9 cases, respectively. ADC value of the tumors were statistically significant lower than that of renal cortex(×10 -3 mm 2 /s)(1.35±0.20 vs 2.09±0.11, P <0.05). Imaging findings were suggestive of hemorrhage( n =4) or necrosis ( n =1) or cystic change ( n =6) or lipid( n =1) in the tumors. On dynamic contrast-enhanced imaging, tumors showed lower signal intensity change (96%±93%, 110%±86% and 103%±46%, respectively) than did renal cortex (285%±109%, 254%±97% and 225%±90%, respectively) ( P <0.05). Tumor capsule showed in 7 cases. Enlarged lymph node was found in renal hilum in one case. Conclusion: MRI findings may show characteristic features of Xp11.2 RCC combined with patients' age and assist in preoperative correct diagnosis.

Renal Cell Carcinoma Associated with Xp11.2 Translocation/TFE3 Gene Fusions: Clinical Features, Treatments and Prognosis.

PubMed

Liu, Ning; Wang, Zhen; Gan, Weidong; Xiong, Lei; Miao, Baolei; Chen, Xiancheng; Guo, Hongqian; Li, Dongmei

2016-01-01

To investigate the clinical characteristics, treatments and prognosis of renal cell carcinoma associated with Xp11.2 translocation/TFE3 gene fusions (Xp11.2 tRCC), the epidemiological features and treatment results of 34 cases of Xp11.2 tRCC, which were diagnosed by immunohistochemistry staining of TFE3 and fluorescence in situ hybridization at our center, were retrospectively reviewed. The 34 patients included 21 females and 13 males aged 3 to 64 years (median age: 27 years). Four patients were children or adolescents (<18 years of age), and 26 patients were young or middle-aged adults (18-45 years). Radical nephrectomy was performed on 25 patients. Laparoscopic nephron-sparing surgery was performed on 9 patients who presented with an isolated mass with a small diameter (<7 cm) and well-defined boundary on computed tomography imaging. Postoperative staging showed that 25 cases (73.53%) were at stage I/II, while 9 cases (26.47%) were at stage III/IV. All stage I/II patients received a favorable prognosis with a three-year overall survival rate of 100%, including the patients who underwent laparoscopic nephron-sparing surgery. With the exception of 2 children, the other 7 stage III/IV patients died or developed recurrence with a median follow-up of 29 months. On univariate analysis, maximum diameter, adjuvant treatment, TNM stage, lymph node metastasis, inferior vena cava tumor thrombosis and tumor boundary were identified as statistically significant factors impacting survival (P<0.05). Multivariate analysis indicated that TNM stage and inferior vena cava tumor thrombosis were independent prognostic factors (P<0.05). In conclusion, Xp11.2 tRCC is a rare subtype of renal cell carcinoma that mainly occurs in young females. Nephron-sparing surgery was confirmed effective preliminarily in the treatment of small Xp11.2 tRCCs with clear rims. Advanced TNM stage and inferior vena cava tumor thrombosis were associated with poor prognosis.

Renal Cell Carcinoma Associated with Xp11.2 Translocation/TFE3 Gene Fusions: Clinical Features, Treatments and Prognosis

PubMed Central

Gan, Weidong; Xiong, Lei; Miao, Baolei; Chen, Xiancheng; Guo, Hongqian; Li, Dongmei

2016-01-01

To investigate the clinical characteristics, treatments and prognosis of renal cell carcinoma associated with Xp11.2 translocation/TFE3 gene fusions (Xp11.2 tRCC), the epidemiological features and treatment results of 34 cases of Xp11.2 tRCC, which were diagnosed by immunohistochemistry staining of TFE3 and fluorescence in situ hybridization at our center, were retrospectively reviewed. The 34 patients included 21 females and 13 males aged 3 to 64 years (median age: 27 years). Four patients were children or adolescents (<18 years of age), and 26 patients were young or middle-aged adults (18–45 years). Radical nephrectomy was performed on 25 patients. Laparoscopic nephron-sparing surgery was performed on 9 patients who presented with an isolated mass with a small diameter (<7 cm) and well-defined boundary on computed tomography imaging. Postoperative staging showed that 25 cases (73.53%) were at stage I/II, while 9 cases (26.47%) were at stage III/IV. All stage I/II patients received a favorable prognosis with a three-year overall survival rate of 100%, including the patients who underwent laparoscopic nephron-sparing surgery. With the exception of 2 children, the other 7 stage III/IV patients died or developed recurrence with a median follow-up of 29 months. On univariate analysis, maximum diameter, adjuvant treatment, TNM stage, lymph node metastasis, inferior vena cava tumor thrombosis and tumor boundary were identified as statistically significant factors impacting survival (P<0.05). Multivariate analysis indicated that TNM stage and inferior vena cava tumor thrombosis were independent prognostic factors (P<0.05). In conclusion, Xp11.2 tRCC is a rare subtype of renal cell carcinoma that mainly occurs in young females. Nephron-sparing surgery was confirmed effective preliminarily in the treatment of small Xp11.2 tRCCs with clear rims. Advanced TNM stage and inferior vena cava tumor thrombosis were associated with poor prognosis. PMID:27893792

Selective mass enhancement close to the quantum critical point in BaFe 2(As 1-xP x) 2

DOE PAGES

Grinenko, V.; Iida, K.; Kurth, F.; ...

2017-07-04

A quantum critical point (QCP) is currently being conjectured for the BaFe 2(As 1-xP x) 2 system at the critical value x c ≈ 0.3. In the proximity of a QCP, all thermodynamic and transport properties are expected to scale with a single characteristic energy, given by the quantum fluctuations. Such a universal behavior has not, however, been found in the superconducting upper critical field H c2. Here we report H c2 data for epitaxial thin films extracted from the electrical resistance measured in very high magnetic fields up to 67 Tesla. Using a multi-band analysis we find that Hmore » c2 is sensitive to the QCP, implying a significant charge carrier effective mass enhancement at the doping-induced QCP that is essentially band-dependent. Our results point to two qualitatively different groups of electrons in BaFe 2(As 1-xP x) 2. The first one (possibly associated to hot spots or whole Fermi sheets) has a strong mass enhancement at the QCP, and the second one is insensitive to the QCP. The observed duality could also be present in many other quantum critical systems.« less

Report: Passaic Valley Sewerage Commissioners – Unallowable Costs Claimed Under EPA Grant XP98237601

EPA Pesticide Factsheets

Report #08-2-0226, August 6, 2008. The grantee claimed $2,385,634 for pre-award costs under Grant XP98237601 that were incurred prior to the grant award and thus were unallowable under the grant administrative conditions and OMB Circular A-87.

Review of renal carcinoma associated with Xp11.2 translocations/TFE3 gene fusions with focus on pathobiological aspect.

PubMed

Kuroda, Naoto; Mikami, Shuji; Pan, Chin-Chen; Cohen, Ronald J; Hes, Ondrej; Michal, Michal; Nagashima, Yoji; Tanaka, Yukichi; Inoue, Keiji; Shuin, Taro; Lee, Gang-Hong

2012-02-01

The concept of Xp11.2 renal cell carcinoma (RCC) was recently established as a tumor affecting 15% of RCC patients <45 years. Many patients present with advanced stage with frequent lymph node metastases. Histologically, Xp11.2 RCC is characterized by mixed papillary nested/alveolar growth pattern and tumor cells with clear and/or eosinophilic, voluminous cytoplasm. Neoplastic cells show intense nuclear immunoreactivity to TFE3, while focal immunostaining for melanocytic markers, including melanosome-associated antigen or Melan A in some cases, are also noted. Alpha smooth muscle actin and TFEB are consistently negative. Ultrastructurally, the ASPL-TFE3 RCC variant contains rhomboid crystals in the cytoplasm, similar to that observed in alveolar soft part sarcoma. The fusion of the TFE3 gene with several different genes, including ASPL(17q25), PRCC(1q21), PSF(1q34), NonO (Xq12) and CLTC (17q23) have been identified to date. The behavior of Xp11.2 RCC in children and young adults is considered as indolent even when diagnosed at advanced stage, including lymph node metastasis. However, Xp11.2 RCC in older patients behaves in a more aggressive fashion. Therapy includes nephrectomy with extended lymphadenectomy. There may be a role for new protease inhibitors in advanced inoperable disease. Further research is required to correlate clinical behavior with the expanding genetic spectrum of this tumor, and to establish standard therapy protocols for primary and metastatic lesions.

Deployable Command and Control System for Over the Horizon Small Boat Operations

DTIC Science & Technology

2006-09-01

the HP iPAQ Navigation System bundle. There is no programmable Application Programming Interface (API), nor otherwise accessible methods to ...High Point Software which comes complete with a C# library to allow customized programs to access Bluetooth enabled GPS devices. GPSAccess...data could be displayed along with ownship’s positional data, but the program was designed to only work with the Ross radios and the MS Windows XP

Autism in Two Females with Duplications Involving Xp11.22-p11.23

ERIC Educational Resources Information Center

Edens, Anna C.; Lyons, Michael J.; Duron, Reyna M.; DuPont, Barbara R.; Holden, Kenton R.

2011-01-01

We present two phenotypically similar females with Xp duplication who have autism and epilepsy. Case 1 is a 14-year-old Honduran female with autism and medically refractory complex partial, secondarily generalized epilepsy. Case 2 is a 3-year-old Austrian female with autism and medically refractory complex partial epilepsy. Both patients also…

Unassisted Water Splitting Using a GaSb xP (1- x ) Photoanode

DOE PAGES

Martinez-Garcia, Alejandro; Russell, Harry B.; Paxton, William; ...

2018-02-21

Here in this work, unbiased water splitting with 2% solar-to-hydrogen efficiency under AM 1.5 G illumination using new materials based on GaSb 0.03P 0.97 alloy is reported. Freestanding GaSb xP 1-x is grown using halide vapor phase epitaxy. The native conductivity type of the alloy is modified by silicon doping, resulting in an open-circuit potential (OCP) of 750 mV, photocurrents of 7 mA cm -2 at 10 sun illumination, and corrosion resistance in an aqueous acidic environment. Alloying GaP with Sb at 3 at% improves the absorption of high-energy photons above 2.68 eV compared to pure GaP material. Electrochemical Impedancemore » Spectroscopy and illuminated OCP measurements show that the conduction band of GaSb xP 1-x is at -0.55 V versus RHE irrespective of the Sb concentration, while photocurrent spectroscopy indicates that only radiation with photon energies greater than 2.68 eV generate mobile and extractable charges, thus suggesting that the higher-laying conduction bands in the Γ 1 valley of the alloys are responsible for exciton generation.« less

Unassisted Water Splitting Using a GaSb xP (1- x ) Photoanode

DOE Office of Scientific and Technical Information (OSTI.GOV)

Martinez-Garcia, Alejandro; Russell, Harry B.; Paxton, William

Here in this work, unbiased water splitting with 2% solar-to-hydrogen efficiency under AM 1.5 G illumination using new materials based on GaSb 0.03P 0.97 alloy is reported. Freestanding GaSb xP 1-x is grown using halide vapor phase epitaxy. The native conductivity type of the alloy is modified by silicon doping, resulting in an open-circuit potential (OCP) of 750 mV, photocurrents of 7 mA cm -2 at 10 sun illumination, and corrosion resistance in an aqueous acidic environment. Alloying GaP with Sb at 3 at% improves the absorption of high-energy photons above 2.68 eV compared to pure GaP material. Electrochemical Impedancemore » Spectroscopy and illuminated OCP measurements show that the conduction band of GaSb xP 1-x is at -0.55 V versus RHE irrespective of the Sb concentration, while photocurrent spectroscopy indicates that only radiation with photon energies greater than 2.68 eV generate mobile and extractable charges, thus suggesting that the higher-laying conduction bands in the Γ 1 valley of the alloys are responsible for exciton generation.« less

Development of a YAC contig covering the minimal region of a CSNB1 locus in Xp11

DOE Office of Scientific and Technical Information (OSTI.GOV)

Boycott, K.M.; Gratton, K.J.; Moore, B.J.

1994-09-01

X-linked congenital stationary night blindness (CSNB1) is an eye disorder that includes impairment of night vision, reduced visual acuity and, in some cases, myopia and congenital nystagmus. Electroretinography reveals a marked reduction of the b-wave in affected individuals suggesting that X-linked CSNB is due to a molecular defect in the bipolar layer of the retina. Based on our studies of a large four generation family with X-linked CSNB, a CSNB1 locus was mapped to a 4-5 cM region at Xp11.23-Xp11.22 bounded telomerically by DXS426 and centromerically by DXS988. Using a panel of radiation and conventional somatic cell hybrids, a detailedmore » map of new and published STSs has been generated for the minimal region of CSNB1. PCR primer pairs for STSs has been generated for the minimal region of CSNB1. PCR primer pairs for twenty-five STSs, including eleven end-clones, were used to isolate YAC clones from CEPH, mega-CEPH, and X chromosome-specific YAC libraries. In total, fifty-two YACs were characterized for STS overlaps and assembled to provide a minimum of 3 Mb of physical coverage in the region between DXS426 and DXS988. Five gaps proximal to SYP are still to be closed. Our physical map suggests the following gene order: Xpter-OTAL1-GF1-DXS1011E-MG81-HUMCRAS2P-SYP-Xcen. STS analysis of the YACs revealed three subregions of the physical map which appear to be particularly susceptible to internal deletions and end-clone analysis demonstrated chimerism in six of seventeen YACs. A physical map of Xp11.23-Xp11.22 will provide a resource for the isolation of candidate genes for the X-linked CSNB gene which maps to this region.« less

Symmetry-Breaking Transitions in RECuAs 2-xP x (RE=Sm, Gd, Ho, and Er)

DOE Office of Scientific and Technical Information (OSTI.GOV)

Mozharivskyj, Yurij

Structural changes resulting in lower symmetries can be understood in terms of electronic instabilities and Coulomb interactions. The interplay of these two interrelated factors is complicated and difficult to analyze. The RECuAs 2-xP x phases, because of the variation in the chemical content (As/P substitution), allow, with the aid of band structures, Madelung energies and Landau theory, a partial unraveling of the forces important in the symmetry-breaking transitions in RECuAs 2-xP x (RE = Sm, Gd, Ho and Er). Distortions of the P layers in SmCu 1.15P 2, GdCuP 2.20 and ErCuP 2 are usefully thought of asmore » generalized Peierls distortions, i.e., they lower the electronic (and total) energy and lead to more stable structures. On the other hand, the P4/nmm → Pmmn transitions, which are observed in all studied arsenophosphide series and occur upon substitution of P for As, originate from the B1g vibrational mode and are structural adaptations to smaller P atoms. These transitions provide tighter atomic packing and better Coulomb interactions. Configurational contribution to the entropy becomes important in stabilizing the mixed occupancy in the RECuAs 2-xP x arsenophosphides. While geometric and electronic factors favor separation of the As and P atoms over two different crystallographic sites, configurational entropy stabilizes the As/P mixing on these two sites.;Progress in the research on RECuAs 2-xP x was dependent upon the ability of Landau theory to predict, explain and dismiss structural models and transitions. The space group Pmmn (arising from the B 1g vibrational mode) in all mixed arsenophosphides and the existence of these mixed arsenophosphides followed from the analysis of GdCuAs 2 and GdCuP 2, using Landau theory. The impossibility of obtaining the high-symmetry structure (P4/nmm) and the low symmetry structure (Pnmm) at the same temperature for the displacive continuous symmetry-breaking transition P4/ nmm → Pmmn led to the conclusion, later

The ClpXP protease is responsible for the degradation of the Epsilon antidote to the Zeta toxin of the streptococcal pSM19035 plasmid.

PubMed

Brzozowska, Iwona; Zielenkiewicz, Urszula

2014-03-14

Most bacterial genomes contain different types of toxin-antitoxin (TA) systems. The ω-ε-ζ proteinaceous type II TA cassette from the streptococcal pSM19035 plasmid is a member of the ε/ζ family, which is commonly found in multiresistance plasmids and chromosomes of various human pathogens. Regulation of type II TA systems relies on the proteolysis of antitoxin proteins. Under normal conditions, the Epsilon antidote neutralizes the Zeta toxin through the formation of a tight complex. In this study, we show, using both in vivo and in vitro analyses, that the ClpXP protease is responsible for Epsilon antitoxin degradation. Using in vivo studies, we examined the stability of the plasmids with active or inactive ω-ε-ζ TA cassettes in B. subtilis mutants that were defective for different proteases. Using in vitro assays, the degradation of purified His6-Epsilon by the His6-LonBs, ClpPBs, and ClpXBs proteases from B. subtilis was analyzed. Additionally, we showed that purified Zeta toxin protects the Epsilon protein from rapid ClpXP-catalyzed degradation.

Vascular endothelial growth factor (VEGF)-targeted therapy for the treatment of adult metastatic Xp11.2 translocation renal cell carcinoma

PubMed Central

Choueiri, Toni K.; Lim, Zita Dubauskas; Hirsch, Michelle S.; Tamboli, Pheroze; Jonasch, Eric; McDermott, David F.; Cin, Paola Dal; Corn, Paul; Vaishampayan, Ulka; Heng, Daniel Y.C.; Tannir, Nizar M.

2015-01-01

Introduction Adult “translocation” renal cell carcinoma (RCC), bearing TFE3 gene fusions at Xp11.2, is a recently recognized unique entity for which prognosis and therapy remain poorly understood. We investigated the effect of vascular-endothelial growth factor (VEGF)-targeted therapy in this distinct subtype of RCC. Patients and Methods We conducted a retrospective review to describe the clinical characteristics and outcome of adult patients with metastatic Xp11.2 RCC, who had strong TFE-3 nuclear immunostaining, and received anti-VEGF therapy. Tumor response to anti-VEGF therapy was evaluated by RECIST. Kaplan-Meier methods were used to estimate progression-free survival (PFS) and overall survival (OS) distributions. Results Fifteen patients were identified of which 10, 3, and 2 received sunitinib, sorafenib and monoclonal anti-VEGF antibodies, respectively. The median follow-up was 19.1 months, the median age of the patients was 41 years, and the female:male ratio was 4:1. Initial histologic description included clear cell (n=8), papillary (n=1) or mixed clear cell/papillary RCC (n=6). Five patients had prior systemic therapy. Five patients had FISH analysis and all demonstrated a translocation involving chromosome Xp11.2. When treated with VEGF-targeted therapy, 3 patients had a partial response, 7 patients had stable disease and 5 patients had progressive disease. The median PFS and OS of the entire cohort were 7.1 months and 14.3 months respectively. Conclusion Adult-onset translocation-associated metastatic RCC is an aggressive disease that affects a younger population of patients with a female predominance. VEGF-targeted agents demonstrated some efficacy in this small retrospective series. PMID:20665500

Location of Dual Sites in E. coli FtsZ Important for Degradation by ClpXP; One at the C-Terminus and One in the Disordered Linker

PubMed Central

Camberg, Jodi L.; Viola, Marissa G.; Rea, Leslie; Hoskins, Joel R.; Wickner, Sue

2014-01-01

ClpXP is a two-component ATP-dependent protease that unfolds and degrades proteins bearing specific recognition signals. One substrate degraded by Escherichia coli ClpXP is FtsZ, an essential cell division protein. FtsZ forms polymers that assemble into a large ring-like structure, termed the Z-ring, during cell division at the site of constriction. The FtsZ monomer is composed of an N-terminal polymerization domain, an unstructured linker region and a C-terminal conserved region. To better understand substrate selection by ClpXP, we engineered FtsZ mutant proteins containing amino acid substitutions or deletions near the FtsZ C-terminus. We identified two discrete regions of FtsZ important for degradation of both FtsZ monomers and polymers by ClpXP in vitro. One region is located 30 residues away from the C-terminus in the unstructured linker region that connects the polymerization domain to the C-terminal region. The other region is near the FtsZ C-terminus and partially overlaps the recognition sites for several other FtsZ-interacting proteins, including MinC, ZipA and FtsA. Mutation of either region caused the protein to be more stable and mutation of both caused an additive effect, suggesting that both regions are important. We also observed that in vitro MinC inhibits degradation of FtsZ by ClpXP, suggesting that some of the same residues in the C-terminal site that are important for degradation by ClpXP are important for binding MinC. PMID:24722340

Vascular endothelial growth factor-targeted therapy for the treatment of adult metastatic Xp11.2 translocation renal cell carcinoma.

PubMed

Choueiri, Toni K; Lim, Zita Dubauskas; Hirsch, Michelle S; Tamboli, Pheroze; Jonasch, Eric; McDermott, David F; Dal Cin, Paola; Corn, Paul; Vaishampayan, Ulka; Heng, Daniel Y C; Tannir, Nizar M

2010-11-15

Adult "translocation" renal cell carcinoma (RCC), bearing transcription factor E3 (TFE3) gene fusions at Xp11.2, is a recently recognized, unique entity for which prognosis and therapy remain poorly understood. In the current study, the authors investigated the effect of vascular endothelial growth factor (VEGF)-targeted therapy in this distinct subtype of RCC. A retrospective review was conducted to describe the clinical characteristics and outcome of adult patients with metastatic Xp11.2 RCC who had strong TFE3 nuclear immunostaining and received anti-VEGF therapy. Tumor response to anti-VEGF therapy was evaluated using Response Evaluation Criteria in Solid Tumors (RECIST) criteria. The Kaplan-Meier method was used to estimate progression-free survival (PFS) and overall survival (OS) distributions. Fifteen patients were identified, of whom 10, 3, and 2 received sunitinib, sorafenib, and monoclonal anti-VEGF antibodies, respectively. The median follow-up was 19.1 months, the median age of the patients was 41 years, and the female:male ratio was 4:1. Initial histologic description included clear cell (n = 8 patients), papillary (n = 1 patient), or mixed clear cell/papillary RCC (n = 6 patients). Five patients had received prior systemic therapy. Five patients had undergone fluorescent in situ hybridization analysis and all demonstrated a translocation involving chromosome Xp11.2. When treated with VEGF-targeted therapy, 3 patients achieved a partial response, 7 patients had stable disease, and 5 patients developed progressive disease. The median PFS and OS of the entire cohort were 7.1 months and 14.3 months, respectively. Adult-onset, translocation-associated metastatic RCC is an aggressive disease that affects a younger population of patients with a female predominance. In the current study, VEGF-targeted agents appeared to demonstrate some efficacy. Copyright © 2010 American Cancer Society.

Lateral stability and control tests of the XP-77 airplane in the NACA full-scale tunnel, 16 June 1944

NASA Technical Reports Server (NTRS)

Czarnecki, K. R.; Donlan, C. J.

1976-01-01

Tests were made in the NACA full-scale tunnel to determine the lateral stability and control characteristics of the XP-77 airplane. Measurements were made of the forces and moments on the airplane at various angles of attack and angles of yaw. The measurements were made with the propeller removed and with the propeller installed and operating at various thrust coefficients, and with the landing flaps retracted and deflected. The effects of aileron, elevator, and rudder deflection on control surface effectiveness and hinge moments were determined. The tests were planned to obtain the data required to evaluate as completely as possible the Army Air Force requirements on lateral stability and control for pursuit-type airplanes.

MiT translocation renal cell carcinomas: two subgroups of tumours with translocations involving 6p21 [t (6; 11)] and Xp11.2 [t (X;1 or X or 17)].

PubMed

Hora, Milan; Urge, Tomáš; Trávníček, Ivan; Ferda, Jiří; Chudáček, Zdeněk; Vaněček, Tomáš; Michal, Michal; Petersson, Fredrik; Kuroda, Naoto; Hes, Ondřej

2014-01-01

MiT translocation renal cell carcinomas (TRCC) predominantly occur in younger patients with only 25% of patients being over 40 years. TRCC contains two main subgroups with translocations involving 6p21 or Xp11.2. Herein we present 10 cases. Eight cases were treated at main author's institution (identified among 1653 (0.48%) cases of kidney tumours in adults). Two cases were retrieved from the Pilsen (CZ) Tumour Registry. Six cases were type Xp11.2 and four 6p21; 7 female, 3 male patients; Xp11.2 4:2, 6p21 3:1. The mean age 49 years (range: 21-80), 5 patients (50%) over 40 years. The mean age of the group with Xp11.2 TRCCs was 55 (median 51) and 6p21 41 (32) years. One female with a 6p21 tumour (24 years) underwent nephrectomy at 4 months of pregnancy. Stage (UICC, 7th ed. 2009) was 5xI, 3xIII, 2xIV. The mean size of tumour was 80 (40-165) mm. The mean follow-up was 33.2 (1-92) months. In patients with 6p21 tumours, one (25%) died after 3 months due to widely metastatic disease. In patients with Xp11.2 tumours, 3 (50%) succumbed due to metastatic disease (range 1-8 months). Three patients with Xp11.2 are alive at 7, 52 and 92 months of follow-up, were diagnosed at early stage (T1a). TRCCs were more common in females. Patient with 6p21 tumours were younger than those with Xp11.2. Both types have definitive malignant potential Type Xp11.2 seems to be a more aggressive neoplasm than 6p21. The case with metastatic 6p21 tumour is the 4th case described in the English literature.

Discovery and identification of O, O-diethyl O-(4-(5-phenyl-4, 5-dihydroisoxazol-3-yl) phenyl) phosphorothioate (XP-1408) as a novel mode of action of organophosphorus insecticides.

PubMed

Zeng, Zhigang; Yan, Ying; Wang, Bingfeng; Liu, Niu; Xu, Hanhong

2017-06-15

Organophosphorus (OP) insecticides play an important role in pest control. Many OP insecticides have been removed from the market because of their high toxicity to humans. We designed and synthesized a new OP insecticide with the goal of providing a low cost, and less toxic insecticide. The mode of action of O, O-diethyl O-(4-(5-phenyl-4, 5-dihydroisoxazol-3-yl) phenyl) phosphorothioate (XP-1408) was studied in Drosophila melanogaster. Bioassays showed that XP-1408 at a concentration of 50 mg/L delayed larval development. Molecular docking into Drosophila acetylcholinesterase (AChE) and voltage-gated sodium channels suggested that XP-1408 fitted into their active sites and could be inhibitory. Whole-cell patch clamp recordings indicated that XP-1408 exhibited synergistic effects involving the inhibition of cholinergic synaptic transmission and blockage of voltage-gated potassium (K v ) channels and sodium (Na v ) channels. In conclusion, the multiple actions of XP-1408 rendered it as a lead compound for formulating OP insecticides with a novel mode of action.

Renal carcinomas associated with Xp11.2 translocations/TFE3 gene fusions: findings on MRI and computed tomography imaging.

PubMed

Liu, Kefu; Xie, Ping; Peng, Weijun; Zhou, Zhengrong

2014-08-01

To retrospectively analyze MRI and computed tomographic (CT) findings from renal carcinomas associated with Xp11.2 translocations/TFE3 gene fusions (Xp11-RCC). Institutional review board permission was obtained to review patient medical records, and the requirement for informed consent was waved . The clinical and MRI/CT features of five cases with Xp11-RCC that were confirmed by pathology were analyzed retrospectively. The image characteristics included the lesion location and size, contribution of cystic and solid components, intratumoral necrosis or hemorrhage, invasion of perinephric tissue and renal sinus, lymphadenopathy, major venous or arterial vascular invasion, pattern of the tumor growth, intratumor calcification and lipids, homogeneity of SI on T2-weighted images, attenuation and SI of the mass with respect to the normal renal cortex on precontrast and contrasted CT/MRI images, tumor SIs, tumor attenuations and tumor-to-cortex indices, homogeneity of enhancement on the contrasted images. The mean age was 32 years (range, 15-47 years). Most patients (4/5) were women. All tumors showed a cortical location. The average tumor size was 9 cm (range, 4-18 cm). Four tumors comprised a predominantly solid lesion with focal necrosis, and one tumor comprised a solid lesion with significant necrosis. All tumors showed intertumor hemorrhage, infiltrative growth and invasion of the perirenal adipose/renal sinus. Four cases showed retroperitoneal lymphadenopathy, of which one case showed simultaneous mediastinal and supraclavicular lymphadenopathy. All tumors from four cases showed mild hyperintensity on T1-weighted MRI images, and three tumors showed hypointensity on T2-weighted MRI images relative to the renal cortex except for 1 tumor that showed significant hemorrhage and a relative hyperintensity. For 3 cases who were imaged with CT, two tumors imaged using nonenhanced CT images showed mild hyperdensity relative to the renal cortex. Calcification was noted in all

Biological characteristics of pediatric renal cell carcinoma associated with Xp11.2 translocations/TFE3 gene fusions.

PubMed

Song, Hong Cheng; Sun, Ning; Zhang, Wei Ping; He, LeJian; Fu, Libing; Huang, ChengRu

2014-04-01

To investigate the clinical features of pediatric Xp11.2 translocation renal cell carcinoma (RCC). A retrospective review of 22 cases over 35 years. Xp11.2 translocation RCCs were identified in 13 boys and 9 girls with a median age of 10.5 years (range: 2.5-16 years). RCC presented with hematuria in 17, abdominal mass in 1, abdominal masses with hematuria in 2, abdominal pain with hematuria in 1, and as an incidental finding in 1 patient. Ten patients were classified stage I, 10 were stage III, and two were stage IV. Of the 10 patients with stage I RCCs, 3 patients with tumor measuring less than 7 cm had nephron-sparing surgery (NSS) and 17 patients underwent simple nephrectomy. A 15-cm tumor was incompletely removed in one patient and another patient with a 25-cm × 18-cm × 15-cm tumor had gross residual. Of the 15 patients followed up between 6 months and 35 years, 13 were still living and 2 had died after surgery. Xp11.2 translocation RCC is the predominant form of pediatric RCC, associated with advanced stage at presentation. Nephrectomy is the usual treatment for RCC but NSS is an option for patients with tumors measuring<7 cm. Patients with N+M0 maintained a favorable prognosis following surgery alone. © 2014.

An adult Xp11.2 translocation renal carcinoma showing response to treatment with sunitinib.

PubMed

Pwint, Thinn P; Macaulay, Valentine; Roberts, Ian S D; Sullivan, Mark; Protheroe, Andrew

2011-01-01

A rare variant of renal cell carcinoma (RCC) with a translocation involving Xp11.2 has become increasingly recognized as a separate entity in the 2004 World Health Organization (WHO) kidney carcinoma classification. These tumors predominantly affect children and young adults and tend to present with advanced stage disease. Although reported to be indolent in children, adult cases run a more aggressive course. Little is known about their natural history, prognosis and response to therapy. We report a case of Xp11 translocation renal cancer in a 33-year-old male patient who presented with widespread rapidly progressive metastatic disease involving extensive intra-thoracic lymph nodes, supra-clavicular, retroperitoneal lymph nodes, lung nodules, and peritoneal mass. He had failed to respond to treatment with high dose interleukin 2, but showed a significant clinical response to treatment with the multikinase inhibitor sunitinib. CT scan performed after 3 cycles (18 weeks) of therapy revealed more than 65% reduction of measurable disease by response evaluation criteria in solid tumors (RECIST) criteria, resolution of other assessable lesions, and a clinical benefit that lasted for over 13 months. But unfortunately, this was subsequently followed by a rapidly progressive course. The well-recognized clinical efficacy of multikinase inhibitors such as sunitinib and sorafenib is based on the outcomes in patients with clear cell histology. There is limited data on efficacy in non-clear cell RCC, but activity in translocation RCC has not been reported. To our knowledge, this is the first documented case of Xp11 translocation carcinoma to have demonstrated an objective durable response to sunitinib. It remains unclear how resistance to sunitinib develops, but the results to date support further evaluation of sunitinib in cases of translocation RCC. Copyright © 2011 Elsevier Inc. All rights reserved.

Phosphorylated 4EBP1 is associated with tumor progression and poor prognosis in Xp11.2 translocation renal cell carcinoma.

PubMed

Qu, Yuanyuan; Zhao, Rui; Wang, Hongkai; Chang, Kun; Yang, Xiaoqun; Zhou, Xiaoyan; Dai, Bo; Zhu, Yao; Shi, Guohai; Zhang, Hailiang; Ye, Dingwei

2016-03-30

Two main signaling pathways, PI3K-AKT-mTOR and RAS-MAPK, are involved in transmitting the proliferative signals which play critical roles in human cancers. However, the functions of these pathways in Xp11.2 RCC remain undefined. This study aimed to explore the expression of mTOR and MAPK cascades in Xp11.2 RCC and to assess the prognostic significance of proteins evaluated. Immunohistochemistry was performed to evaluate the expression of 4EBP1, p-4EBP1, p-mTOR, p-S6K and p-MAPK in 36 adult Xp11.2 RCC patients who were confirmed by FISH assay. Cox regression models were used to evaluate the prognostic value of all covariates. Among 36 assessed patients, 14 (38.9%), 26 (72.2%), 16 (44.4%), 19 (52.8%), and 9 (25.0%) patients showed high expression of 4EBP1, p-4EBP1, p-mTOR, p-S6K, and p-MAPK, respectively. We noted that p-4EBP1 expression was significantly correlated with lymph node metastases (P = 0.027). Multivariate analysis showed that high p-4EBP1 expression was an independent adverse prognostic factor for both PFS (HR = 33.750, P = 0.017) and OS (HR = 56.111, P = 0.026). Our findings suggest that p-4EBP1 may serve as a funnel factor that converge the upstream proliferative oncogenic signals. Effective inhibition of the pathways responsible for 4E-BP1 phosphorylation might be a useful strategy to improve the outcome of Xp11.2 RCC patients.

SR450 and Superhawk XP applications of Bacillus thuringiensis israelensis de Barjac against Culex quinquefasciatus Say

USDA-ARS?s Scientific Manuscript database

Sprayer comparisons and larval morality assays were conducted following SR450 backpack mist blower and Superhawk XP thermal fogger applications of Vectobac® WDG Bacillus thuringiensis israelensis (Bti) de Barjac against Culex quinquefasciatus Say. Bacillus thuringiensis israelensis was applied at m...

Performance evaluation of the Sysmex(®) XP-300 in an oncology setting: evaluation and comparison of hematological parameters with the Sysmex(®) XN-3000.

PubMed

van Dievoet, M A; Louagie, H; Ghys, T

2016-10-01

The Sysmex XP-300(®) (XP-300) is a new, fully automated hematology analyzer, designed to generate complete blood counts (CBC) with 3-part differential. In our study, the XP-300 was evaluated as a point-of-care (POC) analyzer in an oncology setting. In which blood samples from patients with different pathologies and treatments, affecting hematopoiesis, were analyzed. Performance was evaluated according to the International Council for Standardization in Haematology (ICSH) guidelines and CLSI protocol H20-A2 . Beside precision, linearity and carry-over, a comparison study with the Sysmex(®) XN-3000 (XN-3000) and a manual reference leukocyte differential was performed. Flagging performance was also evaluated. XP-300 showed excellent precision and linearity results. For within- and between-run precision, the criteria, according to Ricos et al. , were met for all parameters tested, except for platelets in the low level. Less than or equal to 0.5% carry-over was seen for all parameters tested. Comparison studies showed an acceptable correlation with both XN-3000 and the manual reference leukocyte count. A suboptimal flagging performance was demonstrated. In the context of diagnosing cytopenia due to myelosuppressing agents or leukocytosis due to infection, the XP-300 showed good analytical performance. However, in the thrombocytopenic range, precision was suboptimal. In follow-up of hematological malignancies with the occurrence of abnormal cells, we advise verification with a more advanced analyzer or with microscopic review, although further studies with a higher prevalence of abnormal cells are needed. © 2016 John Wiley & Sons Ltd.

High-speed Photometric Analysis for Minor Planets 1586 Thiele, 4246 Telemann, (10662) 32-1 T-2, and (49880) 1999 XP 135

NASA Astrophysics Data System (ADS)

Childers, Daniel; Church, Alyssia

2007-12-01

Photometric observations of 1586 Thiele, 4246 Telemann, (10662) 3201 T-2, and (49880) 1999 XP135 were performed in September and October of 2005. The periods and amplitudes found were: 1586 Thiele 3.086 ± 0.038 h, 0.136 ± 0.011 mag; 4246 Telemann 8.960 ± 0.038 h, 0.109 ± 0.027 mag; (10662) 3201 T-2, 3.072 ± 0.038 h, 0.151 ± 0.04 mag; and (49880) 1999 XP135, 11.111 ± 0.038 h, 0.102 ± 0.035 mag.

Substance P and Calcitonin gene-related peptide expression in human periodontal ligament after root canal preparation with Reciproc Blue, WaveOne Gold, XP EndoShaper and hand files.

PubMed

Caviedes-Bucheli, J; Rios-Osorio, N; Rey-Rojas, M; Laguna-Rivero, F; Azuero-Holguin, M M; Diaz, L E; Curtidor, H; Castaneda-Ramirez, J J; Munoz, H R

2018-05-17

To quantify Substance P (SP) and Calcitonin gene-related peptide (CGRP) expression in healthy human periodontal ligament from premolars after root canal preparation with Reciproc Blue, WaveOne Gold, XP EndoShaper and hand files. A total of 50 human periodontal ligament samples were obtained from healthy mandibular premolars where extraction was indicated for orthodontic reasons. Prior to extraction, 40 of these premolars were equally divided into four groups, and root canals were prepared using four different systems: Reciproc Blue, WaveOne Gold, XP EndoShaper and a hand instrumentation technique. The remaining 10 healthy premolars were extracted without treatment and served as a negative control group. All periodontal ligament samples were processed, and SP and CGRP were measured by radioimmunoassay. The Kruskal-Wallis test was used to establish significant differences between groups and LSD post hoc comparisons were also performed. Greater SP and CGRP values were found in the hand instrumentation group, followed by the XP EndoShaper, WaveOne Gold and the Reciproc groups. The lower SP and CGRP values were for the healthy periodontal ligament group. The Kruskal-Wallis test revealed significant differences between groups (P < 0.05). Post hoc Least Significant Difference (LSD) tests revealed significant differences (P < 0.05) in SP and CGRP expression between all the comparisons except for the Reciproc Blue and WaveOne Gold group (P > 0.05). All the root canal preparation techniques tested increased SP and CGRP expression in human periodontal ligament, with hand files and XP EndoShaper instruments being associated with greater neuropeptide release compared to Reciproc Blue and WaveOne Gold files. © 2018 International Endodontic Journal. Published by John Wiley & Sons Ltd.

Torsional resistance of XP-endo Shaper at body temperature compared with several nickel-titanium rotary instruments.

PubMed

Elnaghy, A M; Elsaka, S E

2018-05-01

To compare the torsional resistance of XP-endo Shaper (XPS; size 30, .01 taper, FKG Dentaire, La Chaux-de-Fonds, Switzerland) instruments at body temperature with TRUShape (TRS; size 30, .06 taper, Dentsply Tulsa Dental Specialties, Tulsa, OK, USA), ProFile Vortex (PV; size 30, .04 taper, Dentsply Tulsa Dental Specialties) and FlexMaster (FM; size 30, .04 taper, VDW GmbH, Munich, Germany) nickel-titanium rotary instruments. A metal block with a square-shaped mould (5 mm × 5 mm × 5 mm) was positioned inside a glass container. Five millimetres of the tip of each instrument was held inside the metal block by filling the mould with a resin composite. The instruments were tested for torsional resistance in saline solution at 37 °C. Data were analysed using one-way analysis of variance (anova) and Tukey post hoc tests. The significance level was set at P < 0.05. FM had the greatest torsional resistance amongst the instruments tested (P < 0.001). There was no significant difference between FM and PV instruments (P = 0.211). The ranking for torsional resistance values was: FM > PV > TRS > XPS. FlexMaster and ProFile Vortex instruments were more resistant to torsional stress compared with TRUShape and XP-endo Shaper instruments. The manufacturing process used to produce XP-endo Shaper instruments did not enhance their resistance to torsional stress as compared with the other instruments. © 2017 International Endodontic Journal. Published by John Wiley & Sons Ltd.

Phase II trial of capecitabine plus modified cisplatin (mXP) as first-line therapy in Japanese patients with metastatic gastric cancer (KSCC1104).

PubMed

Satake, Hironaga; Iwatsuki, Masaaki; Uenosono, Yoshikazu; Shiraishi, Takeshi; Tanioka, Hiroaki; Saeki, Hiroshi; Sugimachi, Keishi; Kitagawa, Dai; Shimokawa, Mototsugu; Oki, Eiji; Emi, Yasunori; Kakeji, Yoshihiro; Tsuji, Akihito; Akagi, Yoshito; Natsugoe, Shoji; Baba, Hideo; Maehara, Yoshihiko

2017-01-01

Capecitabine plus cisplatin (XP) is a standard therapy for metastatic gastric cancer (mGC). However, while results from previous phase III trials suggested that the cisplatin dosage should be reduced in Japanese patients, no clinical data exist to support this. Here, we conducted a multicenter study to evaluate the efficacy and safety of modified XP (mXP) in Japanese patients with mGC. Patients with previously untreated mGC received mXP (cisplatin 60 mg/m 2 on day 1 plus capecitabine 1000 mg/m 2 twice daily on days 1-14) every 3 weeks. The primary endpoint was the Response Evaluation Criteria in Solid Tumors-confirmed overall response rate (ORR). A sample size of 40 was planned for a threshold ORR of 30% and an expected value of 50%, with a one-sided α of 0.05 and a beta of approximately 0.2. Forty-two patients were enrolled. One patient did not fulfill the eligibility criteria; therefore, a total of 41 patients were assessed. The results were as follows: complete response in 2 patients, partial response in 16, stable disease in 14, progressive disease in 8, and no evaluation in 1. The confirmed ORR was 43.9% (95% confidence interval 28.7-59.1%). The median progression-free survival and median overall survival were 4.6 and 11.3 months, respectively. The most common grade 3 or 4 adverse events were neutropenia (37.5%), anemia (24.4%), anorexia (24.4%), and nausea (12.2%). First-line chemotherapy with mXP in Japanese patients with mGC did not reach its primary objective. However, it did show a promising response rate and an acceptable tolerability profile.

Phosphorylated 4EBP1 is associated with tumor progression and poor prognosis in Xp11.2 translocation renal cell carcinoma

PubMed Central

Qu, Yuanyuan; Zhao, Rui; Wang, Hongkai; Chang, Kun; Yang, Xiaoqun; Zhou, Xiaoyan; Dai, Bo; Zhu, Yao; Shi, Guohai; Zhang, Hailiang; Ye, Dingwei

2016-01-01

Two main signaling pathways, PI3K-AKT-mTOR and RAS-MAPK, are involved in transmitting the proliferative signals which play critical roles in human cancers. However, the functions of these pathways in Xp11.2 RCC remain undefined. This study aimed to explore the expression of mTOR and MAPK cascades in Xp11.2 RCC and to assess the prognostic significance of proteins evaluated. Immunohistochemistry was performed to evaluate the expression of 4EBP1, p-4EBP1, p-mTOR, p-S6K and p-MAPK in 36 adult Xp11.2 RCC patients who were confirmed by FISH assay. Cox regression models were used to evaluate the prognostic value of all covariates. Among 36 assessed patients, 14 (38.9%), 26 (72.2%), 16 (44.4%), 19 (52.8%), and 9 (25.0%) patients showed high expression of 4EBP1, p-4EBP1, p-mTOR, p-S6K, and p-MAPK, respectively. We noted that p-4EBP1 expression was significantly correlated with lymph node metastases (P = 0.027). Multivariate analysis showed that high p-4EBP1 expression was an independent adverse prognostic factor for both PFS (HR = 33.750, P = 0.017) and OS (HR = 56.111, P = 0.026). Our findings suggest that p-4EBP1 may serve as a funnel factor that converge the upstream proliferative oncogenic signals. Effective inhibition of the pathways responsible for 4E-BP1 phosphorylation might be a useful strategy to improve the outcome of Xp11.2 RCC patients. PMID:27026382

Low temperature growth of Ga 1- xIn xP bulk crystals from InSb-rich melt

NASA Astrophysics Data System (ADS)

Gennett, A.; Lewis, D.; Dutta, P. S.

2010-04-01

Bulk growth of phosphorus and arsenic based ternary III-V semiconductor crystals using pseudo-binary melts such as GaP-InP, GaP-GaAs, AlAs-GaAs, etc. is significantly challenging due to the high vapor pressures of group V species in conjunction with slow growth rates and the need for melt replenishment and mixing during growth. Lowering the growth temperature is desirable such that the vapor pressures of P and As can be easily handled. Low growth temperatures could be achieved by using Ga or In rich solutions. However, this approach is less attractive for growing bulk crystals due to several experimental difficulties including sticking of the growth solution to the crucible wall and to the grown crystal, making it challenging for crystal extraction. Growth of ternary crystals from low temperature quaternary melts has been found to be attractive. In this paper, we will present a new method for the growth of Ga 1- xIn xP from InSb rich Ga 1- xIn xP ySb 1- y melts at low growth temperatures in the range of 800-1050 °C. Thermodynamic phase diagrams calculated at various temperatures using a Gibbs free energy minimization software and materials databases commercially available from Thermo-Calc software will be presented along with experimental validation for Ga 1- xIn xP crystals grown at 1000 °C.

A 13-year-old female with Xp11.2 translocation renal cell carcinoma; the first case diagnosed at Siriraj Hospital.

PubMed

Hanamornroongruang, Suchanan; Treetipsatit, Jitsupa; Pongtanakul, Bunchoo; Seangchai, Napakorn

2012-07-01

Xp11.2 translocation renal cell carcinomas are rare tumors characterized by translocations involving chromosome Xp11.2. These tumors are predominantly reported in pediatric patients. The authors report Xp11.2 translocation renal cell carcinoma in a 13-year-old girl who presented with asymptomatic palpable right renal mass. Right radical nephrectomy was performed and revealed a well-defined solid mass at the lower pole of the kidney. Microscopically, the tumor was composed of sheets and nests of clear to pale eosinophilic cells with some alveolar growth pattern. Psammoma bodies were detected. Immunohistochemically, the tumor cells marked with TFE3, focally marked with smooth muscle actin, HMB-45, CD68, progesterone receptor (PR) and CD10 but did not mark with epithelial markers (AE1/AE3, EMA and CAM5.2), vimentin, S-100 and p53. The presence of psammoma bodies is an important diagnostic clue for these tumors. Cytogenetic study and/or immunohistochemistry for TFE3 protein are needed for confirming the diagnosis. Currently, surgery seems to be the most effective therapy Pediatric patients with these tumors are believed to have a favorable prognosis.

Verification of Bioanalytical Method for Quantification of Exogenous Insulin (Insulin Aspart) by the Analyser Advia Centaur® XP.

PubMed

Mihailov, Rossen; Stoeva, Dilyana; Pencheva, Blagovesta; Pentchev, Eugeni

2018-03-01

In a number of cases the monitoring of patients with type I diabetes mellitus requires measurement of the exogenous insulin levels. For the purpose of a clinical investigation of the efficacy of a medical device for application of exogenous insulin aspart, a verification of the method for measurement of this synthetic analogue of the hormone was needed. The information in the available medical literature for the measurement of the different exogenous insulin analogs is insufficient. Thus, verification was required to be in compliance with the active standards in Republic of Bulgaria. A manufactured method developed for ADVIA Centaur XP Immunoassay, Siemens Healthcare, was used which we verified using standard solutions and a patient serum pool by adding the appropriate quantity exogenous insulin aspart. The method was verified in accordance with the bioanalytical method verification criteria and regulatory requirements for using a standard method: CLIA chemiluminescence immunoassay ADVIA Centaur® XP. The following parameters are determined and monitored: intra-day precision and accuracy, inter-day precision and accuracy, limit of detection and lower limit of quantification, linearity, analytical recovery. The routine application of the method for measurement of immunoreactive insulin using the analyzer ADVIA Centaur® XP is directed to the measurement of endogenous insulin. The method is applicable for measuring different types of exogenous insulin, including insulin aspart.

ClpXP protease targets long-lived DNA translocation states of a helicase-like motor to cause restriction alleviation

PubMed Central

Simons, Michelle; Diffin, Fiona M.; Szczelkun, Mark D.

2014-01-01

We investigated how Escherichia coli ClpXP targets the helicase-nuclease (HsdR) subunit of the bacterial Type I restriction–modification enzyme EcoKI during restriction alleviation (RA). RA is a temporary reduction in endonuclease activity that occurs when Type I enzymes bind unmodified recognition sites on the host genome. These conditions arise upon acquisition of a new system by a naïve host, upon generation of new sites by genome rearrangement/mutation or during homologous recombination between hemimethylated DNA. Using recombinant DNA and proteins in vitro, we demonstrate that ClpXP targets EcoKI HsdR during dsDNA translocation on circular DNA but not on linear DNA. Protein roadblocks did not activate HsdR proteolysis. We suggest that DNA translocation lifetime, which is elevated on circular DNA relative to linear DNA, is important to RA. To identify the ClpX degradation tag (degron) in HsdR, we used bioinformatics and biochemical assays to design N- and C-terminal mutations that were analysed in vitro and in vivo. None of the mutants produced a phenotype consistent with loss of the degron, suggesting an as-yet-unidentified recognition pathway. We note that an EcoKI nuclease mutant still produces cell death in a clpx− strain, consistent with DNA damage induced by unregulated motor activity. PMID:25260590

Mental retardation in a boy with an interstitial deletion at Xp22.3 involving STS, KAL1, and OA1: Implication for the MRX locus

DOE Office of Scientific and Technical Information (OSTI.GOV)

Muroya, Koji; Ogata, Tsutomu; Natsuo, Nobutake

Although genotype-phenotype correlations in male patients with various types of nullisomy for Xp22.3 have assigned a locus for X-linked mental retardation (MRX) to an approximately 3-Mb region between DXS31 and STS, the precise location has not been determined. In this paper, we describe a 14 7/12 year old Japanese boy with mental retardation and an interstitial deletion at Xp22.3 involving STS, KAL1, and OA1, and compare the deletion map with that of previously reported three familial male patients with low-normal intelligence and a similar interstitial deletion at Xp22.3. The results suggest that the MRX gene is further localized to themore » roughly 1.5-Mb region between DXS1060 and DXS1139. 31 refs., 4 figs.« less

A case study of metastatic Xp11.2 translocation renal cell carcinoma effectively treated with sunitinib.

PubMed

Numakura, Kazuyuki; Tsuchiya, Norihiko; Yuasa, Takeshi; Saito, Mitsuru; Obara, Takashi; Tsuruta, Hiroshi; Narita, Shintaro; Horikawa, Yohei; Satoh, Shigeru; Habuchi, Tomonori

2011-10-01

We report a case of Xp11.2 translocation renal cell carcinoma (RCC) whose lung metastases were effectively treated with sunitinib. A 43-year-old woman presenting with upper abdominal pain was diagnosed with a left renal tumor. Laparoscopic left radical nephrectomy was performed. Histopathological examination of the surgical specimen revealed a clear-cell carcinoma of the left kidney. Two years later, multiple lung metastases were detected and the patient was treated daily with 50 mg sunitinib. A computed tomography scan performed after 2 cycles of sunitinib treatment revealed partial regression of these metastases. The partial regression has been maintained for >3 years. In retrospective evaluation of the primary RCC, tumor cells showed strong nuclear staining for transcription factor E3 (TFE3) protein and TFE3 split-fluorescence in-situ hybridization revealed translocation involving the TFE3 gene. These findings strongly support diagnosis of Xp11.2 translocation RCC.

Uncommon Localization of Extrarenal Xp11.2 Translocation-associated Renal Cell Carcinoma (RCC): Case Report.

PubMed

Al-Maghrabi, Jaudah Ahmed; Khabaz, Mohamad Nidal

2017-09-04

The World Health Organization has recognized Xp11.2 translocation-associated renal cell carcinoma (RCC) as a distinct neoplasm that arises within the kidney. Although many reports of extrarenal carcinoma may be found in the literature, to the best of our knowledge, Xp11 translocation-associated RCC with intact kidneys has not been documented. This report describes a multilobulated right retroperitoneal soft tissue mass (7.9×5.3×12.6 cm) of a 37-year-old man complaining of abdominal pain in the right side. The patient underwent a computed tomography-guided biopsy. Microscopic evaluation reveals a tumor with papillary and sheaths architectures with cells revealing clear to eosinophilic cytoplasm. Immunohistochemical evaluation on the biopsy reveals that the tumor is positive for PAX-8, CD10, and TFE3. It is negative for CK7, EMA, Vimentin, RCC, CK8/18, D20, CD3, PLAP, OCT4, CD30, MART-1, Inhibin, S-100, HMB-45, Desmin, SMA, and DOG-1. The diagnosis was malignant epithelioid neoplasm and the diagnosis of translocation RCC was suggested. Excision was recommended. The patient underwent right radical nephrectomy with removal of this large mass. Pathologic examination showed a large cystic and solid, nonhomogenous mass with some necrotic areas, originating from the perirenal fat between the adrenal gland and the kidney. Microscopic features showed a tumor with papillary, rhabdoid, and clear cell features. Immunohistochemical stains showed that the tumor cells positively expressed AMACR, PAX-8, CD10, RCC, and TFE3, but were negative for cytokeratins, vimentin, HMB-45, desmin, SMA, EMA, and MSA. Cytogenetic studies confirmed the diagnosis of Xp11.2 translocation-associated RCC with positive TFE3 gene rearrangement. To the best of our knowledge, this type of extrarenal tumor has never been reported.

Bioconversion of Pinoresinol Diglucoside and Pinoresinol from Substrates in the Phenylpropanoid Pathway by Resting Cells of Phomopsis sp.XP-8

PubMed Central

Zhang, Yan; Shi, Junling; Liu, Laping; Gao, Zhenhong; Che, Jinxin; Shao, Dongyan; Liu, Yanlin

2015-01-01

Pinoresinol diglucoside (PDG) and pinoresinol (Pin) are normally produced by plant cells via the phenylpropanoid pathway. This study reveals the existence of a related pathway in Phomopsis sp. XP-8, a PDG-producing fungal strain isolated from the bark of the Tu-chung tree (Eucommiaulmoides Oliv.). After addition of 0.15 g/L glucose to Phomopsis sp. XP-8, PDG and Pin formed when phenylalanine, tyrosine, leucine, cinnamic acid, and p-coumaric acid were used as the substrates respectively. No PDG formed in the absence of glucose, but Pin was detected after addition of all these substrates except leucine. In all systems in the presence of glucose, production of PDG and/or Pin and the accumulation of phenylalanine, cinnamic acid, or p-coumaric acid correlated directly with added substrate in a time- and substrate concentration- dependent manner. After analysis of products produced after addition of each substrate, the mass flow sequence for PDG and Pin biosynthesis was defined as: glucose to phenylalanine, phenylalanine to cinnamic acid, then to p-coumaric acid, and finally to Pin or PDG. During the bioconversion, the activities of four key enzymes in the phenylpropanoid pathway were also determined and correlated with accumulation of their corresponding products. PDG production by Phomopsis sp. exhibits greater efficiency and cost effectiveness than the currently-used plant-based system and will pave the way for large scale production of PDG and/or Pin for medical applications. PMID:26331720

Clinicopathological characteristics of Xp11.2 translocation renal cell carcinoma in adolescents and adults: Diagnosis using immunostaining of transcription factor E3 and fluorescence in situ hybridization analysis.

PubMed

Hirobe, Megumi; Masumori, Naoya; Tanaka, Toshiaki; Kitamura, Hiroshi; Tonooka, Akiko; Hasegawa, Tadashi; Tsukamoto, Taiji

2016-02-01

To determine the rate and clinicopathological features of Xp11.2 translocation carcinoma using immunostaining of transcription factor E3 and fluorescence in situ hybridization analysis. We evaluated 638 patients with renal cell carcinoma treated at Sapporo Medical University Hospital, Sapporo, Japan, from 1990 to 2009 by reviewing all hematoxylin-eosin-stained sections and carrying out immunostaining of transcription factor E3 for all cases. Fluorescence in situ hybridization analysis was carried out for patients with positive immunostaining or with findings suspicious for Xp11.2 translocation carcinoma on hematoxylin-eosin-stained sections. In this analysis, we set a cut-off level for split signals of at least 10% of nuclei. Of the 631 patients, 20 (3.2%) were positive for immunostaining. Finally, five patients were diagnosed with Xp11.2 translocation carcinoma (0.8%). Four of these patients were female and aged less than 50 years, and three cases were diagnosed as stage IV with multiple regional lymph nodal or visceral metastases. The positive predictive value of immunostaining was 25%. Patients with Xp11 translocation renal cell carcinoma tend to be younger, more frequently female and diagnosed at a more advanced stage. Immunostaining followed by fluorescence in situ hybridization analysis is an accurate and cost-effective approach for diagnosis of Xp11 translocation renal cell carcinoma. © 2015 The Japanese Urological Association.

Renal Cell Carcinoma Associated with Xp11.2 Translocation/TFE3 Gene Fusion: A Rare Case Report with Review of the Literature.

PubMed

Ahluwalia, Puneet; Nair, Balagopal; Kumar, Ginil

2013-01-01

Introduction. The recently recognized renal cell carcinomas associated with Xp11.2 translocations are rare tumors predominantly reported in children. Chromosome Xp11.2 translocation results in gene fusion related to transcription factor E3 (TFE3) that plays an important role in proliferation and survival. Case Report. Herein, we present two cases of a TFE3 translocation-associated RCC in young female adults, one detected incidentally and the other one presenting with gross hematuria. Tumor is characterized by immunohistochemistry and a literature review with optimal treatment regimen is presented. Discussion. Xp11.2 translocation RCCs in adult patients are associated with advanced stages, large tumors, and extracapsular disease and usually have an aggressive clinical course. Conclusion. In TFE3 RCC, the genetic background may not only contribute to tumorigenesis, but also determine the response to chemotherapy and targeted therapy. Therefore it is necessary to diagnose this tumor entity accurately. Because of the small number of TFE3 gene fusion-related renal tumors described in the literature, the exact biologic behavior and impact of current treatment modalities remain to be uncertain.

Renal Cell Carcinoma Associated with Xp11.2 Translocation/TFE3 Gene Fusion: A Rare Case Report with Review of the Literature

PubMed Central

Ahluwalia, Puneet; Nair, Balagopal; Kumar, Ginil

2013-01-01

Introduction. The recently recognized renal cell carcinomas associated with Xp11.2 translocations are rare tumors predominantly reported in children. Chromosome Xp11.2 translocation results in gene fusion related to transcription factor E3 (TFE3) that plays an important role in proliferation and survival. Case Report. Herein, we present two cases of a TFE3 translocation-associated RCC in young female adults, one detected incidentally and the other one presenting with gross hematuria. Tumor is characterized by immunohistochemistry and a literature review with optimal treatment regimen is presented. Discussion. Xp11.2 translocation RCCs in adult patients are associated with advanced stages, large tumors, and extracapsular disease and usually have an aggressive clinical course. Conclusion. In TFE3 RCC, the genetic background may not only contribute to tumorigenesis, but also determine the response to chemotherapy and targeted therapy. Therefore it is necessary to diagnose this tumor entity accurately. Because of the small number of TFE3 gene fusion-related renal tumors described in the literature, the exact biologic behavior and impact of current treatment modalities remain to be uncertain. PMID:24455396

Identification of a microdeletion at Xp22.13 in a Taiwanese family presenting with Nance-Horan syndrome.

PubMed

Liao, Hsiao-Mei; Niu, Dau-Ming; Chen, Yan-Jang; Fang, Jye-Siung; Chen, Shih-Jen; Chen, Chia-Hsiang

2011-01-01

Nance-Horan syndrome (NHS) is a rare X-linked disorder characterized by congenital cataracts, dental anomalies and mental retardation. The disease has been linked to a novel gene termed NHS located at Xp22.13. The majority of pathogenic mutations of the disease include nonsense mutations and small deletions and insertions that lead to truncation of the NHS protein. In this study, we identified a microdeletion of ∼ 0.92 Mb at Xp22.13 detected by array-based comparative genomic hybridization in two brothers presenting congenital cataract, dental anomalies, facial dysmorphisms and mental retardation. The deleted region encompasses the REPS2, NHS, SCML1 and RAI2 genes, and was transmitted from their carrier mother who presented only mild cataract. Our findings are in line with several recent case reports to indicate that genomic rearrangement involving the NHS gene is an important genetic etiology underlying NHS.

Force Tests of a 1/5-Scale Model of the McDonnell XP-85 Airplane with Conventional Tail Assembly in the Langley Free-Flight Tunnel

NASA Technical Reports Server (NTRS)

Paulson, John W.; Johnson, Joseph L.

1947-01-01

At the request of the Air Materiel Command, Army Air Forces an investigation of the low-speed, power-off stability and control characteristics of the McDonnell XP-85 airplane is being conducted in the Langley free-flight tunnel. The XP-85 airplane is a parasite fighter carried in a bomb bay of the B-36 airplane. As a part of the investigation a few force tests were made of a 1/5 scale model of the XP-85 with a conventional tail assembly installed in place of the original design five-unit tail assembly. The total area of the conventional assembly was approximately 80 percent of the area of the five-unit assembly. The results of this investigation showed that the conventional tail assembly gave about the same longitudinal stability characteristics as the original configuration and improved the directional and lateral stability.

TFE3 break-apart FISH has a higher sensitivity for Xp11.2 translocation-associated renal cell carcinoma compared with TFE3 or cathepsin K immunohistochemical staining alone: expanding the morphologic spectrum.

PubMed

Rao, Qiu; Williamson, Sean R; Zhang, Shaobo; Eble, John N; Grignon, David J; Wang, Mingsheng; Zhou, Xiao-Jun; Huang, Wenbin; Tan, Puay-Hoon; Maclennan, Gregory T; Cheng, Liang

2013-06-01

Renal cell carcinoma (RCC) associated with Xp11.2 translocation is uncommon, characterized by several different translocations involving the TFE3 gene. We assessed the utility of break-apart fluorescence in situ hybridization (FISH) in establishing the diagnosis for suspected or unclassified cases with negative or equivocal TFE3 immunostaining by analyzing 24 renal cancers with break-apart TFE3 FISH and comparing the molecular findings with the results of TFE3 and cathepsin K immunostaining in the same tumors. Ten tumors were originally diagnosed as Xp11.2 RCC on the basis of positive TFE3 immunostaining, and 14 were originally considered unclassified RCCs with negative or equivocal TFE3 staining, but with a range of features suspicious for Xp11.2 RCC. Seventeen cases showed TFE3 rearrangement associated with Xp11.2 translocation by FISH, including all 13 tumors with moderate or strong TFE3 (n=10) or cathepsin K (n=7) immunoreactivity. FISH-positive cases showed negative or equivocal immunoreactivity for TFE3 or cathepsin K in 7 and 10 tumors, respectively (both=3). None had positive immunohistochemistry but negative FISH. Morphologic features were typical for Xp11.2 RCC in 10/17 tumors. Unusual features included 1 melanotic Xp11.2 renal cancer, 1 tumor with mixed features of Xp11.2 RCC and clear cell RCC, and other tumors mimicking clear cell RCC, multilocular cystic RCC, or high-grade urothelial carcinoma. Morphology mimicking high-grade urothelial carcinoma has not been previously reported in these tumors. Psammoma bodies, hyalinized stroma, and intracellular pigment were preferentially identified in FISH-positive cases compared with FISH-negative cases. Our results support the clinical application of a TFE3 break-apart FISH assay for diagnosis and confirmation of Xp11.2 RCC and further expand the histopathologic spectrum of these neoplasms to include tumors with unusual features. A renal tumor with pathologic or clinical features highly suggestive of

Development of a Low-Latency, High Data Rate, Differential GPS Relative Positioning System for UAV Formation Flight Control

DTIC Science & Technology

2006-09-01

spiral development cycle involved transporting the software processes from a Windows XP / MATLAB environment to a Linux / C++ environment. This...tested on. Additionally, in the case of the GUMSTIX PC boards, the LINUX operating system is burned into the read-only memory. Lastly, both PC-104 and...both the real-time environment and the post-processed en - vironment. When the system operates in real-time mode, an output file is generated which

McDonald XP-85 Airplane in 40x80 foot Wind Tunnel.

NASA Image and Video Library

1948-04-08

Front View of McDonald XP-85 Plan Model. Parasite Airplane designed to be carried in the B-36 bombay (never built) At the time it was the smallest Jet powered airplane. The McDonnell XF-85 Goblin was an American prototype fighter aircraft conceived during World War II by McDonnell Aircraft. It was intended to be deployed from the bomb bay of the giant Convair B-36 bomber as a parasite fighter. The XF-85's intended role was to defend bombers from hostile interceptor aircraft, a need demonstrated during World War II

RBM10-TFE3 Renal Cell Carcinoma: A Potential Diagnostic Pitfall Due to Cryptic Intrachromosomal Xp11.2 Inversion Resulting in False-negative TFE3 FISH.

PubMed

Argani, Pedram; Zhang, Lei; Reuter, Victor E; Tickoo, Satish K; Antonescu, Cristina R

2017-05-01

Xp11 translocation renal cell carcinoma (RCC) are defined by chromosome translocations involving the Xp11 breakpoint which results in one of a variety of TFE3 gene fusions. TFE3 break-apart florescence in situ hybridization (FISH) assays are generally preferred to TFE3 immunohistochemistry (IHC) as a means of confirming the diagnosis in archival material, as FISH is less sensitive to the variable fixation which can result in false positive or false negative IHC. Prompted by a case report in the cytogenetics literature, we identify 3 cases of Xp11 translocation RCC characterized by a subtle chromosomal inversion involving the short arm of the X chromosome, resulting in an RBM10-TFE3 gene fusion. TFE3 rearrangement was not detected by conventional TFE3 break-apart FISH, but was suggested by strong diffuse TFE3 immunoreactivity in a clean background. We then developed novel fosmid probes to detect the RBM10-TFE3 gene fusion in archival material. These cases validate RBM10-TFE3 as a recurrent gene fusion in Xp11 translocation RCC, illustrate a source of false-negative TFE3 break-apart FISH, and highlight the complementary role of TFE3 IHC and TFE3 FISH.

A locus for isolated cataract on human Xp

PubMed Central

Francis, P; Berry, V; Hardcastle, A; Maher, E; Moore, A; Bhattacharya, S

2002-01-01

Purpose: To genetically map the gene causing isolated X linked cataract in a large European pedigree. Methods: Using the patient registers at Birmingham Women's Hospital, UK, we identified and examined 23 members of a four generation family with nuclear cataract. Four of six affected males also had complex congenital heart disease. Pedigree data were collated and leucocyte DNA extracted from venous blood. Linkage analysis by PCR based microsatellite marker genotyping was used to identify the disease locus and mutations within candidate genes screened by direct sequencing. Results: The disease locus was genetically refined to chromosome Xp22, within a 3 cM linkage interval flanked by markers DXS9902 and DXS999 (Zmax=3.64 at θ=0 for marker DXS8036). Conclusions: This is the first report of a locus for isolated inherited cataract on the X chromosome. The disease interval lies within the Nance-Horan locus suggesting allelic heterogeneity. The apparent association with congenital cardiac anomalies suggests a possible new oculocardiac syndrome. PMID:11836358

A locus for isolated cataract on human Xp.

PubMed

Francis, P J; Berry, V; Hardcastle, A J; Maher, E R; Moore, A T; Bhattacharya, S S

2002-02-01

To genetically map the gene causing isolated X linked cataract in a large European pedigree. Using the patient registers at Birmingham Women's Hospital, UK, we identified and examined 23 members of a four generation family with nuclear cataract. Four of six affected males also had complex congenital heart disease. Pedigree data were collated and leucocyte DNA extracted from venous blood. Linkage analysis by PCR based microsatellite marker genotyping was used to identify the disease locus and mutations within candidate genes screened by direct sequencing. The disease locus was genetically refined to chromosome Xp22, within a 3 cM linkage interval flanked by markers DXS9902 and DXS999 (Zmax=3.64 at theta=0 for marker DXS8036). This is the first report of a locus for isolated inherited cataract on the X chromosome. The disease interval lies within the Nance-Horan locus suggesting allelic heterogeneity. The apparent association with congenital cardiac anomalies suggests a possible new oculocardiac syndrome.

Newly Designed Break-Apart and ASPL-TFE3 Dual-Fusion FISH Assay Are Useful in Diagnosing Xp11.2 Translocation Renal Cell Carcinoma and ASPL-TFE3 Renal Cell Carcinoma

PubMed Central

Chen, Xiancheng; Yang, Yang; Gan, Weidong; Xu, Linfeng; Ye, Qing; Guo, Hongqian

2015-01-01

Abstract The diagnosis of Xp11.2 translocation renal cell carcinoma (tRCC), which relies on morphology and immunohistochemistry (IHC), is often either missed in the diagnosis or misdiagnosed. To improve the accuracy of diagnosis of Xp11.2 tRCC and ASPL-TFE3 renal cell carcinoma (RCC), we investigated newly designed fluorescence in situ hybridization (FISH) probes (diagnostic accuracy study). Based on the genetic characteristics of Xp11.2 tRCC and the ASPL-TFE3 RCC, a new break-apart TFE3 FISH probe and an ASPL-TFE3 dual-fusion FISH probe were designed and applied to 65 patients with RCC who were <45 years old or showed suspicious microscopic features of Xp11.2 tRCC in our hospital. To test the accuracy of the probes, we further performed reverse transcriptase–polymerase chain reaction (PCR) on 8 cases for which frozen tissues were available. Among the 65 cases diagnosed with RCC, TFE3 IHC was positive in 24 cases. Twenty-two cases were confirmed as Xp11.2 tRCC by break-apart TFE3 FISH, and 6 of these cases were further diagnosed as ASPL-TFE3 RCC by ASPL-TFE3 dual-fusion FISH detection. Importantly, reverse transcriptase–PCR showed concordant results with the results of FISH assay in the 8 available frozen cases. The break-apart and ASPL-TFE3 dual-fusion FISH assay can accurately detect the translocation of the TFE3 gene and ASPL-TFE3 fusion gene and can thus serve as a valid complementary method for diagnosing Xp11.2 tRCC and ASPL-TFE3 RCC. PMID:25984679

Refinement of the NHS locus on chromosome Xp22.13 and analysis of five candidate genes.

PubMed

Toutain, Annick; Dessay, Benoît; Ronce, Nathalie; Ferrante, Maria-Immacolata; Tranchemontagne, Julie; Newbury-Ecob, Ruth; Wallgren-Pettersson, Carina; Burn, John; Kaplan, Josseline; Rossi, Annick; Russo, Silvia; Walpole, Ian; Hartsfield, James K; Oyen, Nina; Nemeth, Andrea; Bitoun, Pierre; Trump, Dorothy; Moraine, Claude; Franco, Brunella

2002-09-01

Nance-Horan syndrome (NHS) is an X-linked condition characterised by congenital cataracts, dental abnormalities, dysmorphic features, and mental retardation in some cases. Previous studies have mapped the disease gene to a 2 cM interval on Xp22.2 between DXS43 and DXS999. We report additional linkage data resulting from the analysis of eleven independent NHS families. A maximum lod score of 9.94 (theta=0.00) was obtained at the RS1 locus and a recombination with locus DXS1195 on the telomeric side was observed in two families, thus refining the location of the gene to an interval of around 1 Mb on Xp22.13. Direct sequencing or SSCP analysis of the coding exons of five genes (SCML1, SCML2, STK9, RS1 and PPEF1), considered as candidate genes on the basis of their location in the critical interval, failed to detect any mutation in 12 unrelated NHS patients, thus making it highly unlikely that these genes are implicated in NHS.

Analysis of Parent-of-Origin Effects on the X Chromosome in Asian and European Orofacial Cleft Triads Identifies Associations with DMD, FGF13, EGFL6, and Additional Loci at Xp22.2.

PubMed

Skare, Øivind; Lie, Rolv T; Haaland, Øystein A; Gjerdevik, Miriam; Romanowska, Julia; Gjessing, Håkon K; Jugessur, Astanand

2018-01-01

Background: Although both the mother's and father's alleles are present in the offspring, they may not operate at the same level. These parent-of-origin (PoO) effects have not yet been explored on the X chromosome, which motivated us to develop new methods for detecting such effects. Orofacial clefts (OFCs) exhibit sex-specific differences in prevalence and are examples of traits where a search for various types of effects on the X chromosome might be relevant. Materials and Methods: We upgraded our R-package Haplin to enable genome-wide analyses of PoO effects, as well as power simulations for different statistical models. 14,486 X-chromosome SNPs in 1,291 Asian and 1,118 European case-parent triads of isolated OFCs were available from a previous GWAS. For each ethnicity, cleft lip with or without cleft palate (CL/P) and cleft palate only (CPO) were analyzed separately using two X-inactivation models and a sliding-window approach to haplotype analysis. In addition, we performed analyses restricted to female offspring. Results: Associations were identified in "Dystrophin" ( DMD , Xp21.2-p21.1), "Fibroblast growth factor 13" ( FGF13 , Xq26.3-q27.1) and "EGF-like domain multiple 6" ( EGFL6 , Xp22.2), with biologically plausible links to OFCs. Unlike EGFL6 , the other associations on chromosomal region Xp22.2 had no apparent connections to OFCs. However, the Xp22.2 region itself is of potential interest because it contains genes for clefting syndromes [for example, "Oral-facial-digital syndrome 1" ( OFD1 ) and "Midline 1" ( MID1 )]. Overall, the identified associations were highly specific for ethnicity, cleft subtype and X-inactivation model, except for DMD in which associations were identified in both CPO and CL/P, in the model with X-inactivation and in Europeans only. Discussion/Conclusion: The specificity of the associations for ethnicity, cleft subtype and X-inactivation model underscores the utility of conducting subanalyses, despite the ensuing need to adjust

Adult-onset renal cell carcinoma associated with Xp11.2 translocations/TFE3 gene fusion with smooth muscle stroma and abnormal vessels.

PubMed

Kuroda, Naoto; Tamura, Masato; Tanaka, Yukichi; Hes, Ondrej; Michal, Michal; Inoue, Kaori; Ohara, Masahiko; Mizuno, Keiko; Lee, Gang-Hong

2009-07-01

Renal cell carcinoma (RCC) associated with Xp11.2 translocation/TFE3 gene fusion has been recently identified. Herein is presented a case of RCC with Xp11.2 translocations/TFE3 gene fusions with unusual histological findings. A 68-year-old Japanese woman was incidentally found to have a renal mass on CT. Histological examination showed clear cell neoplasm with alveolar and papillary growth patterns. The nuclear atypia corresponded to Fuhrman grade 3. Additionally, smooth muscle stroma was observed and abnormal vessels showing a heterogeniety in thickness were also identified. On immunohistochemistry, neoplastic cells were diffusely positive for transcription factor E3 (TFE3) and Melan A, and focally positive for CD10 and RCC marker. The smooth muscle stroma was positive for alpha-smooth muscle actin and h-caldesmon, but reverse transcription-polymerase chain reaction of the tumor using frozen material could not detect any previously reported chimeric transcripts including ASPL-TFE3, PRCC-TFE3, CLTC-TFE3, PSF-TFE3 or NoNo-TFE3. G-band karyotype was unsuccessful. Pathologists should pay attention to the afore-described unusual stromal reaction of adult-onset RCC associated with Xp11.2 translocations/TFE3 gene fusions.

A patient with de-novo partial deletion of Xp (p11.4-pter) and partial duplication of 22q (q11.2-qter).

PubMed

Armour, Christine M; McGowan-Jordan, Jean; Lawrence, Sarah E; Bouchard, Amélie; Basik, Mark; Allanson, Judith E

2008-01-01

We report on a girl with partial deletion of Xp and partial duplication of 22q. Family studies demonstrate that both the patient's mother and her nonidentical twin sister carry the corresponding balanced translocation; 46,X,t(X;22)(p11.4;q11.2). This girl has developmental delay, microcephaly, mild dysmorphisms and hearing loss but otherwise shows few of the features described in individuals with duplications of the long arm of chromosome 22. She does manifest characteristics, such as short stature and biochemical evidence of ovarian failure, which are seen in partial or complete Xp deletions and Turner's syndrome.

Diagnostic pitfall on the histological spectrum of adult-onset renal carcinoma associated with Xp11.2 translocations/TFE3 gene fusions.

PubMed

Kuroda, Naoto; Katto, Kazunobu; Tanaka, Yukichi; Yamaguchi, Tadanori; Inoue, Kaori; Ohara, Masahiko; Mizuno, Keiko; Hes, Ondrej; Michal, Michal; Lee, Gang-Hong

2010-06-01

Renal cell carcinoma (RCC) associated with Xp11.2 translocation/TFE3 gene fusion recently has been found. In this article, we demonstrate an unusual features of such a case. A 73-year-old Japanese woman presented with macroscopic hematuria. The imaging examinations disclosed the renal tumor. Histological examination showed the finding of ASPL-TFE3 RCC, which was characterized by papillary, alveolar, or solid growth of voluminous cell with clear and eosinophilic cells, and stromal psammoma body and hyaline nodules. Additionally, shrunken nuclei, thick cell border, and perinuclear clearing characteristic of chromophobe renal cell carcinoma were observed in the alveolar growth area and the transitional zone between stromal hyalinization, and osseous metaplasia was identified. Immunohistochemically, nuclei of tumorous cell were diffusely positive for TFE3. A RT-PCR study revealed the ASPL-TFE3 chimeric transcript. Finally, pathologists should recognize that the histology of RCC associated with Xp11.2 translocation/TFE3 gene fusion may focally resemble that of chromophobe RCC, but TFE3 immunohistochemistry and molecular genetic study may be helpful in the differential diagnosis. Moreover, osseous metaplasia as well as psammoma bodies should be added to the histological spectrum of the stromal change in RCC associated with Xp11.2 translocations/TFE3 gene fusions.

Temsirolimus in the treatment of renal cell carcinoma associated with Xp11.2 translocation/TFE gene fusion proteins: a case report and review of literature.

PubMed

Parikh, Jigarkumar; Coleman, Teresa; Messias, Nidia; Brown, James

2009-12-28

Xp11.2 translocation renal cell carcinomas (TRCCs) are a rare family of tumors newly recognized by the World Health Organization (WHO) in 2004. These tumors result in the fusion of partner genes to the TFE3 gene located on Xp11.2. They are most common in the pediatric population, but have been recently implicated in adult renal cell carcinoma (RCC) presenting at an early age. TFE3-mediated direct transcriptional upregulation of the Met tyrosine kinase receptor triggers dramatic activation of downstream signaling pathways including the protein kinase B (Akt)/phosphatidylinositol-3 kinase (PI3K) and mammalian target of rapamycin (mTOR) pathways. Temsirolimus is an inhibitor of mammalian target of rapamycin (mTOR) kinase, a component of intracellular signaling pathways involved in the growth and proliferation of malignant cells. Here we present a case of a 22-year old female who has been treated with temsirolimus for her Xp11.2/TFE3 gene fusion RCC.

Temsirolimus in the treatment of renal cell carcinoma associated with Xp11.2 translocation/TFE gene fusion proteins: a case report and review of literature

PubMed Central

Parikh, Jigarkumar; Coleman, Teresa; Messias, Nidia; Brown, James

2009-01-01

Xp11.2 translocation renal cell carcinomas (TRCCs) are a rare family of tumors newly recognized by the World Health Organization (WHO) in 2004. These tumors result in the fusion of partner genes to the TFE3 gene located on Xp11.2. They are most common in the pediatric population, but have been recently implicated in adult renal cell carcinoma (RCC) presenting at an early age. TFE3-mediated direct transcriptional upregulation of the Met tyrosine kinase receptor triggers dramatic activation of downstream signaling pathways including the protein kinase B (Akt)/phosphatidylinositol-3 kinase (PI3K) and mammalian target of rapamycin (mTOR) pathways. Temsirolimus is an inhibitor of mammalian target of rapamycin (mTOR) kinase, a component of intracellular signaling pathways involved in the growth and proliferation of malignant cells. Here we present a case of a 22-year old female who has been treated with temsirolimus for her Xp11.2/TFE3 gene fusion RCC. PMID:21139932

Validation and utilization of a TFE3 break-apart FISH assay for Xp11.2 translocation renal cell carcinoma and alveolar soft part sarcoma.

PubMed

Pradhan, Dinesh; Roy, Somak; Quiroga-Garza, Gabriela; Cieply, Kathleen; Mahaffey, Alyssa L; Bastacky, Sheldon; Dhir, Rajiv; Parwani, Anil V

2015-09-29

Xp11.2 or TFE3 translocation renal cell carcinomas (RCC) and alveolar soft part sarcoma (ASPS) are characterized by chromosome translocations involving the Xp11.2 breakpoint resulting in transcription factor TFE3 gene fusions. The most common translocations documented in TFE3 RCCs are t(X;1) (p11.2;q21) and t(X;17) (p11.2;q25) which leads to fusion of TFE3 gene on Xp11.2 with PRCC or ASPL respectively. TFE3 immunohistochemistry (IHC) has been inconsistent over time due to background staining problems in part related to fixation issues. Karyotyping to detect TFE3 gene rearrangement requires typically unavailable fresh tissue. Reverse transcriptase-polymerase chain reaction (RT-PCR) is generally very challenging due to degradation of RNA in archival material. The study objective was to develop and validate a TFE3 break-apart fluorescence in situ hybridization (FISH) assay to confirm Xp11 translocation RCCs and ASPS. Representative sections of formalin-fixed paraffin-embedded tissue blocks were selected in 40 possible cases. Approximately 60 tumor cells were analyzed in the targeted region. The validation of TFE3 FISH was done with 11 negative and two positive cases. Cut off for a positive result was validated as >7.15 % positive nuclei with any pattern of break-apart signals. FISH evaluation was done blinded of the immunohistochemical or karyotype data. Three out of forty cases were positive for the TFE3 break-apart signals by FISH. The negative cases were reported as clear cell RCC with papillary features (10), clear cell RCC with sarcomatoid areas (2), Papillary RCC with clear cell areas (9), Chromophobe RCC (2), RCC, unclassified type (3) and renal medullary carcinoma (1). 3 of the negative cases were consultation cases for renal tumor with unknown histology. Seven negative cases were soft tissue tumor suspicious for ASPS. Our study validates the utility of TFE3 break-apart FISH on formalin-fixed paraffin-embedded tissue sections for diagnosis and confirmation of

Wind-Tunnel Development of Ailerons for the Curtiss XP-60 Airplanem Special Report

NASA Technical Reports Server (NTRS)

Rogallo, F. M.; Lowry, John G.

1942-01-01

An investigation was made in the LWAL 7- by 10-foot tunnel of internally balanced, sealed ailerons for the Curtiss XP-60 airplane. Ailerons with tabs and. with various amounts of balance were tested. Stick forces were estimated for several aileron arrangements including an arrangement recommended for the airplane. Flight tests of the recommended arrangement are discussed briefly in an appendix, The results of the wind-tunnel and flight tests indicate that the ailerons of large or fast airplanes may be satisfactorily balanced by the method developed.

Operating Systems.

ERIC Educational Resources Information Center

Denning, Peter J.; Brown, Robert L.

1984-01-01

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

Identification and characterization of a novel serine-threonine kinase gene from the Xp22 region.

PubMed

Montini, E; Andolfi, G; Caruso, A; Buchner, G; Walpole, S M; Mariani, M; Consalez, G; Trump, D; Ballabio, A; Franco, B

1998-08-01

Eukaryotic protein kinases are part of a large and expanding family of proteins. Through our transcriptional mapping effort in the Xp22 region, we have isolated and sequenced the full-length transcript of STK9, a novel cDNA highly homologous to serine-threonine kinases. A number of human genetic disorders have been mapped to the region where STK9 has been localized including Nance-Horan (NH) syndrome, oral-facial-digital syndrome type 1 (OFD1), and a novel locus for nonsyndromic sensorineural deafness (DFN6). To evaluate the possible involvement of STK9 in any of the above-mentioned disorders, a 2416-bp full-length cDNA was assembled. The entire genomic structure of the gene, which is composed of 20 coding exons, was determined. Northern analysis revealed a transcript larger than 9.5 kb in several tissues including brain, lung, and kidney. The mouse homologue (Stk9) was identified and mapped in the mouse in the region syntenic to human Xp. This location is compatible with the location of the Xcat mutant, which shows congenital cataracts very similar to those observed in NH patients. Sequence homologies, expression pattern, and mapping information in both human and mouse make STK9 a candidate gene for the above-mentioned disorders. Copyright 1998 Academic Press.

[Health management system in outpatient follow-up of kidney transplantation patients].

PubMed

Zhang, Hong; Xie, Jinliang; Yao, Hui; Liu, Ling; Tan, Jianwen; Geng, Chunmi

2014-07-01

To develop a health management system for outpatient follow-up of kidney transplant patients. Access 2010 database software was used to establish the health management system for kidney transplantation patients in Windows XP operating system. Database management and post-operation follow-up of the kidney transplantation patients were realized through 6 function modules including data input, data query, data printing, questionnaire survey, data export, and follow-up management. The system worked stably and reliably, and the data input was easy and fast. The query, the counting and printing were convenient. Health management system for patients after kidney transplantation not only reduces the work pressure of the follow-up staff, but also improves the efficiency of outpatient follow-up.

Genotype-phenotype characterization in 13 individuals with chromosome Xp11.22 duplications.

PubMed

Grams, Sarah E; Argiropoulos, Bob; Lines, Matthew; Chakraborty, Pranesh; Mcgowan-Jordan, Jean; Geraghty, Michael T; Tsang, Marilyn; Eswara, Marthand; Tezcan, Kamer; Adams, Kelly L; Linck, Leesa; Himes, Patricia; Kostiner, Dana; Zand, Dina J; Stalker, Heather; Driscoll, Daniel J; Huang, Taosheng; Rosenfeld, Jill A; Li, Xu; Chen, Emily

2016-04-01

We report 13 new individuals with duplications in Xp11.22-p11.23. The index family has one male and two female members in three generations with mild-severe intellectual disability (ID), speech delay, dysmorphic features, early puberty, constipation, and/or hand and foot abnormalities. Affected individuals were found to have two small duplications in Xp11.22 at nucleotide position (hg19) 50,112,063-50,456,458 bp (distal) and 53,160,114-53,713,154 bp (proximal). Collectively, these two regions include 14 RefSeq genes, prompting collection of a larger cohort of patients, in an attempt to delineate critical genes associated with the observed phenotype. In total, we have collected data on nine individuals with duplications overlapping the distal duplication region containing SHROOM4 and DGKK and eight individuals overlapping the proximal region including HUWE1. Duplications of HUWE1 have been previously associated with non-syndromic ID. Our data, with previously published reports, suggest that duplications involving SHROOM4 and DGKK may represent a new syndromic X-linked ID critical region associated with mild to severe ID, speech delay +/- dysarthria, attention deficit disorder, precocious puberty, constipation, and motor delay. We frequently observed foot abnormalities, 5th finger clinodactyly, tapering fingers, constipation, and exercise intolerance in patients with duplications of these two genes. Regarding duplications including the proximal region, our observations agree with previous studies, which have found associations with intellectual disability. In addition, expressive language delay, failure to thrive, motor delay, and 5th finger clinodactyly were also frequently observed in patients with the proximal duplication. © 2015 Wiley Periodicals, Inc.

Simulation of n-qubit quantum systems. III. Quantum operations

NASA Astrophysics Data System (ADS)

Radtke, T.; Fritzsche, S.

2007-05-01

During the last decade, several quantum information protocols, such as quantum key distribution, teleportation or quantum computation, have attracted a lot of interest. Despite the recent success and research efforts in quantum information processing, however, we are just at the beginning of understanding the role of entanglement and the behavior of quantum systems in noisy environments, i.e. for nonideal implementations. Therefore, in order to facilitate the investigation of entanglement and decoherence in n-qubit quantum registers, here we present a revised version of the FEYNMAN program for working with quantum operations and their associated (Jamiołkowski) dual states. Based on the implementation of several popular decoherence models, we provide tools especially for the quantitative analysis of quantum operations. Apart from the implementation of different noise models, the current program extension may help investigate the fragility of many quantum states, one of the main obstacles in realizing quantum information protocols today. Program summaryTitle of program: Feynman Catalogue identifier: ADWE_v3_0 Program summary URL:http://cpc.cs.qub.ac.uk/summaries/ADWE_v3_0 Program obtainable from: CPC Program Library, Queen's University of Belfast, N. Ireland Licensing provisions: None Operating systems: Any system that supports MAPLE; tested under Microsoft Windows XP, SuSe Linux 10 Program language used:MAPLE 10 Typical time and memory requirements: Most commands that act upon quantum registers with five or less qubits take ⩽10 seconds of processor time (on a Pentium 4 processor with ⩾2 GHz or equivalent) and 5-20 MB of memory. Especially when working with symbolic expressions, however, the memory and time requirements critically depend on the number of qubits in the quantum registers, owing to the exponential dimension growth of the associated Hilbert space. For example, complex (symbolic) noise models (with several Kraus operators) for multi-qubit systems

Operations management system

NASA Technical Reports Server (NTRS)

Brandli, A. E.; Eckelkamp, R. E.; Kelly, C. M.; Mccandless, W.; Rue, D. L.

1990-01-01

The objective of an operations management system is to provide an orderly and efficient method to operate and maintain aerospace vehicles. Concepts are described for an operations management system and the key technologies are highlighted which will be required if this capability is brought to fruition. Without this automation and decision aiding capability, the growing complexity of avionics will result in an unmanageable workload for the operator, ultimately threatening mission success or survivability of the aircraft or space system. The key technologies include expert system application to operational tasks such as replanning, equipment diagnostics and checkout, global system management, and advanced man machine interfaces. The economical development of operations management systems, which are largely software, will require advancements in other technological areas such as software engineering and computer hardware.

Extreme-phenotype genome-wide association study (XP-GWAS): a method for identifying trait-associated variants by sequencing pools of individuals selected from a diversity panel.

PubMed

Yang, Jinliang; Jiang, Haiying; Yeh, Cheng-Ting; Yu, Jianming; Jeddeloh, Jeffrey A; Nettleton, Dan; Schnable, Patrick S

2015-11-01

Although approaches for performing genome-wide association studies (GWAS) are well developed, conventional GWAS requires high-density genotyping of large numbers of individuals from a diversity panel. Here we report a method for performing GWAS that does not require genotyping of large numbers of individuals. Instead XP-GWAS (extreme-phenotype GWAS) relies on genotyping pools of individuals from a diversity panel that have extreme phenotypes. This analysis measures allele frequencies in the extreme pools, enabling discovery of associations between genetic variants and traits of interest. This method was evaluated in maize (Zea mays) using the well-characterized kernel row number trait, which was selected to enable comparisons between the results of XP-GWAS and conventional GWAS. An exome-sequencing strategy was used to focus sequencing resources on genes and their flanking regions. A total of 0.94 million variants were identified and served as evaluation markers; comparisons among pools showed that 145 of these variants were statistically associated with the kernel row number phenotype. These trait-associated variants were significantly enriched in regions identified by conventional GWAS. XP-GWAS was able to resolve several linked QTL and detect trait-associated variants within a single gene under a QTL peak. XP-GWAS is expected to be particularly valuable for detecting genes or alleles responsible for quantitative variation in species for which extensive genotyping resources are not available, such as wild progenitors of crops, orphan crops, and other poorly characterized species such as those of ecological interest. © 2015 The Authors The Plant Journal published by Society for Experimental Biology and John Wiley & Sons Ltd.

An Xp11.2 translocation renal cell carcinoma with SMARCB1 (INI1) inactivation in adult end-stage renal disease: a case report.

PubMed

Yu, Lu; Li, Jun; Xu, Sanpeng; Navia Miranda, Mariajose; Wang, Guoping; Duan, Yaqi

2016-10-12

Xp11.2 translocation/transcription factor E3 (TFE3) rearrangement renal cell carcinoma (RCC) is a rare subtype of RCC with limited clinical and pathological data. Here we present an unusual high-grade Xp11.2 translocation RCC with a rhabdoid feature and SMARCB1 (INI1) inactivation in a 40-year-old man with end-stage kidney disease. The histological examination of the dissected left renal tumor showed an organoid architecture of the eosinophilic or clear neoplastic cells with necrosis and high mitotic activity. In some areas, non-adhesive tumor cells with eccentric nuclei were observed. Immunohistochemically (IHC), the tumor cells are positive for TFE3 and the renal tubular markers (PAX2 and PAX8), and completely negative for SMARCB1, an oncosuppressor protein. Break-apart florescence in situ hybridization and reverse transcription polymerase chain reaction confirmed TFE3 rearrangement on Xp11.2 and the presence of ASPSCR1-TFE3 fusion gene. DNA sequencing revealed a frameshift mutation in exon 4 of SMARCB1 gene. It is important to recognize this rare RCC with both TFE3 rearrangement and SMARCB1 inactivation, as the prognosis and therapeutic strategies, particularly targeted therapies for such tumors, might be different.

Cyclic fatigue resistance of XP-endo Shaper compared with different nickel-titanium alloy instruments.

PubMed

Elnaghy, Amr; Elsaka, Shaymaa

2018-04-01

The aims of this study were to assess and compare the resistance to cyclic fatigue of XP-endo Shaper (XPS; FKG Dentaire, La Chaux-de-Fonds, Switzerland) instruments with TRUShape (TRS; Dentsply Tulsa Dental Specialties, Tulsa, OK, USA), HyFlex CM (HCM; Coltene, Cuyahoga Falls, OH, USA), Vortex Blue (VB; Dentsply Tulsa Dental Specialties), and iRace (iR; FKG Dentaire) nickel-titanium rotary instruments at body temperature. Size 30, 0.01 taper of XPS, size 30, 0.04 taper of HCM, VB, iR, and size 30, 0.06 taper of TRS instruments were immersed in saline at 37 ± 1 °C during cyclic fatigue testing. The instruments were tested with 60° angle of curvature and a 3-mm radius of curvature. The number of cycles to failure (NCF) was calculated and the length of the fractured segment was measured. Fractographic examination of the fractured surface was performed using a scanning electron microscope. The data were analyzed statistically using Kruskal-Wallis H test and Mann-Whitney U tests. Statistical significance was set at P < 0.05. XPS had a significantly greater NCF compared with the other instruments (P < 0.001). The topographic appearance of the fracture surfaces of tested instruments revealed ductile fracture of cyclic fatigue failure. XPS instruments exhibited greater cyclic fatigue resistance compared with the other tested instruments. XP-endo Shaper instruments could be used more safely in curved canals due to their higher fatigue resistance.

Clinical characteristics of XP11.2 translocation/TFE3 gene fusion renal cell carcinoma: a systematic review and meta-analysis of observational studies.

PubMed

Cheng, Xiangming; Gan, Weidong; Zhang, Gutian; Li, Xiaogong; Guo, Hongqian

2016-07-11

Renal cell carcinoma (RCC) associated with Xp11.2 translocation/TFE3 gene fusion (Xp11.2 RCC) is a rare subtype of RCC which is firstly described as a distinct entity in 2004 so that clinical characteristics of Xp11.2 RCC in different gender and age are unknown. The purpose of systematic review and meta-analysis is to provide a comprehensive assessment on them. MEDLINE, EMBASE and Cochrane databases were searched for studies which evaluate the clinical characteristics of Xp11.2 RCC. The literature published between July 2004 and May 2014 was searched. A total of 15 studies with 147 participants were included. The meta-analysis demonstrated that number of patients of all age in female was higher than in male with pooled OR of 3.93(95 % CI = 1.66-9.34). However, incidence of distant metastases (OR = 0.34, 95 % CI = 0.12-1.57) and lymphatic metastases (OR = 0.51, 95 % CI = 0.14-1.91), tumor stage (OR = 0.85, 95 % CI = 0.34-2.15) and overall survival (OS) (OR = 0.46, 95 % CI = 0.05-4.34) between male and female were comparable. Incidence in female was higher than in male with pooled OR of 5.13(95 % CI = 1.67-15.72) in adults, while in children no gender-related predominance (OR = 1.19, 95 % CI = 0.38-3.72) was observed. In addition, incidence of distant metastases (OR = 1.00, 95 % CI = 0.13-7.84) and lymphatic metastases (OR = 1.00, 95 % CI = 0.07-13.67) and tumor stage (OR = 1.94, 95 % CI = 0.20-19.03) between children and adults were comparable. Survival curves presented comparable outcomes between male and female (P = 0.707) as well as between children and adults (P = 0.383). Female patients with Xp11.2 RCC in adults exhibit a high incidence compared to male, but not in children. Comparable clinical characteristics including incidence of distant and lymphatic metastases, tumor stage and prognosis is presented between male and female as well as between children and adults.

Newly designed break-apart and ASPL-TFE3 dual-fusion FISH assay are useful in diagnosing Xp11.2 translocation renal cell carcinoma and ASPL-TFE3 renal cell carcinoma: a STARD-compliant article.

PubMed

Chen, Xiancheng; Yang, Yang; Gan, Weidong; Xu, Linfeng; Ye, Qing; Guo, Hongqian

2015-05-01

The diagnosis of Xp11.2 translocation renal cell carcinoma (tRCC), which relies on morphology and immunohistochemistry (IHC), is often either missed in the diagnosis or misdiagnosed. To improve the accuracy of diagnosis of Xp11.2 tRCC and ASPL-TFE3 renal cell carcinoma (RCC), we investigated newly designed fluorescence in situ hybridization (FISH) probes (diagnostic accuracy study).Based on the genetic characteristics of Xp11.2 tRCC and the ASPL-TFE3 RCC, a new break-apart TFE3 FISH probe and an ASPL-TFE3 dual-fusion FISH probe were designed and applied to 65 patients with RCC who were <45 years old or showed suspicious microscopic features of Xp11.2 tRCC in our hospital. To test the accuracy of the probes, we further performed reverse transcriptase-polymerase chain reaction (PCR) on 8 cases for which frozen tissues were available.Among the 65 cases diagnosed with RCC, TFE3 IHC was positive in 24 cases. Twenty-two cases were confirmed as Xp11.2 tRCC by break-apart TFE3 FISH, and 6 of these cases were further diagnosed as ASPL-TFE3 RCC by ASPL-TFE3 dual-fusion FISH detection. Importantly, reverse transcriptase-PCR showed concordant results with the results of FISH assay in the 8 available frozen cases.The break-apart and ASPL-TFE3 dual-fusion FISH assay can accurately detect the translocation of the TFE3 gene and ASPL-TFE3 fusion gene and can thus serve as a valid complementary method for diagnosing Xp11.2 tRCC and ASPL-TFE3 RCC.

Radiologic-pathologic correlation of renal cell carcinoma associated with Xp11.2 translocation.

PubMed

Koo, Hyun Jung; Choi, Hyuck Jae; Kim, Mi-hyun; Cho, Kyoung-Sik

2013-09-01

The prognosis of translocation RCCs in adult patients is relatively poor compared to that of other subtypes of RCCs. Although there have been several reports regarding radiologic findings of translocation RCC, studies with histologic correlation could help to understand the imaging features. To explore the correlation between radiologic and pathologic findings in Xp11.2 translocation renal cell carcinoma (RCC) and provide clues for translocation RCC diagnosis. CT scans of six patients (one man and five women; age range, 8-71 years; mean age, 34 years) with histologically-proven Xp11.2 translocation RCCs were retrospectively evaluated in consensus by two radiologists. Tumor size, presence of necrosis, hemorrhage, fat or calcification, enhancement patterns of the tumor, presence of lymphadenopathy, and distant metastases were evaluated. The average size of the tumors was 6 cm (range, 2.7-12 cm). All six tumors appeared as well-defined masses with areas of low attenuation representing hemorrhage or necrosis. Four tumors contained high attenuating solid portions, compared to the surrounding renal cortex seen on unenhanced images, where representing dense cellular component on microscopic examination. Peripheral rim enhancement pattern that correlated with histologic finding of a fibrous capsule was seen in five cases. In two patients who underwent kidney MR, the masses showed low signal intensity on T2-weighted images. One patient had lymphadenopathy. No distant metastasis was noted in any patient. Translocation RCC appeared as a well-defined mass that contain high attenuating solid portions on unenhanced images and low attenuating necrotic or hemorrhagic foci; the tumor also showed gradual peripheral rim enhancement due to a fibrous capsule surrounding the tumor.

Sunitinib-induced nephrotic syndrome in association with drug response in a patient with Xp11.2 translocation renal cell carcinoma.

PubMed

Liu, Yao-Chung; Chang, Peter Mu-Hsin; Liu, Chun-Yu; Yang, Chih-Yu; Chen, Ming-Han; Pan, Chin-Chen; Chen, Ming-Huang

2011-11-01

We report the case of a patient with metastatic renal cell carcinoma with Xp11.2 translocation/transcription factor E3 (TFE3) gene fusion who had presented with sunitinib-induced nephrotic syndrome in association with favorable and durable treatment response. The nephrotic syndrome was managed successfully by discontinuing sunitinib and symptomatic treatment. The 27-year-old female patient presenting with right upper abdominal pain was diagnosed with Xp11.2 translocation renal cell carcinoma on the right side with multiple pulmonary and hepatic metastases. She underwent radical nephrectomy and took a daily dose of 37.5 mg sunitinib. Partial response to sunitinib was achieved and maintained for 5 months, but when nephrotic syndrome occurred, drug intake was discontinued. The nephrotic syndrome gradually resolved around 2 months after discontinuation of sunitinib and medical management. Our case highlighted the favorable response of a particular non-clear cell type renal cell carcinoma to sunitinib and the specific toxicity associated with the antiangiogenic effect of sunitinib.

Pediatric renal cell carcinomas with Xp11.2 rearrangements are immunoreactive for hMLH1 and hMSH2 proteins.

PubMed

Rakheja, Dinesh; Kapur, Payal; Tomlinson, Gail E; Margraf, Linda R

2005-01-01

Alveolar soft part sarcoma and pediatric renal cell carcinoma share a similar chromosomal abnormality, t(X;17)(p11.2;q25). Recently, it has been suggested that the inactivation of DNA mismatch repair genes hMLH1 and hMSH2 may play an additional role in the pathogenesis of alveolar soft part sarcoma. Immunohistochemical expression of the proteins hMLH1 and hMSH2 is indicative of the activation status of the corresponding genes. We performed immunohistochemistry for hMLH1 and hMSH2 in 4 cases of pediatric renal cell carcinomas with Xp11.2 rearrangements. All cases showed nuclear immunoreactivity for both proteins, although the staining was patchy. Our study demonstrates that inactivation of the DNA mismatch repair genes hMLH1 and hMSH2 does not appear to play a role in the tumorigenesis of pediatric renal cell carcinomas with Xp11.2 rearrangements.

Stability and Control Characteristics of a 1/10-Scale Model of the McDonnell XP-85 Airplane While Attached to the Trapeze

NASA Technical Reports Server (NTRS)

Johnson, Joseph L.

1947-01-01

At the request of the Air Materiel Command, Army Air Forces, an investigation of the low-speed, power-off, stability and control characteristics of the McDonnell XP-85 airplane has been conducted in the Langley free-flight tunnel. The results of the portion of the investigation consisting of tests of a 1/10-scale model to study the stability of the XP-85 when attached to the trapeze and during retraction into the B-36 bomb bay are presented herein. In the power-off condition the stability was satisfactory with all oscillations well damped and the nose-restraining collar could be placed in position without difficulty. In a simulated power-on condition the model had a constant-amplitude rolling and sidewise motion and when the collar was layered, a violent motion resulted if the collar struck the model but failed to hold it in the proper manner. Folding of the wings and retraction into the bomb bay offered no problem once the airplane was properly held by the collar. It is recommended that the power be cut immediately after hooking on and that a restricting mechanism be incorporated in the center of the trapeze to eliminate the sidewise motion. It also appears desirable to have the retracting procedure controlled by the XP-85 pilot or an observer in the mother ship to insure that the parasite is in proper position after hooking up before bringing the collar down.

Network operating system

NASA Technical Reports Server (NTRS)

1985-01-01

Long-term and short-term objectives for the development of a network operating system for the Space Station are stated. The short-term objective is to develop a prototype network operating system for a 100 megabit/second fiber optic data bus. The long-term objective is to establish guidelines for writing a detailed specification for a Space Station network operating system. Major milestones are noted. Information is given in outline form.

X-Linked Syndrome of Polyendocrinopathy, Immune Dysfunction, and Diarrhea Maps to Xp11.23-Xq13.3

PubMed Central

Bennett, Craig L.; Yoshioka, Ritsuko; Kiyosawa, Hidenori; Barker, David F.; Fain, Pamela R.; Shigeoka, Ann O.; Chance, Phillip F.

2000-01-01

Summary We describe genetic analysis of a large pedigree with an X-linked syndrome of polyendocrinopathy, immune dysfunction, and diarrhea (XPID), which frequently results in death during infancy or childhood. Linkage analysis mapped the XPID gene to a 17-cM interval defined by markers DXS8083 and DXS8107 on the X chromosome, at Xp11.23-Xq13.3. The maximum LOD score was 3.99 (recombination fraction0) at DXS1235. Because this interval also harbors the gene for Wiskott-Aldrich syndrome (WAS), we investigated mutations in the WASP gene, as the molecular basis of XPID. Northern blot analysis detected the same relative amount and the same-sized WASP message in patients with XPID and in a control. Analysis of the WASP coding sequence, an alternate promoter, and an untranslated upstream first exon was carried out, and no mutations were found in patients with XPID. A C→T transition within the alternate translation start site cosegregated with the XPID phenotype in this family; however, the same transition site was detected in a normal control male. We conclude that XPID maps to Xp11.23-Xq13.3 and that mutations of WASP are not associated with XPID. PMID:10677306

New process of preparation, X-ray characterisation, structure and vibrational studies of a solid solution LiTiOAs 1-xP xO 4 (0⩽ x⩽1)

NASA Astrophysics Data System (ADS)

Chakir, M.; El Jazouli, A.; Chaminade, J. P.; Bouree, F.; de Waal, D.

2006-01-01

LiTiOAs 1-xP xO 4 (0⩽ x⩽1) compounds have been prepared using solutions of Li, Ti, As and P elements as starting products. Selected compositions have been investigated by powder X-ray or neutrons diffraction analysis, Raman and infrared spectroscopy. The structure of LiTiOAs 1-xP xO 4 ( x=0, 0.5 and 1) samples determined by Rietveld analysis is orthorhombic with Pnma space group. It is formed by a 3D network of TiO 6 octahedra and XO 4 ( X=As 1-xP x) tetrahedra where octahedral cavities are occupied by lithium atoms. TiO 6 octahedra are linked together by corners and form infinite chains along a-axis. Ti atoms are displaced from the centre of octahedral units in alternating short (1.700-1.709 Å) and long (2.301-2.275 Å) Ti-O bonds. Raman and infrared studies confirm the existence of Ti-O-Ti chains. Thermal stability of LiTiOAsO 4 has been reported.

Effect of p–d hybridization, structural distortion and cation electronegativity on electronic properties of ZnSnX{sub 2} (X=P, As, Sb) chalcopyrite semiconductors

DOE Office of Scientific and Technical Information (OSTI.GOV)

Mishra, S.; Ganguli, B., E-mail: biplabg@nitrkl.ac.in

2013-04-15

Significant effects of p–d hybridization, structural distortion and cation-electro-negativity are found on band gap in ZnSnX{sub 2} (X=P, As, Sb). Our study suggests these compounds to be direct band gap semiconductors with band gaps of 1.23, 0.68 and 0.19 eV respectively. Lattice constants, tetragonal distortion (η), anion displacement, bond lengths and bulk moduli are calculated by Density Functional Theory based on Tight binding Linear Muffin-Tin orbital method. Our result of structural properties is in good agreement with the available experimental and other theoretical results. Calculated band gaps also agree well with the experimental works within LDA limitation. Unlike other semiconductorsmore » in the group II–IV–V{sub 2}, there is a reduction in the band gap of 0.22, 0.20 and 0.24 eV respectively in ZnSnX{sub 2} (X=P, As, Sb) due to p–d hybridization. Structural distortion decreases band gap by 0.20, 0.12 and 0.10 eV respectively. We find that cation electronegativity effect is responsible for increasing the band gap relative to their binary analogs GaInP{sub 2}, InGaAs{sub 2} and GaInSb{sub 2} respectively and increment are 0.13, 0.04 and 0.13 eV respectively. - Graphical abstract: One unit cell of ZnSnX{sub 2} (X=P, As, Sb) chalcopyrite semiconductor. Semiconductors ZnSnX{sub 2} (X=P, As, Sb) are found to be direct band gap semiconductors with band gaps 1.23, 0.68 and 0.19 eV respectively. The quantitative estimate of effects of p–d hybridization, structural distortion and cation electronegativity shows band gaps change significantly due to these effects. Highlights: ► ZnSnX{sub 2} (X=P, As, Sb) are direct band gap semiconductors. ► These have band gaps of 1.23 eV, 0.68 eV and 0.19 eV respectively. ► The band gap reduction due to p–d hybridization is 13.41%, 18.51% and 40% respectively. ► Band gap reduction due to structural distortion is 12.12%, 11.11% and 16.66% respectively. ► Band gap increases 8.38%, 3.70% and 21

[Renal cell carcinoma associated with Xp11.2 translocations/TFE3 gene fusions: a study of 11 cases and review of literature].

PubMed

Rao, Qiu; Zhou, Xiao-jun; Wu, Bo; Ma, Heng-hui; Zhou, Hang-bo; Liu, Xiao-hong; Chen, Jie-yu

2007-04-01

To study the clinicopathologic features, differential diagnosis and prognosis of renal cell carcinoma associated with Xp11.2 translocations/TFE3 gene fusions. The histopathologic findings and immunophenotype of 11 cases of renal cell carcinoma associated with Xp11.2 translocations/TFE3 gene fusions were studied. Follow-up data (ranged from 10 to 112 months) were also analyzed. There were a total of 7 females and 4 males. The age of patients ranged from 8 to 26 years (mean = 16.3 years). The diameter of the tumors varied from 2.5 to 6.0 cm. Histologically, two morphologic patterns were seen. The first pattern consisted of alveolar, papillary or nested architecture. The tumor cells contained voluminous, clear to eosinophilic cytoplasm, distinct cell borders, vesicular chromatin, and prominent nucleoli. Psammoma bodies were frequently found and could be abundant. In contrast, the second pattern was composed of nested and compact architecture. The tumor cells possessed less abundant cytoplasm and inconspicuous nucleoli. Few psammoma bodies were detected. Immunohistochemical study showed that all cases strongly expressed TFE3, CD10 and P504s. Variable positivity for pan-cytokeratin, epithelial membrane antigen and vimentin was also noted. None of them expressed CK7, Ksp-cadherin and CD117. Renal cell carcinoma associated with Xp11.2 translocations/TFE3 gene fusions is a newly described but rarely encountered subtype of renal cell carcinoma. Pathologic diagnosis can be established when taken age of the patients, histopathologic findings and immunoreactivity for TFE3 protein into consideration.

Isolated 46,XY gonadal dysgenesis in two sisters caused by a Xp21.2 interstitial duplication containing the DAX1 gene.

PubMed

Barbaro, Michela; Oscarson, Mikael; Schoumans, Jacqueline; Staaf, Johan; Ivarsson, Sten A; Wedell, Anna

2007-08-01

Testis development is a tightly regulated process that requires an efficient and coordinated spatiotemporal action of many factors, and it has been shown that several genes involved in gonadal development exert a dosage effect. Chromosomal imbalances have been reported in several patients presenting with gonadal dysgenesis as part of severe dysmorphic phenotypes. We screened for submicroscopic DNA copy number variations in two sisters with an apparent normal 46,XY karyotype and female external genitalia due to gonadal dysgenesis, and in which mutations in known candidate genes had been excluded. By high-resolution tiling bacterial artificial chromosome array comparative genome hybridization, a submicroscopic duplication at Xp21.2 containing DAX1 (NR0B1) was identified. Using fluorescence in situ hybridization, multiple ligation probe amplification, and PCR, the rearrangement was further characterized. This revealed a 637-kb tandem duplication that in addition to DAX1 includes the four MAGEB genes, the hypothetical gene CXorf21, GK, and part of the MAP3K7IP3 gene. Sequencing and analysis of the breakpoint boundaries and duplication junction suggest that the duplication originated through a coupled homologous and nonhomologous recombination process. This represents the first duplication on Xp21.2 identified in patients with isolated gonadal dysgenesis because all previously described XY subjects with Xp21 duplications presented with gonadal dysgenesis as part of a more complex phenotype, including mental retardation and/or malformations. Thus, our data support DAX1 as a dosage sensitive gene responsible for gonadal dysgenesis and highlight the importance of considering DAX1 locus duplications in the evaluation of all cases of 46,XY gonadal dysgenesis.

Inversion (X)(p11.4q22) associated with Norrie disease in a four generation family.

PubMed

Pettenati, M J; Rao, P N; Weaver, R G; Thomas, I T; McMahan, M R

1993-03-01

We report on a 4-generation family in which Norrie disease occurs together with a pericentric inversion of the X chromosome in all affected males and carrier females. The breakpoint in the short arm of the X chromosome appears to be at the purported location of the Norrie disease gene. This is the second report of an association between Norrie disease and a chromosome aberration involving Xp11, and the first report of a specific gene disruption, thus physical gene location, due to a pericentric chromosome inversion.

Cutaneous metastases during an aggressive course of Xp11.2 translocation renal cell carcinoma in a teenager.

PubMed

Sudour-Bonnange, Helene; Leroy, Xavier; Chauvet, Marie-Pierre; Classe, Marion; Robin, P M; Leblond, Pierre

2014-09-01

We reported a rare case of cutaneous metastases of renal cell carcinoma (RCC) with an Xp11.2 translocation in a 15-year-old female. Clinicians should be aware of the possibility of this uncommon site of metastasis, which can indicate multivisceral dissemination of the disease. We discuss the feasibility and opportunity of treating such a patient with multiple line of tyrosine kinase inhibitor (TKI) targeting vascular endothelial and platelet-derived growth factor receptors. © 2014 Wiley Periodicals, Inc.

MI-ANFIS: A Multiple Instance Adaptive Neuro-Fuzzy Inference System

DTIC Science & Technology

2015-08-02

AUTHORS 7. PERFORMING ORGANIZATION NAMES AND ADDRESSES 15. SUBJECT TERMS b. ABSTRACT 2 . REPORT TYPE 17. LIMITATION OF ABSTRACT 15. NUMBER OF...fuzzy logic can deal with the uncertainty of human cognition [ 2 ]. ANFIS offers an alternative to rules’ identification. While Mamdani [3] and Sugeno [4...dimensional vector with elements xpjk corresponding to features, i.e., Bp =  xp11 xp12 . . . xp1D xp21 xp22 . . . xp2D ... ... . . . ... xpMp1

Unusual X-chromosome inactivation pattern in patients with Xp11.23-p11.22 duplication: Report and review.

PubMed

Di-Battista, Adriana; Meloni, Vera Ayres; da Silva, Magnus Dias; Moysés-Oliveira, Mariana; Melaragno, Maria Isabel

2016-12-01

In females carrying structural rearrangements of an X-chromosome, cells with the best dosage balance are preferentially selected, frequently resulting in a skewed inactivation pattern and amelioration of the phenotype. The Xp11.23-p11.22 region is involved in a recently described microduplication syndrome associated with severe clinical consequences in males and females, causing intellectual disability, behavior problems, epilepsy with electroencephalogram anomalies, minor facial anomalies, and early onset of puberty. Female carriers usually present an unusual X-chromosome inactivation pattern in favor of the aberrant chromosome, resulting in functional disomy of the duplicated segment. Here, we describe a girl carrying a de novo ∼9.7 Mb Xp11.3-p11.22 duplication of paternal origin and skewed X-chromosome inactivation pattern of the normal X-chromosome. We reviewed other cases previously reported and determined the minimal critical region possibly responsible for this unusual inactivation pattern. The critical region encompasses 36 RefSeq genes, including at least 10 oncogenes and/or genes related to the cell cycle control. We discuss the molecular mechanisms that underlie the positive selection of the cells with the active duplicated chromosome. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

Multitasking operating systems for microprocessors

DOE Office of Scientific and Technical Information (OSTI.GOV)

Cramer, T.

1981-01-01

Microprocessors, because of their low cost, low power consumption, and small size, have caused an explosion in the number of innovative computer applications. Although there is a great deal of variation in microprocessor applications software, there is relatively little variation in the operating-system-level software from one application to the next. Nonetheless, operating system software, especially when multitasking is involved, can be very time consuming and expensive to develop. The major microprocessor manufacturers have acknowledged the need for operating systems in microprocessor applications and are now supplying real-time multitasking operating system software that is adaptable to a wide variety of usermore » systems. Use of this existing operating system software will decrease the number of redundant operating system development efforts, thus freeing programmers to work on more creative and productive problems. This paper discusses the basic terminology and concepts involved with multitasking operating systems. It is intended to provide a general understanding of the subject, so that the reader will be prepared to evaluate specific operating system software according to his or her needs. 2 references.« less

Wolf-Hirschhorn Syndrome with Epibulbar Dermoid: An Unusual Association in a Patient with 4p Deletion and Functional Xp Disomy.

PubMed

Bragagnolo, Silvia; Colovati, Mileny E S; Guilherme, Roberta S; Dantas, Anelisa G; de Souza, Malú Zamariolli; de Soares, Maria F; Melaragno, Maria I; Perez, Ana B

2016-01-01

Wolf-Hirschhorn syndrome (WHS) is a contiguous gene and multiple malformation syndrome that results from a deletion in the 4p16.3 region. We describe here a 6-month-old girl that presented with WHS features but also displayed unusual findings, such as epibulbar dermoid in the left eye, ear tags, and left microtia. Although on G-banding her karyotype appeared to be normal, chromosomal microarray analysis revealed an ∼13-Mb 4p16.3p15.33 deletion and an ∼9-Mb Xp22.33p22.31 duplication, resulting from a balanced maternal t(X;4)(p22.31;p15.33) translocation. The patient presented with functional Xp disomy due to an unbalanced X-autosome translocation, a rare cytogenetic finding in females with unbalanced rearrangements. Sequencing of both chromosome breakpoints detected no gene disruption. To the best of our knowledge, this is the first patient described in the literature with WHS and epibulbar dermoid, a typical characteristic of the oculoauriculovertebral spectrum (OAVS). Our data suggest that possible candidate genes for OAVS may have been deleted along with the WHS critical region. © 2016 S. Karger AG, Basel.

Mapping X-linked ophthalmic diseases. IV. Provisional assignment of the locus for X-linked congenital cataracts and microcornea (the Nance-Horan syndrome) to Xp22.2-p22.3.

PubMed

Lewis, R A; Nussbaum, R L; Stambolian, D

1990-01-01

The Nance-Horan syndrome (NHS) is an infrequent X-linked disorder typified by dense congenital central cataracts, microcornea, anteverted and simplex pinnae, brachymetacarpalia, and numerous dental anomalies. The regional location of the genetic mutation causing NHS is unknown. The authors applied the modern molecular techniques of analysis of restriction fragment length polymorphisms to five multigenerational kindreds in which NHS segregated. Provisional linkage is established to two DNA markers--DXS143 at Xp22.3-p22.2 and DXS43 at Xp22.2. Regional localization of NHS will provide potential antenatal diagnosis in families at risk for the disease and will enhance understanding of the multifaceted genetic defects.

Functional relationships of landfill and landraise capacity with design and operation parameters.

PubMed

Aivaliotis, Vassilis; Dokas, Ioannis; Hatzigiannakou, Maria; Panagiotakopoulos, Demetrios

2004-08-01

Solid waste management presses for effective landfill design and operation. While planning and operating a landfill (LF) or a landraise (LR), choices need to be made regarding: (1) LF-LR morphology (base shape, side slopes, final cover thickness, LR/LF height/depth); (2) cell geometry (height, length, slopes); and (3) operation parameters (waste density, working face length, cover thicknesses). These parameters affect LF/LR capacity, operation lifespan and construction/ operation costs. In this paper, relationships are generated between capacity (C, space available for waste) and the above parameters. Incorporating real data into simulation kgamma A1.38, runs, two types of functions are developed: first, C = where A is the LF/LR base area size and kgamma a base shape-dependent coefficient; and second, C = alpha(p,gamma,A) + delta(p,gamma,A)Xp for every parameter p, where Xp is the value of p and alpha(p,gamma,A) and delta(p,gamma,A) are parameter- and base (shape/size)-specific coefficients. Moreover, the relationship between LF depth and LR height that balances excavation volume with cover material, is identified. Another result is that, for a symmetrical combination of LF/LR, with base surface area shape between square and 1:2 orthogonal, and final density between 500 and 800 kg m(-3), waste quantity placed ranges from 1.76A1.38 to 2.55A1.38 tons. The significance of such functions is obvious, as they allow the analyst to investigate alternative LF/LR schemes and make trade-off analyses.

Cardiopulmonary bypass-assisted surgery for the treatment of Xp11.2 translocation/TFE3 gene fusion renal cell carcinoma with a tumor thrombus within the inferior vena cava: A case report.

PubMed

Zhu, Guanchen; Qiu, Xuefeng; Chen, Xianchen; Liu, Guangxiang; Zhang, Gutian; Gan, Weidong; Guo, Hongqian

2015-12-01

Renal cell carcinoma (RCC) accounts for 85-90% of kidney cancers, which in turn account for 2-3% of all malignant tumors in adults. Xp11.2 translocation/TFE3 gene fusion RCC is currently classified as a distinct type of RCC. RCC is capable of invading the renal vein and inferior vena cava to form a tumor thrombus. The incidence of RCC with tumor thrombi within the renal vein or inferior vena cava is 7-10% in China. In the present case report, the patient underwent radical resection of the renal tumor and removal of the tumor thrombus, assisted by cardiopulmonary bypass, for the treatment of Xp11.2 translocation/TFE3 gene fusion RCC. The patient was followed-up for 12 months subsequent to treatment. The patient's renal function remained within the normal range, and computed tomography examination revealed no evidence of disease recurrence or metastases. The present case report aimed to provide a reference for the development of guidelines for the diagnosis and treatment of Xp11.2 translocation/TFE3 gene fusion RCC.

Cardiopulmonary bypass-assisted surgery for the treatment of Xp11.2 translocation/TFE3 gene fusion renal cell carcinoma with a tumor thrombus within the inferior vena cava: A case report

PubMed Central

ZHU, GUANCHEN; QIU, XUEFENG; CHEN, XIANCHEN; LIU, GUANGXIANG; ZHANG, GUTIAN; GAN, WEIDONG; GUO, HONGQIAN

2015-01-01

Renal cell carcinoma (RCC) accounts for 85–90% of kidney cancers, which in turn account for 2–3% of all malignant tumors in adults. Xp11.2 translocation/TFE3 gene fusion RCC is currently classified as a distinct type of RCC. RCC is capable of invading the renal vein and inferior vena cava to form a tumor thrombus. The incidence of RCC with tumor thrombi within the renal vein or inferior vena cava is 7–10% in China. In the present case report, the patient underwent radical resection of the renal tumor and removal of the tumor thrombus, assisted by cardiopulmonary bypass, for the treatment of Xp11.2 translocation/TFE3 gene fusion RCC. The patient was followed-up for 12 months subsequent to treatment. The patient's renal function remained within the normal range, and computed tomography examination revealed no evidence of disease recurrence or metastases. The present case report aimed to provide a reference for the development of guidelines for the diagnosis and treatment of Xp11.2 translocation/TFE3 gene fusion RCC. PMID:26788164

Molecular cytogenetic analysis for TFE3 rearrangement in Xp11.2 renal cell carcinoma and alveolar soft part sarcoma: validation and clinical experience with 75 cases.

PubMed

Hodge, Jennelle C; Pearce, Kathryn E; Wang, Xiaoke; Wiktor, Anne E; Oliveira, Andre M; Greipp, Patricia T

2014-01-01

Renal cell carcinoma with TFE3 rearrangement at Xp11.2 is a distinct subtype manifesting an indolent clinical course in children, with recent reports suggesting a more aggressive entity in adults. This subtype is morphologically heterogeneous and can be misclassified as clear cell or papillary renal cell carcinoma. TFE3 is also rearranged in alveolar soft part sarcoma. To aid in diagnosis, a break-apart strategy fluorescence in situ hybridization (FISH) probe set specific for TFE3 rearrangement and a reflex dual-color, single-fusion strategy probe set involving the most common TFE3 partner gene, ASPSCR1, were validated on formalin-fixed, paraffin-embedded tissues from nine alveolar soft part sarcoma, two suspected Xp11.2 renal cell carcinoma, and nine tumors in the differential diagnosis. The impact of tissue cut artifact was reduced through inclusion of a chromosome X centromere control probe. Analysis of the UOK-109 renal carcinoma cell line confirmed the break-apart TFE3 probe set can distinguish the subtle TFE3/NONO fusion-associated inversion of chromosome X. Subsequent extensive clinical experience was gained through analysis of 75 cases with an indication of Xp11.2 renal cell carcinoma (n=54), alveolar soft part sarcoma (n=13), perivascular epithelioid cell neoplasms (n=2), chordoma (n=1), or unspecified (n=5). We observed balanced and unbalanced chromosome X;17 translocations in both Xp11.2 renal cell carcinoma and alveolar soft part sarcoma, supporting a preference but not a necessity for the translocation to be balanced in the carcinoma and unbalanced in the sarcoma. We further demonstrate the unbalanced separation is atypical, with TFE3/ASPSCR1 fusion and loss of the derivative X chromosome but also an unanticipated normal X chromosome gain in both males and females. Other diverse sex chromosome copy number combinations were observed. Our TFE3 FISH assay is a useful adjunct to morphologic analysis of such challenging cases and will be applicable to

[Xp11.2 translocation renal carcinoma in adults over 50 years of age: about four cases].

PubMed

Arnoux, V; Long, J-A; Fiard, G; Pasquier, D; Bensaadi, L; Terrier, N; Rambeaud, J-J; Descotes, J-L

2012-11-01

To describe demographic, therapeutic and follow-up data of four cases of renal cell carcinoma with Xp11.2 translocation in adults older than 50 years of age. Between January 2008 and December 2011, 170 patients underwent surgery for renal cell carcinoma in our center. Systematic histopathologic analysis of specimen removed was performed. Complementary immunohistochemical analysis was performed only in cases with uncertain diagnosis or in patients younger than 40 years of age. Among these 170 patients with a median age of 59years old (21-89), immunohistochemistry helped find a TFE3 translocation in four cases (2.4%). There were three women and one man of 53, 71, 75 and 86years old respectively. One patient was metastatic at diagnosis. Radical nephrectomy was first performed in all cases. TNM staging was T3aN2R0, T3bN0R0, T2N2R0 and T3aN2R2, with a Furhman grade of 4. Two patients progressed with metastasis 5 and 7months after surgery, and two with lymphatic invasion 2 and 9months after nephrectomy. One patient died during follow-up. Xp11.2 translocation renal cell carcinoma was uncommon after 50years of age in our series, but probably under estimated. It seemed to be associated with a poor prognosis. Larger studies must be performed to optimize its specific treatment. Copyright © 2012 Elsevier Masson SAS. All rights reserved.

A multiprocessor operating system simulator

DOE Office of Scientific and Technical Information (OSTI.GOV)

Johnston, G.M.; Campbell, R.H.

1988-01-01

This paper describes a multiprocessor operating system simulator that was developed by the authors in the Fall of 1987. The simulator was built in response to the need to provide students with an environment in which to build and test operating system concepts as part of the coursework of a third-year undergraduate operating systems course. Written in C++, the simulator uses the co-routine style task package that is distributed with the AT and T C++ Translator to provide a hierarchy of classes that represents a broad range of operating system software and hardware components. The class hierarchy closely follows thatmore » of the Choices family of operating systems for loosely and tightly coupled multiprocessors. During an operating system course, these classes are refined and specialized by students in homework assignments to facilitate experimentation with different aspects of operating system design and policy decisions. The current implementation runs on the IBM RT PC under 4.3bsd UNIX.« less

A Multiprocessor Operating System Simulator

NASA Technical Reports Server (NTRS)

Johnston, Gary M.; Campbell, Roy H.

1988-01-01

This paper describes a multiprocessor operating system simulator that was developed by the authors in the Fall semester of 1987. The simulator was built in response to the need to provide students with an environment in which to build and test operating system concepts as part of the coursework of a third-year undergraduate operating systems course. Written in C++, the simulator uses the co-routine style task package that is distributed with the AT&T C++ Translator to provide a hierarchy of classes that represents a broad range of operating system software and hardware components. The class hierarchy closely follows that of the 'Choices' family of operating systems for loosely- and tightly-coupled multiprocessors. During an operating system course, these classes are refined and specialized by students in homework assignments to facilitate experimentation with different aspects of operating system design and policy decisions. The current implementation runs on the IBM RT PC under 4.3bsd UNIX.

In vitro Repair of Oxidative DNA Damage by Human Nucleotide Excision Repair System: Possible Explanation for Neurodegeneration in Xeroderma Pigmentosum Patients

NASA Astrophysics Data System (ADS)

Reardon, Joyce T.; Bessho, Tadayoshi; Kung, Hsiang Chuan; Bolton, Philip H.; Sancar, Aziz

1997-08-01

Xeroderma pigmentosum (XP) patients fail to remove pyrimidine dimers caused by sunlight and, as a consequence, develop multiple cancers in areas exposed to light. The second most common sign, present in 20-30% of XP patients, is a set of neurological abnormalities caused by neuronal death in the central and peripheral nervous systems. Neural tissue is shielded from sunlight-induced DNA damage, so the cause of neurodegeneration in XP patients remains unexplained. In this study, we show that two major oxidative DNA lesions, 8-oxoguanine and thymine glycol, are excised from DNA in vitro by the same enzyme system responsible for removing pyrimidine dimers and other bulky DNA adducts. Our results suggest that XP neurological disease may be caused by defective repair of lesions that are produced in nerve cells by reactive oxygen species generated as by-products of an active oxidative metabolism.

Xp11.22 deletions encompassing CENPVL1, CENPVL2, MAGED1 and GSPT2 as a cause of syndromic X-linked intellectual disability.

PubMed

Grau, Christina; Starkovich, Molly; Azamian, Mahshid S; Xia, Fan; Cheung, Sau Wai; Evans, Patricia; Henderson, Alex; Lalani, Seema R; Scott, Daryl A

2017-01-01

By searching a clinical database of over 60,000 individuals referred for array-based CNV analyses and online resources, we identified four males from three families with intellectual disability, developmental delay, hypotonia, joint hypermobility and relative macrocephaly who carried small, overlapping deletions of Xp11.22. The maximum region of overlap between their deletions spanned ~430 kb and included two pseudogenes, CENPVL1 and CENPVL2, whose functions are not known, and two protein coding genes-the G1 to S phase transition 2 gene (GSPT2) and the MAGE family member D1 gene (MAGED1). Deletions of this ~430 kb region have not been previously implicated in human disease. Duplications of GSPT2 have been documented in individuals with intellectual disability, but the phenotypic consequences of a loss of GSPT2 function have not been elucidated in humans or mouse models. Changes in MAGED1 have not been associated with intellectual disability in humans, but loss of MAGED1 function is associated with neurocognitive and neurobehavioral phenotypes in mice. In all cases, the Xp11.22 deletion was inherited from an unaffected mother. Studies performed on DNA from one of these mothers did not show evidence of skewed X-inactivation. These results suggest that deletions of an ~430 kb region on chromosome Xp11.22 that encompass CENPVL1, CENPVL2, GSPT2 and MAGED1 cause a distinct X-linked syndrome characterized by intellectual disability, developmental delay, hypotonia, joint hypermobility and relative macrocephaly. Loss of GSPT2 and/or MAGED1 function may contribute to the intellectual disability and developmental delay seen in males with these deletions.

The embedded operating system project

NASA Technical Reports Server (NTRS)

Campbell, R. H.

1984-01-01

This progress report describes research towards the design and construction of embedded operating systems for real-time advanced aerospace applications. The applications concerned require reliable operating system support that must accommodate networks of computers. The report addresses the problems of constructing such operating systems, the communications media, reconfiguration, consistency and recovery in a distributed system, and the issues of realtime processing. A discussion is included on suitable theoretical foundations for the use of atomic actions to support fault tolerance and data consistency in real-time object-based systems. In particular, this report addresses: atomic actions, fault tolerance, operating system structure, program development, reliability and availability, and networking issues. This document reports the status of various experiments designed and conducted to investigate embedded operating system design issues.

Renal cell carcinoma with Xp11.2 translocation in a 7-year-old boy.

PubMed

Jayasinghe, C; Siegler, N; Leuschner, I; Fleischhack, G; Born, M; Müller, A M

2010-05-01

More than 90% of pediatric renal tumors are nephroblastomas while renal cell carcinomas (RCC) are rare in children (< 5%). According to the clinical diagnoses of a nephroblastoma stage IV a 7-year-old boy with a kidney tumor and peripheral pulmonary lesion was preoperatively treated for 8 weeks with Vincristine, Actinomycin D and Adriamycin. The resected kidney displayed a RCC with Xp11.2 translocation. There was no tumor regression and the pulmonary lesion was no longer detectable. Hence chemotherapy was put to a halt. Fine needle aspiration biopsy (FNA) would have allowed to adjust the tumor subtype. Prognosis of pediatric RCC with translocation seems more favourable than without translocation though definitive evidence will only be possible by documentation in a clinical diagnose-related register.

Renal Cell Carcinoma Associated With Xp11.2 Translocation/TFE3 Gene-fusion: A Long Response to mammalian target of rapamycin (mTOR) Inhibitors.

PubMed

Rua Fernández, Oliver R; Escala Cornejo, Roberto; Navarro Martín, Miguel; García Muñoz, María; Antunez Plaza, Patricia; García Dominguez, Aracely Rocío; Cruz Hernández, Juan J

2018-04-24

To demonstrate that patients with Xp11.2/TFE3 gene-fusion translocation renal cell carcinoma (RCC), despite having an aggressive course in young adults, could have valid treatment options such as mammalian target of rapamycin (mTOR) inhibitors with good outcomes. Furthermore, to explain possible mechanisms of action of mTOR inhibitors in this type of RCC. We report a case of a 44-year-old man who has been treated with everolimus for a Xp11.2 translocation/TFE3 gene-fusion RCC after 2 previous failed treatments with tyrosine kinase inhibitor. During the follow-up, we evaluated type and duration of response with everolimus. The patient obtained a long-lasting response of disease of 25 months with everolimus without any symptom. We believe that mTOR inhibitors could be a good line option treatment to consider for this type of patients. Copyright © 2018 Elsevier Inc. All rights reserved.

Operator Support System Design forthe Operation of RSG-GAS Research Reactor

NASA Astrophysics Data System (ADS)

Santoso, S.; Situmorang, J.; Bakhri, S.; Subekti, M.; Sunaryo, G. R.

2018-02-01

The components of RSG-GAS main control room are facing the problem of material ageing and technology obsolescence as well, and therefore the need for modernization and refurbishment are essential. The modernization in control room can be applied on the operator support system which bears the function in providing information for assisting the operator in conducting diagnosis and actions. The research purpose is to design an operator support system for RSG-GAS control room. The design was developed based on the operator requirement in conducting task operation scenarios and the reactor operation characteristics. These scenarios include power operation, low power operation and shutdown/scram reactor. The operator support system design is presented in a single computer display which contains structure and support system elements e.g. operation procedure, status of safety related components and operational requirements, operation limit condition of parameters, alarm information, and prognosis function. The prototype was developed using LabView software and consisted of components structure and features of the operator support system. Information of each component in the operator support system need to be completed before it can be applied and integrated in the RSG-GAS main control room.

Cloning and characterization of a novel zinc finger gene in Xp11.2

DOE Office of Scientific and Technical Information (OSTI.GOV)

Derry, J.M.J.; Jess, U.; Francke, U.

1995-11-20

During a systematic search for open reading frames in chromosome band Xp11.2, a novel gene (ZNF157) that encodes a putative 506-amino-acid protein with the sequence characteristics of a zinc-finger-containing transcription factor was isolated. ZNF157 is encoded by four exons distributed over >20 kb of genomic DNA. The second and third exons contain sequences similar to those of the previously described KRAB-A and KRAB-B domains, motifs that have been shown to mediate transcriptional repression in other members of the protein family. A fourth exon contains 12 zinc finger DNA binding motifs and finger linking regions characteristic of ZNF proteins of themore » Krueppel family. ZNF157 maps to the telomeric end of a cluster of ZNF genes that includes ZNF21, ZNF41, and ZNF81. 19 refs., 2 figs.« less

Xp 11.2 translocation renal carcinoma in young adults; recently classified distinct subtype

PubMed Central

Kmetec, Andrej; Jeruc, Jera

2014-01-01

Background XP11.2 renal translocation carcinomas are often encountered in paediatric group of patients where they are believed to be rather indolent. They are rare but more aggressive in young adults. They are slow growing, sometimes without characteristic symptoms and their biologic behaviour is uncertain. Case report We report two cases of this type of tumour in Slovenian young adult males with long and unusual history. Tumours were confirmed imunohistologically by positive reaction for CD10, P504S and TFE3. Conclusions According to the indications in the literature prognosis of these tumours in young adults depends upon the stage. It seems that cysts, haematomas and necrosis around the kidney are often encountered in these tumours. In advanced stage with lymph nodes involvement or distant metastases, the prognosis is poor. Surgery seems to be basic mode of therapy. PMID:24991210

Optical phonon modes and polaron related parameters in GaxIn1-xP

NASA Astrophysics Data System (ADS)

Bouarissa, N.; Algarni, H.; Al-Hagan, O. A.; Khan, M. A.; Alhuwaymel, T. F.

2018-02-01

Based on a pseudopotential approach under the virtual crystal approximation that includes the effect of compositional disorder, the optical lattice vibration frequencies and polaron related parameters in zinc-blende GaxIn1-xP have been studied. Our findings showed generally reasonably good accord with data in the literature. Other case, our results are predictions. The composition dependence of longitudinal optical (LO) and transverse optical (TO) phonon modes, LO-TO splittings, Frӧhlich coupling parameter, Debye temperature of LO phonon frequency, and polaron effective mass has been analyzed and discussed. While a non-monotonic behavior has been noticed for the LO and TO phonon frequencies versus Ga concentration x, a monotonic behavior has been observed for the rest of the features of interest. The information derived from this investigation may be useful for optoelectronic technological applications.

The embedded operating system project

NASA Technical Reports Server (NTRS)

Campbell, R. H.

1985-01-01

The design and construction of embedded operating systems for real-time advanced aerospace applications was investigated. The applications require reliable operating system support that must accommodate computer networks. Problems that arise in the construction of such operating systems, reconfiguration, consistency and recovery in a distributed system, and the issues of real-time processing are reported. A thesis that provides theoretical foundations for the use of atomic actions to support fault tolerance and data consistency in real-time object-based system is included. The following items are addressed: (1) atomic actions and fault-tolerance issues; (2) operating system structure; (3) program development; (4) a reliable compiler for path Pascal; and (5) mediators, a mechanism for scheduling distributed system processes.

Review of Flight Tests of NACA C and D Cowlings on the XP-42 Airplane

NASA Technical Reports Server (NTRS)

Johnston, J Ford

1943-01-01

Results of flight tests of the performance and cooling characteristics of three NACA D cowlings and of a conventional NACA D cowling on the XP-42 airplane are summarized and compared. The D cowling is, in general, characterized by the use of an annular inlet and diffuser section for the engine-cooling air. The D cowlings tested were a long-nose high-inlet-velocity cowling, a short-nose high-inlet-velocity cowling, and a short-nose low inlet-velocity cowling. The use of wide-chord propeller cuffs or an axial-flow fan with the D cowlings increased the cooling pressure recoveries in the climb condition at the expense of some of the improvement in speed.

Development of the prototype data management system of the solar H-alpha full disk observation

NASA Astrophysics Data System (ADS)

Wei, Ka-Ning; Zhao, Shi-Qing; Li, Qiong-Ying; Chen, Dong

2004-06-01

The Solar Chromospheric Telescope in Yunnan Observatory generates about 2G bytes fits format data per day. Huge amounts of data will bring inconvenience for people to use. Hence, data searching and sharing are important at present. Data searching, on-line browsing, remote accesses and download are developed with a prototype data management system of the solar H-alpha full disk observation, and improved by the working flow technology. Based on Windows XP operating system and MySQL data management system, a prototype system of browse/server model is developed by JAVA and JSP. Data compression, searching, browsing, deletion need authority and download in real-time have been achieved.

Secure Video Surveillance System Acquisition Software

DOE Office of Scientific and Technical Information (OSTI.GOV)

2009-12-04

The SVSS Acquisition Software collects and displays video images from two cameras through a VPN, and store the images onto a collection controller. The software is configured to allow a user to enter a time window to display up to 2 1/2, hours of video review. The software collects images from the cameras at a rate of 1 image per second and automatically deletes images older than 3 hours. The software code operates in a linux environment and can be run in a virtual machine on Windows XP. The Sandia software integrates the different COTS software together to build themore » video review system.« less

Magnetic resonance imaging and computed tomography characteristics of renal cell carcinoma associated with Xp11.2 translocation/TFE3 gene fusion.

PubMed

Wang, Wei; Ding, Jianhui; Li, Yuan; Wang, Chaofu; Zhou, Liangping; Zhu, Hui; Peng, Weijun

2014-01-01

To characterize Xp11.2 translocation renal cell carcinoma (RCC) using magnetic resonance imaging (MRI) and computed tomography (CT). This study retrospectively collected the MRI and CT data of twelve patients with Xp11.2 translocation RCC confirmed by pathology. Nine cases underwent dynamic contrast-enhanced MRI (DCE-MRI) and 6 cases underwent CT, of which 3 cases underwent MRI and CT simultaneously. The MRI and CT findings were analyzed in regard to tumor position, size, hemorrhagic, cystic or necrotic components, calcification, tumor density, signal intensity and enhancement features. The age of the 12 patients ranged from 13 to 46 years (mean age: 23 years). T2WI revealed heterogeneous intensity, hyper-intensity, and slight hypo-intensity in 6 cases, 2 cases, and 1 case, respectively. On DCE-MR images, mild, moderate, and marked rim enhancement of the tumor in the corticomedullary phase (CMP) were observed in 1, 6, and 2 cases, respectively. The tumor parenchyma showed iso-attenuation (n = 4) or slight hyper-attenuation (n = 1) compared to the normal renal cortex on non-contrast CT images. Imaging findings were suggestive of hemorrhage (n = 4) or necrosis (n = 8) in the tumors, and there was evidence of calcification in 8 cases by CT (n = 3) and pathology (n = 8). On dynamic contrast-enhanced CT images, 3 cases and 1 case manifested moderate and strong CMP enhancement, respectively. Nine tumors by MRI and 4 tumors by CT showed prolonged enhancement. Three neoplasms presented at stage I, 2 at stage II, 3 at stage III, and 4 at stage IV according the 2010 AJCC staging criteria. XP11.2 translocation RCC should be considered when a child or young adult patient presents with a renal tumor with heterogeneous features such as hemorrhage, necrosis, cystic changes, and calcification on CT and MRI and/or is accompanied by metastatic evidence.

Magnetic Resonance Imaging and Computed Tomography Characteristics of Renal Cell Carcinoma Associated with Xp11.2 Translocation/TFE3 Gene Fusion

PubMed Central

Li, Yuan; Wang, Chaofu; Zhou, Liangping; Zhu, Hui; Peng, Weijun

2014-01-01

Purpose To characterize Xp11.2 translocation renal cell carcinoma (RCC) using magnetic resonance imaging (MRI) and computed tomography (CT). Methods This study retrospectively collected the MRI and CT data of twelve patients with Xp11.2 translocation RCC confirmed by pathology. Nine cases underwent dynamic contrast-enhanced MRI (DCE-MRI) and 6 cases underwent CT, of which 3 cases underwent MRI and CT simultaneously. The MRI and CT findings were analyzed in regard to tumor position, size, hemorrhagic, cystic or necrotic components, calcification, tumor density, signal intensity and enhancement features. Results The age of the 12 patients ranged from 13 to 46 years (mean age: 23 years). T2WI revealed heterogeneous intensity, hyper-intensity, and slight hypo-intensity in 6 cases, 2 cases, and 1 case, respectively. On DCE-MR images, mild, moderate, and marked rim enhancement of the tumor in the corticomedullary phase (CMP) were observed in 1, 6, and 2 cases, respectively. The tumor parenchyma showed iso-attenuation (n = 4) or slight hyper-attenuation (n = 1) compared to the normal renal cortex on non-contrast CT images. Imaging findings were suggestive of hemorrhage (n = 4) or necrosis (n = 8) in the tumors, and there was evidence of calcification in 8 cases by CT (n = 3) and pathology (n = 8). On dynamic contrast-enhanced CT images, 3 cases and 1 case manifested moderate and strong CMP enhancement, respectively. Nine tumors by MRI and 4 tumors by CT showed prolonged enhancement. Three neoplasms presented at stage I, 2 at stage II, 3 at stage III, and 4 at stage IV according the 2010 AJCC staging criteria. Conclusions XP11.2 translocation RCC should be considered when a child or young adult patient presents with a renal tumor with heterogeneous features such as hemorrhage, necrosis, cystic changes, and calcification on CT and MRI and/or is accompanied by metastatic evidence. PMID:24926688

Xp11.2 translocation renal cell carcinoma with egg-shell calcification mimicking a benign renal tumour: A case report.

PubMed

Liang, Wenjie; Xu, Shunliang

2015-11-01

The present study reports the case of a 20-year-old female who was identified to have a left renal angiomyolipoma (AML) with hemorrhage. Following temporary conservative observation, the patient received continuous ultrasonic follow-up. Due to the rapid growth of the lesion, further examinations were performed. Computed tomography (CT) plain scans revealed a partly high-density mass with marginal egg-shell calcification. Enhanced CT revealed a solid tumor with a rich blood supply. Since no fats were detected, the possibility of a typical AML was excluded, but the diagnoses of epithelioid AML or renal cancer were considered. Finally, the left kidney was partially excised laparoscopically. The intraoperative frozen section indicated a diagnosis of renal cell carcinoma (RCC). The left kidney was subsequently radically excised. Routine histopathological and immunohistochemical tests confirmed that the lesion was an RCC with an Xp11.2 translocation. The present study introduces the pitfalls in the diagnosis of Xp11.2 translocation RCC, which is a rare RCC subtype accompanied with uncommon imaging manifestations. The study suggests that when a rapidly-growing AML is detected by ultrasound, renal cancer with marginal calcification should be considered. Moreover, although egg-shell calcification mostly occurs in benign renal lesions, further examinations, such as enhanced CT, are recommended for identifying the nature of the masses and excluding the possibility of malignant tumors.

Xp11.2 translocation renal cell carcinoma with egg-shell calcification mimicking a benign renal tumour: A case report

PubMed Central

LIANG, WENJIE; XU, SHUNLIANG

2015-01-01

The present study reports the case of a 20-year-old female who was identified to have a left renal angiomyolipoma (AML) with hemorrhage. Following temporary conservative observation, the patient received continuous ultrasonic follow-up. Due to the rapid growth of the lesion, further examinations were performed. Computed tomography (CT) plain scans revealed a partly high-density mass with marginal egg-shell calcification. Enhanced CT revealed a solid tumor with a rich blood supply. Since no fats were detected, the possibility of a typical AML was excluded, but the diagnoses of epithelioid AML or renal cancer were considered. Finally, the left kidney was partially excised laparoscopically. The intraoperative frozen section indicated a diagnosis of renal cell carcinoma (RCC). The left kidney was subsequently radically excised. Routine histopathological and immunohistochemical tests confirmed that the lesion was an RCC with an Xp11.2 translocation. The present study introduces the pitfalls in the diagnosis of Xp11.2 translocation RCC, which is a rare RCC subtype accompanied with uncommon imaging manifestations. The study suggests that when a rapidly-growing AML is detected by ultrasound, renal cancer with marginal calcification should be considered. Moreover, although egg-shell calcification mostly occurs in benign renal lesions, further examinations, such as enhanced CT, are recommended for identifying the nature of the masses and excluding the possibility of malignant tumors. PMID:26722310

Smear layer removal evaluation of different protocol of Bio Race file and XP- endo Finisher file in corporation with EDTA 17% and NaOCl.

PubMed

Zand, Vahid; Mokhtari, Hadi; Reyhani, Mohammad-Frough; Nahavandizadeh, Neda; Azimi, Shahram

2017-11-01

The aim of the present study was to compare the amount of the smear layer remaining in prepared root canals with different protocols of Bio RaCe files and XP-endo Finisher file (XPF) in association with 17% EDTA and sodium hypochlorite solution. A total of 68 extracted single-rooted teeth were randomly divided into 4 experimental groups (n=14) and two control groups (n=6). The root canals were prepared with Bio RaCe files (FKG Dentaire, Switzerland) using the crown-down technique based on manufacturer's instructions and irrigated according to the following irrigation techniques: Group 1: XPF with 2 mL of 2.5% NaOCl for 1 minute. Group 2:, XPF with 1 mL of 17% EDTA for one minute. Group 3: XPF was used for 1 minute in association with normal saline solution. Group 4: XP-endo Finisher file for 30 seconds in association with 2.5% NaOCl and 17% EDTA for 30 seconds. The negative control group: NaOCl (2.5%) was used during root canal preparation, followed by irrigation with 17% EDTA at the end of root canal preparation. The positive control group: Normal saline solution was used for irrigation during root canal preparation. In all the groups, during preparation of the root canals with Bio RaCe file, 20 mL of 2.5% NaOCl was used for root canal irrigation and at the end of the procedural steps 20 mL of normal saline solution was used as a final irrigant. The samples were analyzed under SEM at ×1000‒2000 magnification and evaluated using Torabinejad scoring system. Data were analyzed with non-parametric Kruskal-Wallis test and post hoc Mann-Whitney U test, using SPSS. Statistical significant was defined at P <0.05. The results of the study showed the least amount of the smear layer at coronal, middle and apical thirds of the root canals in groups 2, which was not significantly different from the negative control group ( P <0.5). Under the limitations of the present study, use of a combination of NaOCl and EDTA in association with XPF exhibited the best efficacy for the

A Binaural CI Research Platform for Oticon Medical SP/XP Implants Enabling ITD/ILD and Variable Rate Processing

PubMed Central

Adiloğlu, K.; Herzke, T.

2015-01-01

We present the first portable, binaural, real-time research platform compatible with Oticon Medical SP and XP generation cochlear implants. The platform consists of (a) a pair of behind-the-ear devices, each containing front and rear calibrated microphones, (b) a four-channel USB analog-to-digital converter, (c) real-time PC-based sound processing software called the Master Hearing Aid, and (d) USB-connected hardware and output coils capable of driving two implants simultaneously. The platform is capable of processing signals from the four microphones simultaneously and producing synchronized binaural cochlear implant outputs that drive two (bilaterally implanted) SP or XP implants. Both audio signal preprocessing algorithms (such as binaural beamforming) and novel binaural stimulation strategies (within the implant limitations) can be programmed by researchers. When the whole research platform is combined with Oticon Medical SP implants, interaural electrode timing can be controlled on individual electrodes to within ±1 µs and interaural electrode energy differences can be controlled to within ±2%. Hence, this new platform is particularly well suited to performing experiments related to interaural time differences in combination with interaural level differences in real-time. The platform also supports instantaneously variable stimulation rates and thereby enables investigations such as the effect of changing the stimulation rate on pitch perception. Because the processing can be changed on the fly, researchers can use this platform to study perceptual changes resulting from different processing strategies acutely. PMID:26721923

A Binaural CI Research Platform for Oticon Medical SP/XP Implants Enabling ITD/ILD and Variable Rate Processing.

PubMed

Backus, B; Adiloğlu, K; Herzke, T

2015-12-30

We present the first portable, binaural, real-time research platform compatible with Oticon Medical SP and XP generation cochlear implants. The platform consists of (a) a pair of behind-the-ear devices, each containing front and rear calibrated microphones, (b) a four-channel USB analog-to-digital converter, (c) real-time PC-based sound processing software called the Master Hearing Aid, and (d) USB-connected hardware and output coils capable of driving two implants simultaneously. The platform is capable of processing signals from the four microphones simultaneously and producing synchronized binaural cochlear implant outputs that drive two (bilaterally implanted) SP or XP implants. Both audio signal preprocessing algorithms (such as binaural beamforming) and novel binaural stimulation strategies (within the implant limitations) can be programmed by researchers. When the whole research platform is combined with Oticon Medical SP implants, interaural electrode timing can be controlled on individual electrodes to within ±1 µs and interaural electrode energy differences can be controlled to within ±2%. Hence, this new platform is particularly well suited to performing experiments related to interaural time differences in combination with interaural level differences in real-time. The platform also supports instantaneously variable stimulation rates and thereby enables investigations such as the effect of changing the stimulation rate on pitch perception. Because the processing can be changed on the fly, researchers can use this platform to study perceptual changes resulting from different processing strategies acutely. © The Author(s) 2015.

Wind-Tunnel Tests of the 1/9-Scale Model of the Curtiss XP-62 Airplane with Various Vertical Tail Arrangements

NASA Technical Reports Server (NTRS)

Wallace, Arthur R.; Recant, I.G.

1943-01-01

The effect of various vertical tail arrangements upon the stability and control characteristics of an XP-62 fighter model was investigated. Rudder-free yaw characteristics with take-off power and flaps deflected were satisfactory after dorsal fin modifications. Directional stability was obtained with all modified vertical tails. Satisfactory rudder effectiveness resulted partly because the dual-rotation propellers produced no asymmetric yawing moments. Pedal forces in sideslips were undesirably large but may be easily reduced.

Effect of PH 3 poisoning on a Ni-YSZ anode-supported solid oxide fuel cell under various operating conditions

NASA Astrophysics Data System (ADS)

Xu, Chunchuan; Zondlo, John W.; Gong, Mingyang; Liu, XingBo

The Ni-YSZ anode-supported solid oxide fuel cell (SOFC) can generate electrical power by using coal-derived syngas as the fuel. However, trace contamination of phosphine (PH 3) in the syngas can cause irreversible degradation in cell performance. A series of tests at 10 ppm PH 3 in the fuel gas was carried out under a variety of operating conditions, viz, with/without electrochemical reaction in syngas and with/without H 2O in H 2 fuel at 750 °C, 800 °C and 850 °C. The poisoning effects were evaluated by both electrochemical methods and chemical analyses. The post-mortem analyses of the SOFC anode were performed by means of XRD, SEM/EDS, and XPS. The results show that the degradation rate is larger at the higher cell working temperature using syngas with PH 3 in a 200 h test though PH 3 is more reactive with Ni in the anode at lower working temperature and produces a secondary nickel phosphide (Ni xP y) phase. The dominant compositions of Ni xP y on the cell anode are Ni 5P 2 with the presence of H 2O, and Ni 12P 5 without the presence of H 2O. The production of Ni xP y can be generated on the cell anode using syngas or dry H 2 fuel with 10 ppm PH 3 contaminant. Further, the appearance of Ni xP y phases is independent of the electrochemical reactions in the cell.

Operator Performance Support System (OPSS)

NASA Technical Reports Server (NTRS)

Conklin, Marlen Z.

1993-01-01

In the complex and fast reaction world of military operations, present technologies, combined with tactical situations, have flooded the operator with assorted information that he is expected to process instantly. As technologies progress, this flow of data and information have both guided and overwhelmed the operator. However, the technologies that have confounded many operators today can be used to assist him -- thus the Operator Performance Support Team. In this paper we propose an operator support station that incorporates the elements of Video and Image Databases, productivity Software, Interactive Computer Based Training, Hypertext/Hypermedia Databases, Expert Programs, and Human Factors Engineering. The Operator Performance Support System will provide the operator with an integrating on-line information/knowledge system that will guide expert or novice to correct systems operations. Although the OPSS is being developed for the Navy, the performance of the workforce in today's competitive industry is of major concern. The concepts presented in this paper which address ASW systems software design issues are also directly applicable to industry. the OPSS will propose practical applications in how to more closely align the relationships between technical knowledge and equipment operator performance.

Operating System Abstraction Layer (OSAL)

NASA Technical Reports Server (NTRS)

Yanchik, Nicholas J.

2007-01-01

This viewgraph presentation reviews the concept of the Operating System Abstraction Layer (OSAL) and its benefits. The OSAL is A small layer of software that allows programs to run on many different operating systems and hardware platforms It runs independent of the underlying OS & hardware and it is self-contained. The benefits of OSAL are that it removes dependencies from any one operating system, promotes portable, reusable flight software. It allows for Core Flight software (FSW) to be built for multiple processors and operating systems. The presentation discusses the functionality, the various OSAL releases, and describes the specifications.

Preliminary Evaluation of the Low-Speed Stability and Control Characteristics of the McDonnell XP-85 Airplane from Tests of an Unballasted 1/5-Scale Model in the Langley Free-Flight Tunnel

NASA Technical Reports Server (NTRS)

Paulson, John W.; Johnson, Joseph L.

1947-01-01

At the request of the Air Material Command, Army Air Forces an investigation of the low-speed, power-off stability and control characteristics of the McDonnell XP-85 airplane is being conducted in the Langley free-flight tunnel. The XP-85 airplane is a jet propelled, parasite fighter with a 34 deg sweepback at the wing quarter chord. It was designed to be carried in a bomb bay of the B-36 air plane. The first portion of the investigation consists of a preliminary evaluation of the stability and control characteristics of the airplane from force and fight tests of an unballasted 1/5-scale model. The second portion of the investigation consists of test of a properly balasted 1/10-scale model which will include a study of the stability of the Xp-85 when attached to the trapeze for retraction into the B-36 bomb bay. The results of the preliminary test with the 1/5-scale model are presented herein. This portion fo the investigation included tests of the model with various center fin arrangements. Both the design nose flap and a stall control vane were investigated.

Systems Operations Studies for Automated Guideway Transit Systems : Quantitative Analysis of Alternative AGT Operational Control Strategies

DOT National Transportation Integrated Search

1981-10-01

The objectives of the Systems Operation Studies (SOS) for automated guideway transit (AGT) systems are to develop models for the analysis of system operations, to evaluate performance and cost, and to establish guidelines for the design and operation...

9 CFR 205.201 - System operator.

Code of Federal Regulations, 2014 CFR

2014-01-01

... 9 Animals and Animal Products 2 2014-01-01 2014-01-01 false System operator. 205.201 Section 205... Interpretive Opinions § 205.201 System operator. The system operator can be the Secretary of State of a State... system refers to operation by the Secretary of State of a State, but the definition in (c)(11) of...

9 CFR 205.201 - System operator.

Code of Federal Regulations, 2011 CFR

2011-01-01

... 9 Animals and Animal Products 2 2011-01-01 2011-01-01 false System operator. 205.201 Section 205... Interpretive Opinions § 205.201 System operator. The system operator can be the Secretary of State of a State... system refers to operation by the Secretary of State of a State, but the definition in (c)(11) of...

9 CFR 205.201 - System operator.

Code of Federal Regulations, 2012 CFR

2012-01-01

... 9 Animals and Animal Products 2 2012-01-01 2012-01-01 false System operator. 205.201 Section 205... Interpretive Opinions § 205.201 System operator. The system operator can be the Secretary of State of a State... system refers to operation by the Secretary of State of a State, but the definition in (c)(11) of...

9 CFR 205.201 - System operator.

Code of Federal Regulations, 2010 CFR

2010-01-01

... 9 Animals and Animal Products 2 2010-01-01 2010-01-01 false System operator. 205.201 Section 205... Interpretive Opinions § 205.201 System operator. The system operator can be the Secretary of State of a State... system refers to operation by the Secretary of State of a State, but the definition in (c)(11) of...

Measurement of SIFT operating system overhead

NASA Technical Reports Server (NTRS)

Palumbo, D. L.; Butler, R. W.

1985-01-01

The overhead of the software implemented fault tolerance (SIFT) operating system was measured. Several versions of the operating system evolved. Each version represents different strategies employed to improve the measured performance. Three of these versions are analyzed. The internal data structures of the operating systems are discussed. The overhead of the SIFT operating system was found to be of two types: vote overhead and executive task overhead. Both types of overhead were found to be significant in all versions of the system. Improvements substantially reduced this overhead; even with these improvements, the operating system consumed well over 50% of the available processing time.

Ultrashort-Pulse Laser System: Theory of Operation and Operating Procedures

DTIC Science & Technology

1992-07-01

Nov 89 - Jul 92 4. TITLE AND SUBTITLE 5. FUNDING NUMBERS Ultrashort-Pulse Laser System : Theory of Operation and C - F33615-88-C-0631 Operating...i ’IR~A&, D2;" T.&B [E] al uicod [] j 0 Avhi lp.bilty C: oded’ Avail i Qiv ULTRASHORT-PULSE LASER SYSTEM : THEORY OF OPERATION AND OPERATING PROCEDURES

Autonomous Operations System: Development and Application

NASA Technical Reports Server (NTRS)

Toro Medina, Jaime A.; Wilkins, Kim N.; Walker, Mark; Stahl, Gerald M.

2016-01-01

Autonomous control systems provides the ability of self-governance beyond the conventional control system. As the complexity of mechanical and electrical systems increases, there develops a natural drive for developing robust control systems to manage complicated operations. By closing the bridge between conventional automated systems to knowledge based self-awareness systems, nominal control of operations can evolve into relying on safe critical mitigation processes to support any off-nominal behavior. Current research and development efforts lead by the Autonomous Propellant Loading (APL) group at NASA Kennedy Space Center aims to improve cryogenic propellant transfer operations by developing an automated control and health monitoring system. As an integrated systems, the center aims to produce an Autonomous Operations System (AOS) capable of integrating health management operations with automated control to produce a fully autonomous system.

Method and Apparatus Providing Deception and/or Altered Operation in an Information System Operating System

DOEpatents

Cohen, Fred; Rogers, Deanna T.; Neagoe, Vicentiu

2008-10-14

A method and/or system and/or apparatus providing deception and/or execution alteration in an information system. In specific embodiments, deceptions and/or protections are provided by intercepting and/or modifying operation of one or more system calls of an operating system.

Operationally Efficient Propulsion System Study (OEPSS) data book. Volume 3: Operations technology

NASA Technical Reports Server (NTRS)

Vilja, John O.

1990-01-01

The study was initiated to identify operational problems and cost drivers for current propulsion systems and to identify technology and design approaches to increase the operational efficiency and reduce operations costs for future propulsion systems. To provide readily usable data for the Advanced Launch System (ALS) program, the results of the OEPSS study were organized into a series of OEPSS Data Books. This volume describes operations technologies that will enhance operational efficiency of propulsion systems. A total of 15 operations technologies were identified that will eliminate or mitigate operations problems described in Volume 2. A recommended development plan is presented for eight promising technologies that will simplify the propulsion system and reduce operational requirements.

Quantum criticality and development of antiferromagnetic order in the quasikagome Kondo lattice CeR h1 -xP dxSn

NASA Astrophysics Data System (ADS)

Yang, C. L.; Tsuda, S.; Umeo, K.; Yamane, Y.; Onimaru, T.; Takabatake, T.; Kikugawa, N.; Terashima, T.; Uji, S.

2017-07-01

CeRhSn with a quasikagome lattice of Ce atoms in the hexagonal c plane has been expected to be in close vicinity to a zero-field quantum criticality derived from magnetic frustration. We have studied how the ground state changes with substitution of Pd for Rh in CeR h1 -xP dxSn (x ≤0.75 ) by measuring the specific heat C , magnetic susceptibilities χdc and χac, magnetization M , electrical resistivity ρ, and magnetoresistance. For x =0 , the field dependence of χac at T =0.03 K shows a peak at B ∥a =3.5 T , confirming the spin-flop crossover in the field applied along the hard axis. The temperature dependence of χac shows a broad maximum at 0.1 K whereas C /T continues to increase down to 0.08 K. For x ≧0.1 ,ρ (T ) is dominated by incoherent Kondo scattering and both C /T and χac(T ) exhibit peaks, indicating the development of an antiferromagnetic order. The ordering temperature rises to 2.5 K as x is increased to 0.75. Our results indicate that the ground state in the quasikagome Kondo lattice CeR h1 -xP dxSn leaves the quantum critical point at x =0 with increasing x as a consequence of suppression of both the magnetic frustration and Kondo effect.

Gene of a new X-linked syndrome with multiple congenital anomalies and severe mental retardation maps in Xp22-pter

DOE Office of Scientific and Technical Information (OSTI.GOV)

Wittwer, B.; Kircheisen, R.; Leutelt, J.

1994-09-01

We report on a family with 3 males presenting with a not yet described new X-chromosomal syndrome of multiple congenital anomalies and severe mental retardation. Two sisters have (with 3 different partners) 3 severely handicapped sons. In each case, oligohydramnios and intrauterine growth retardation were observed. Delivery was in the 34th, 31st, and 38th gestational week, respectively. Two of the patients had microcephaly (head circumference of the third case at birth is unknown). On physical examination, high and broad forehead, frontal bossing, downslanting palpebral fissures, long philtrum, thin upper lip, high arched palate, and deeply set anteverted ears were seen.more » One of the boys has microphthalmos and sclerocornea, while his cousin shows atrophy of the optic nerve. All three patients show a severe statomotor and mental retardation, they are most likely deaf and blind, have pathologic EEG, and seizures. Important additional findings are hydronephrosis, renal duplication, vesicorenal reflux, and agenesis of corpus callosum. The karyotype is normal (46,XY). We performed a segregation analysis in the family using more than 20 DNA polymorphisms distributed over the X chromosome. Linkage without recombination was found to KAL, DXS278, and DXS16 in Xp22. Analysis of multiple informative meioses suggested a location of the disease locus distal to DXS207. Recombinants were identified with all other marker loci from Xp22-Xpter.« less

Interspecies synteny mapping identifies a quantitative trait locus for bone mineral density on human chromosome Xp22.

PubMed

Parsons, Claire A; Mroczkowski, H Joel; McGuigan, Fiona E A; Albagha, Omar M E; Manolagas, Stavros; Reid, David M; Ralston, Stuart H; Shmookler Reis, Robert J

2005-11-01

Bone mineral density (BMD) is a complex trait with a strong genetic component and an important predictor of osteoporotic fracture risk. Here we report the use of a cross-species strategy to identify genes that regulate BMD, proceeding from quantitative trait mapping in mice to association mapping of the syntenic region in the human genome. We identified a quantitative trait locus (QTL) on the mouse X-chromosome for post-maturity change in spine BMD in a cross of SAMP6 and AKR/J mice and conducted association mapping of the syntenic region on human chromosome Xp22. We studied 76 single nucleotide polymorphisms (SNP) from the human region in two sets of DNA pools prepared from individuals with lumbar spine-BMD (LS-BMD) values falling into the top and bottom 13th percentiles of a population-based study of 3100 post-menopausal women. This procedure identified a region of significant association for two adjacent SNP (rs234494 and rs234495) within the Xp22 locus (P<0.001). Individual genotyping for rs234494 in the BMD pools confirmed the presence of an association for alleles (P=0.018) and genotypes (P=0.008). Analysis of rs234494 and rs234495 in 1053 women derived from the same population who were not selected for BMD values showed an association with LS-BMD for rs234495 (P=0.01) and for haplotypes defined by both SNP (P=0.002). Our study illustrates that interspecies synteny can be used to identify and refine QTL for complex traits and represents the first example where a human QTL for BMD regulation has been mapped using this approach.

Nuclease digestion and mass spectrometric characterization of oligodeoxyribonucleotides containing 1,2-GpG, 1,2-ApG, and 1,3-GpXpG cisplatin intrastrand cross-links.

PubMed

Williams, Renee T; Nalbandian, Jenifer N; Tu, Audrey; Wang, Yinsheng

2013-05-01

The primary mode of action for cis-diamminedichloroplatinum (II), referred to as cisplatin, toward the treatment of solid malignancies is through formation of cross-links with DNA at purine sites, especially guanines. We prepared oligodeoxyribonucleotides (ODNs) containing a 1,2-GpG, 1,2-ApG, or 1,3-GpXpG cisplatin intrastrand cross-link and the corresponding ODNs modified with (15)N2-labeled cisplatin, and characterized these ODNs with electrospray ionization mass spectrometry (ESI-MS) and tandem MS (MS/MS). We also employed LC-MS/MS to characterize the digestion products of these ODNs after treatment with a cocktail of 4 enzymes (nuclease P1, phosphodiesterases I and II, and alkaline phosphatase). 1,2-GpG was released from the ODNs as a dinucleoside monophosphate or a dinucleotide. Analyses of the digestion products of ODNs containing a 1,2-GpG cross-link on the 5' or 3' terminus revealed that the dinucleotide carries a terminal 5' phosphate. On the other hand, digestion of the 1,3-GpXpG intrastrand cross-link yielded 3 dinucleoside products with 0, 1, or 2 phosphate groups. The availability of the ODNs carrying the stable isotope-labeled lesions, MS/MS analyses of the cisplatin-modified ODNs, and the characterization of the enzymatic digestion products of these ODNs set the stage for the future LC-MS/MS quantification of the 1,2-GpG, 1,2-ApG, and 1,3-GpXpG lesions in cellular DNA. Copyright © 2012 Elsevier B.V. All rights reserved.

Nuclease Digestion and Mass Spectrometric Characterization of Oligodeoxyribonucleotides Containing 1,2-GpG, 1,2-ApG, and 1,3-GpXpG Cisplatin Intrastrand Cross-links

PubMed Central

Williams, Renee T.; Nalbandian, Jenifer; Tu, Audrey; Wang, Yinsheng

2013-01-01

Background The primary mode of action for cis-diamminedichloroplatinum (II), referred to as cisplatin, towards the treatment of solid malignancies is through formation of cross-links with DNA at purine sites, especially guanines. Methods We prepared oligodeoxyribonucleotides (ODNs) containing a 1,2-GpG, 1,2-ApG, or 1,3-GpXpG cisplatin intrastrand cross-link and the corresponding ODNs modified with 15N2-labeled cisplatin, and characterized these ODNs with electrospray ionization mass spectrometry (ESI-MS) and tandem MS (MS/MS). We also employed LC-MS/MS to characterize the digestion products of these ODNs after treatment with a cocktail of 4 enzymes (nuclease P1, phosphodiesterases I and II, and alkaline phosphatase). Results 1,2-GpG was released from the ODNs as a dinucleoside monophosphate or a dinucleotide. Analyses of the digestion products of ODNs containing a 1,2-GpG cross-link on the 5′ or 3′ terminus revealed that the dinucleotide carries a terminal 5′ phosphate. On the other hand, digestion of the 1,3-GpXpG intrastrand cross-link yielded 3 dinucleoside products with 0, 1, or 2 phosphate groups. Results The availability of the ODNs carrying the stable isotope-labeled lesions, MS/MS analyses of the cisplatin-modified ODNs, and the characterization of the enzymatic digestion products of these ODNs set the stage for the future LC-MS/MS quantification of the 1,2-GpG, 1,2-ApG, and 1,3-GpXpG lesions in cellular DNA. PMID:23266768

Parametric Optimization of Some Critical Operating System Functions--An Alternative Approach to the Study of Operating Systems Design

ERIC Educational Resources Information Center

Sobh, Tarek M.; Tibrewal, Abhilasha

2006-01-01

Operating systems theory primarily concentrates on the optimal use of computing resources. This paper presents an alternative approach to teaching and studying operating systems design and concepts by way of parametrically optimizing critical operating system functions. Detailed examples of two critical operating systems functions using the…

EOS Operations Systems: EDOS Implemented Changes to Reduce Operations Costs

NASA Technical Reports Server (NTRS)

Cordier, Guy R.; Gomez-Rosa, Carlos; McLemore, Bruce D.

2007-01-01

The authors describe in this paper the progress achieved to-date with the reengineering of the Earth Observing System (EOS) Data and Operations System (EDOS), the experience gained in the process and the ensuing reduction of ground systems operations costs. The reengineering effort included a major methodology change, applying to an existing schedule driven system, a data-driven system approach.

Program Merges SAR Data on Terrain and Vegetation Heights

NASA Technical Reports Server (NTRS)

Siqueira, Paul; Hensley, Scott; Rodriguez, Ernesto; Simard, Marc

2007-01-01

X/P Merge is a computer program that estimates ground-surface elevations and vegetation heights from multiple sets of data acquired by the GeoSAR instrument [a terrain-mapping synthetic-aperture radar (SAR) system that operates in the X and bands]. X/P Merge software combines data from X- and P-band digital elevation models, SAR backscatter magnitudes, and interferometric correlation magnitudes into a simplified set of output topographical maps of ground-surface elevation and tree height.

Renal cell carcinoma in children and young adults: analysis of clinicopathological, immunohistochemical and molecular characteristics with an emphasis on the spectrum of Xp11.2 translocation-associated and unusual clear cell subtypes.

PubMed

Wu, A; Kunju, L P; Cheng, L; Shah, R B

2008-11-01

Recent studies suggest that paediatric renal cell carcinoma (RCC) may represent a distinct group of tumours; however, its biological behaviour and classification remain poorly understood. The aim was to analyse 13 RCCs from patients < or =23 years of age to determine their clinicopathological, immunohistochemical and molecular characteristics. The histological spectrum included: Xp11.2 translocation-associated (6/13 patients, 46%), clear cell (5/13 patients, 38%), papillary (1/13 patients) and unclassified (1/13 patients) types. The Xp11.2 translocation-associated RCCs had a wide morphological spectrum, with high nuclear grade cells with abundant cytoplasm ranging from clear to granular and architecture ranging from solid to papillary. These tumours lacked cytokeratin expression and were confirmed by nuclear reactivity for TFE3 protein. Most of these translocation-associated tumours presented at high stage and had an unfavourable outcome. Three clear cell RCCs had unusual features that have not been previously characterized, including solid and cystic architecture, cells with abundant eosinophilic cytoplasm yet low nuclear grade and focal cytoplasmic inclusions, resembling oncocytoma. Deletion of subtelomeric 3p25 was observed in two of these RCCs. Xp11.2 translocation-associated RCC represents a predominant and aggressive subtype in the paediatric age group. Increased awareness of this subtype is important due to its heterogeneous morphology.

Epileptic encephalopathy in a girl with an interstitial deletion of Xp22 comprising promoter and exon 1 of the CDKL5 gene.

PubMed

Bahi-Buisson, Nadia; Girard, Benoit; Gautier, Agnes; Nectoux, Juliette; Fichou, Yann; Saillour, Yoann; Poirier, Karine; Chelly, Jamel; Bienvenu, Thierry

2010-01-05

We report a 2-year-old girl with early onset seizures variant of Rett syndrome with a deletion at Xp22 detected by multiplex ligation-dependent probe amplification (MLPA) technique. This patient presented with tonic seizures at 7 days of life. Subsequently, she developed infantile spasms at three months and finally refractory myoclonic epilepsy. She demonstrated severe encephalopathy with hypotonia, deceleration of head growth, with eye gaze but limited eye pursuit, no language, limited hand use, and intermittent hand stereotypies. This combination of clinical features, suggestive of early onset variant of Rett syndrome led us to screen the CDKL5 gene. In a first step, screening of the whole coding sequence of the CDKL5 gene revealed no point mutations. In a second step, we searched gross rearrangements by MLPA and identified a microdeletion affecting both the promoter and exon 1 in CDKL5. Subsequent analysis on a Nimblegen HD2 microarray confirmed a deletion of approximately 300 kb at Xp22, including the BEND2, SCML2, and CDKL5 genes. In conclusion, our report suggests that searching for large rearrangements in CDKL5 should be considered in girls with early onset seizures and Rett-like features. (c) 2009 Wiley-Liss, Inc.

The protease ClpXP and the PAS-domain protein DivL regulate CtrA and gene transfer agent production in Rhodobacter capsulatus.

PubMed

Westbye, A B; Kater, L; Wiesmann, C; Ding, H; Yip, C K; Beatty, J T

2018-04-06

Several members of the Rhodobacterales ( Alphaproteobacteria ) produce a conserved horizontal gene transfer vector, called gene transfer agent (GTA), that appears to have evolved from a bacteriophage. The model system to study GTA biology is the R. capsulatus GTA (RcGTA), a small, tailed bacteriophage-like particle produced by a subset of the cells in a culture. The response regulator CtrA is conserved in the Alphaproteobacteria and is an essential regulator of RcGTA production: it controls production and maturation of the RcGTA particle and RcGTA release from cells. CtrA also controls the natural transformation-like system required for cells to receive RcGTA-donated DNA. Here, we report that a dysregulation of the CckA-ChpT-CtrA phosphorelay - either by loss of the PAS-domain protein DivL or by substituting the autophosphorylation residue of the hybrid histidine kinase CckA - decreased CtrA phosphorylation and greatly increased RcGTA protein production in R. capsulatus We show that a loss of the ClpXP protease or the three C-terminal residues of CtrA resulted in increased CtrA levels in R. capsulatus, and identify ClpX(P) as essential for maturation of RcGTA particles. Furthermore, we show that CtrA phosphorylation is important for head spike production. Our results provide novel insight into the regulation of CtrA and GTAs in the Rhodobacterales Importance Members of the Rhodobacterales are abundant in ocean and fresh water environments. The conserved GTA produced by many Rhodobacterales may have an important role in horizontal gene transfer (HGT) in aquatic environments and provide a significant contribution to their adaptation. GTA production is controlled by bacterial regulatory systems, including the conserved CckA-ChpT-CtrA phosphorelay, however several questions about GTA regulation remains. Our identification that a 'short' DivL homologue and ClpXP regulate CtrA in R. capsulatus extends the model of CtrA regulation from Caulobacter crescentus to a member

UNIX-based operating systems robustness evaluation

NASA Technical Reports Server (NTRS)

Chang, Yu-Ming

1996-01-01

Robust operating systems are required for reliable computing. Techniques for robustness evaluation of operating systems not only enhance the understanding of the reliability of computer systems, but also provide valuable feed- back to system designers. This thesis presents results from robustness evaluation experiments on five UNIX-based operating systems, which include Digital Equipment's OSF/l, Hewlett Packard's HP-UX, Sun Microsystems' Solaris and SunOS, and Silicon Graphics' IRIX. Three sets of experiments were performed. The methodology for evaluation tested (1) the exception handling mechanism, (2) system resource management, and (3) system capacity under high workload stress. An exception generator was used to evaluate the exception handling mechanism of the operating systems. Results included exit status of the exception generator and the system state. Resource management techniques used by individual operating systems were tested using programs designed to usurp system resources such as physical memory and process slots. Finally, the workload stress testing evaluated the effect of the workload on system performance by running a synthetic workload and recording the response time of local and remote user requests. Moderate to severe performance degradations were observed on the systems under stress.

Control of hay-scented and New York ferns with Oust Xp® herbicide: Revisiting rate and timing required in mixed oak and northern hardwood stands

Treesearch

Todd E. Ristau

2017-01-01

Dense rhizomatous fern layers compete with desirable tree seedlings for light, which suppresses development and even kills seedlings. Sulfometuron methyl (Oust XP®) herbicide can be safely and effectively used to control ferns. Previous research showed that depending on application timing, as little as 2 ounces of Oust XP per acre controlled ferns while hardwood tree...

Space station operating system study

NASA Technical Reports Server (NTRS)

Horn, Albert E.; Harwell, Morris C.

1988-01-01

The current phase of the Space Station Operating System study is based on the analysis, evaluation, and comparison of the operating systems implemented on the computer systems and workstations in the software development laboratory. Primary emphasis has been placed on the DEC MicroVMS operating system as implemented on the MicroVax II computer, with comparative analysis of the SUN UNIX system on the SUN 3/260 workstation computer, and to a limited extent, the IBM PC/AT microcomputer running PC-DOS. Some benchmark development and testing was also done for the Motorola MC68010 (VM03 system) before the system was taken from the laboratory. These systems were studied with the objective of determining their capability to support Space Station software development requirements, specifically for multi-tasking and real-time applications. The methodology utilized consisted of development, execution, and analysis of benchmark programs and test software, and the experimentation and analysis of specific features of the system or compilers in the study.

Analysis of remote operating systems for space-based servicing operations, volume 1

NASA Technical Reports Server (NTRS)

1985-01-01

A two phase study was conducted to analyze and develop the requirements for remote operating systems as applied to space based operations for the servicing, maintenance, and repair of satellites. Phase one consisted of the development of servicing requirements to establish design criteria for remote operating systems. Phase two defined preferred system concepts and development plans which met the requirements established in phase one. The specific tasks in phase two were to: (1) identify desirable operational and conceptual approaches for selected mission scenarios; (2) examine the potential impact of remote operating systems incorporated into the design of the space station; (3) address remote operating systems design issues, such as mobility, which are effected by the space station configuration; and (4) define the programmatic approaches for technology development, testing, simulation, and flight demonstration.

Xp22.33p22.12 Duplication in a Patient with Intellectual Disability and Dysmorphic Facial Features

PubMed Central

Lintas, Carla; Picinelli, Chiara; Piras, Ignazio S.; Sacco, Roberto; Gabriele, Stefano; Verdecchia, Magda; Persico, Antonio M.

2016-01-01

A novel 19.98-Mb duplication in chromosome Xp22.33p22.12 was detected by array CGH in a 30-year-old man affected by intellectual disability, congenital hypotonia and dysmorphic features. The duplication encompasses more than 100 known genes. Many of these genes (such as neuroligin 4, cyclin-dependent kinase like 5, and others) have already correlated with X-linked intellectual disability and/or neurodevelopmental disorders. Due to the high number of potentially pathogenic genes involved in the reported duplication, we cannot correlate the clinical phenotype to a single gene. Indeed, we suggest that the resulting clinical phenotype may have arisen from the overexpression and consequent perturbation of fine gene dosage. PMID:26997944

Xp22.33p22.12 Duplication in a Patient with Intellectual Disability and Dysmorphic Facial Features.

PubMed

Lintas, Carla; Picinelli, Chiara; Piras, Ignazio S; Sacco, Roberto; Gabriele, Stefano; Verdecchia, Magda; Persico, Antonio M

2016-02-01

A novel 19.98-Mb duplication in chromosome Xp22.33p22.12 was detected by array CGH in a 30-year-old man affected by intellectual disability, congenital hypotonia and dysmorphic features. The duplication encompasses more than 100 known genes. Many of these genes (such as neuroligin 4, cyclin-dependent kinase like 5, and others) have already correlated with X-linked intellectual disability and/or neurodevelopmental disorders. Due to the high number of potentially pathogenic genes involved in the reported duplication, we cannot correlate the clinical phenotype to a single gene. Indeed, we suggest that the resulting clinical phenotype may have arisen from the overexpression and consequent perturbation of fine gene dosage.

Introduction on the operational storm surge forecasting system in Korea Operational Oceanographic System (KOOS)

NASA Astrophysics Data System (ADS)

Kwon, Jae-Il; Park, Kwang-Soon; Choi, Jung-Woon; Lee, Jong-Chan; Heo, Ki-Young; Kim, Sang-Ik

2017-04-01

During last more than 50 years, 258 typhoons passed and affected the Korean peninsula in terms of high winds, storm surges and extreme waves. In this study we explored the performance of the operational storm surge forecasting system in the Korea Operational Oceanographic System (KOOS) with 8 typhoons from 2010 to 2016. The operation storm surge forecasting system for the typhoon in KOOS is based on 2D depth averaged model with tides and CE (U.S. Army Corps of Engineers) wind model. Two key parameters of CE wind model, the locations of typhoon center and its central atmospheric pressure are based from Korea Meteorological administrative (KMA)'s typhoon information provided from 1 day to 3 hour intervals with the approach of typhoon through the KMA's web-site. For 8 typhoons cases, the overall errors, other performances and analysis such as peak time and surge duration are presented in each case. The most important factor in the storm surge errors in the operational forecasting system is the accuracy of typhoon passage prediction.

Disk Operating System User's Guide

DOT National Transportation Integrated Search

1972-05-01

This document serves the purpose of bringing together in one place most of the information a user needs to use the DDP-516 Disk Operating System, (DOS). DOS is a core resident, one user, console-oriented operating system which allows the user to cont...

Extended System Operations Studies for Automated Guideway Transit Systems

DOT National Transportation Integrated Search

1982-02-01

The objectives of the System Operations Studies (SOS) of the Automated Guideway Transit Technology (AGTT) program was to develop models for the analysis of system operations, to evaluate AGT system performance and cost, and to establish guidelines fo...

Adult-onset renal cell carcinoma associated with Xp11.2 translocation/TFE3 gene fusion: 3 case reports and review of literature.

PubMed

Gong, Pengfeng; Zhuang, Qianfeng; Wang, Kun; Xu, Renfang; Chen, Yiming; Wang, Xiaogang; Yin, Shuai

2018-06-01

Renal cell carcinoma associated with Xp11.2 translocations/TFE3 gene fusions is a rare subtype of renal cell carcinoma. This predominantly occurs in juveniles, but rarely seen in adults with lymph node or organic metastasis and a worsened prognosis. Herein, we presented 3 adult cases of Xp11-RCC. Two patients were in early stage and good condition, and the third patient had lymph node metastasis but showed no recurrence after a 3-month follow-up. Case 1: A 50-year-old female without any lumbago and gross hematuria was incidentally detected by left renal mass by ultrasonography. Case 2: A 31-year-old female with 2-year hemodialysis was detected with right renal carcinoma during preoperative examination of renal transplant. Case 3: A 45-year-old male with right lumbago for 1 month was detected with a mass in the lower pole of right kidney by ultrasonography. The characteristics of these 3 images are not consistent with each other, and showed some differences with the previous ones. All these 3 patients underwent laparoscopic radical nephrectomy, and case 1 patient underwent renal hilar lymphnode dissection at the same time. Immunohistochemistry was performed on all the 3 tumors, revealing that the tumor cells were positive for TFE3 and Melan-A. Case 1 showed lymph node metastasis, and received mTOR inhibitors. The 3 patients had no recurrent and new metastasis in other organs after follow-up for 3 months, 2 months, and 11 months, respectively. Whether the adult-onset Xp-RCC has an aggressive clinical course still remains controversial. Characteristics of the images of the 3 adult cases showed some uniformity but still have some differences. Immunohistochemistry results revealed tumor cell positive for TFE3, but have no consistency in carbonic anhydrase IX, CD117, Ki67, CK8/18 AE1/AE3 and so on. Therefore, the uniform and definitive diagnostic standards of the tumors are uncertain. Hence, more cases and findings are required to elaborate the standards of all the

Imprint cytologic features in renal cell carcinoma associated with Xp11.2 translocation/TFE3 gene fusion in an adult: a case report.

PubMed

Yamaguchi, Tadanori; Kuroda, Naoto; Imamura, Yoshiaki; Hes, Ondrej; Kawada, Takako; Nakayama, Keizou

2009-01-01

Adult-onset renal cell carcinoma (RCC) associated with Xp11.2 translocation/TFE3 gene fusion is a very rare tumor. To date, there are no reports on immunocytochemical study of the primary tumor. We describe such a case that we diagnosed by immunocytochemistry of imprint cytology material. A 46-year-old man was found to have a mass in the lower pole of the right kidney. Magnetic resonance imaging (MRI) T2-weighted images showed a hypointense area in the tumor, and papillary RCC was suspected. Imprint cytology showed tumor cells that were isolated or arranged in large or small papillary clusters. Irregularly shaped large oval nuclei, finely granular chromatin and a single large nucleolus were noted. Cytoplasm was abundant and admixed with clear and granular eosinophilic patterns and scattered large vacuolated cells. Almost all tumor cells diffusely expressed immunocytochemical reactivity to TFE3 protein. Hyaline nodules were observed in the stroma. Ultrastructurally, neoplastic cells contained rhomboid crystals identical to those of alveolar soft part sarcoma. The immunocytochemistry of TFE3 protein may be a powerful tool for accurate diagnosis when RCC associated with Xp11.2 translocation/TFE3 gene fusion is suspected by imprint cytology even in adult-onset cases, and cytotechnologists should accurately recognize cytologic findings of this tumor.

Operational Management System for Regulated Water Systems

NASA Astrophysics Data System (ADS)

van Loenen, A.; van Dijk, M.; van Verseveld, W.; Berger, H.

2012-04-01

Most of the Dutch large rivers, canals and lakes are controlled by the Dutch water authorities. The main reasons concern safety, navigation and fresh water supply. Historically the separate water bodies have been controlled locally. For optimizating management of these water systems an integrated approach was required. Presented is a platform which integrates data from all control objects for monitoring and control purposes. The Operational Management System for Regulated Water Systems (IWP) is an implementation of Delft-FEWS which supports operational control of water systems and actively gives advice. One of the main characteristics of IWP is that is real-time collects, transforms and presents different types of data, which all add to the operational water management. Next to that, hydrodynamic models and intelligent decision support tools are added to support the water managers during their daily control activities. An important advantage of IWP is that it uses the Delft-FEWS framework, therefore processes like central data collection, transformations, data processing and presentation are simply configured. At all control locations the same information is readily available. The operational water management itself gains from this information, but it can also contribute to cost efficiency (no unnecessary pumping), better use of available storage and advise during (water polution) calamities.

Mars outpost - System and operations challenges

NASA Technical Reports Server (NTRS)

Roberts, Barney; Guerra, Lisa

1990-01-01

The paper addresses the challenges inherent in establishing an outpost on the planet Mars. For background purposes, the unique, remote Martian environment and the developmental phases of a settlement in such an environment are discussed. Challenges are identified in terms of surface systems and operations. Due to its importance to habitability, the life support system (LSS) is highlighted with various options identified. Operations for the Mars outpost, earth-based and local, are characterized by a decentralized concept. The challenge of integrating logistics analysis early in system design and operations strategy is also addressed. In order to understand and reduce the system and operations challenges, the application of terrestrial and lunar testbeds is explained.

Psychological Attributes Critical to the Performance of MQ-1 Predator and MQ-9 Reaper U.S. Air Force Sensor Operators

DTIC Science & Technology

2010-06-25

Deputy, 711 HPW/XP), Roscoe "Yogi" VanCamp,UCa!. USAF, and Mark Marley, M.A., (project manager ) who were critical to the logistical operations of...national security, and international relations. Understanding basic principles of aviation, crew resource management , communicat ion protocols, geo...ain::raft flight controls, instruments, weight, balance), as well as topics in aircrew coordination (e.g., crew resource management and situational

47 CFR 32.2220 - Operator systems.

Code of Federal Regulations, 2014 CFR

2014-10-01

... 47 Telecommunication 2 2014-10-01 2014-10-01 false Operator systems. 32.2220 Section 32.2220 Telecommunication FEDERAL COMMUNICATIONS COMMISSION (CONTINUED) COMMON CARRIER SERVICES UNIFORM SYSTEM OF ACCOUNTS FOR TELECOMMUNICATIONS COMPANIES Instructions for Balance Sheet Accounts § 32.2220 Operator systems...

47 CFR 32.2220 - Operator systems.

Code of Federal Regulations, 2013 CFR

2013-10-01

... 47 Telecommunication 2 2013-10-01 2013-10-01 false Operator systems. 32.2220 Section 32.2220 Telecommunication FEDERAL COMMUNICATIONS COMMISSION (CONTINUED) COMMON CARRIER SERVICES UNIFORM SYSTEM OF ACCOUNTS FOR TELECOMMUNICATIONS COMPANIES Instructions for Balance Sheet Accounts § 32.2220 Operator systems...

47 CFR 32.2220 - Operator systems.

Code of Federal Regulations, 2012 CFR

2012-10-01

... 47 Telecommunication 2 2012-10-01 2012-10-01 false Operator systems. 32.2220 Section 32.2220 Telecommunication FEDERAL COMMUNICATIONS COMMISSION (CONTINUED) COMMON CARRIER SERVICES UNIFORM SYSTEM OF ACCOUNTS FOR TELECOMMUNICATIONS COMPANIES Instructions for Balance Sheet Accounts § 32.2220 Operator systems...

47 CFR 32.2220 - Operator systems.

Code of Federal Regulations, 2011 CFR

2011-10-01

... 47 Telecommunication 2 2011-10-01 2011-10-01 false Operator systems. 32.2220 Section 32.2220 Telecommunication FEDERAL COMMUNICATIONS COMMISSION (CONTINUED) COMMON CARRIER SERVICES UNIFORM SYSTEM OF ACCOUNTS FOR TELECOMMUNICATIONS COMPANIES Instructions for Balance Sheet Accounts § 32.2220 Operator systems...

47 CFR 32.2220 - Operator systems.

Code of Federal Regulations, 2010 CFR

2010-10-01

... 47 Telecommunication 2 2010-10-01 2010-10-01 false Operator systems. 32.2220 Section 32.2220 Telecommunication FEDERAL COMMUNICATIONS COMMISSION (CONTINUED) COMMON CARRIER SERVICES UNIFORM SYSTEM OF ACCOUNTS FOR TELECOMMUNICATIONS COMPANIES Instructions for Balance Sheet Accounts § 32.2220 Operator systems...

Model Analyst’s Toolkit User Guide, Version 7.1.0

DTIC Science & Technology

2015-08-01

Help > About)  Environment details ( operating system )  metronome.log file, located in your MAT 7.1.0 installation folder  Any log file that...requirements to run the Model Analyst’s Toolkit:  Windows XP operating system (or higher) with Service Pack 2 and all critical Windows updates installed...application icon on your desktop  Create a Quick Launch icon – Creates a MAT application icon on the taskbar for operating systems released

Achieving Operability via the Mission System Paradigm

NASA Technical Reports Server (NTRS)

Hammer, Fred J.; Kahr, Joseph R.

2006-01-01

In the past, flight and ground systems have been developed largely-independently, with the flight system taking the lead, and dominating the development process. Operability issues have been addressed poorly in planning, requirements, design, I&T, and system-contracting activities. In many cases, as documented in lessons-learned, this has resulted in significant avoidable increases in cost and risk. With complex missions and systems, operability is being recognized as an important end-to-end design issue. Never-the-less, lessons-learned and operability concepts remain, in many cases, poorly understood and sporadically applied. A key to effective application of operability concepts is adopting a 'mission system' paradigm. In this paradigm, flight and ground systems are treated, from an engineering and management perspective, as inter-related elements of a larger mission system. The mission system consists of flight hardware, flight software, telecom services, ground data system, testbeds, flight teams, science teams, flight operations processes, procedures, and facilities. The system is designed in functional layers, which span flight and ground. It is designed in response to project-level requirements, mission design and an operations concept, and is developed incrementally, with early and frequent integration of flight and ground components.

Xp11.2 microduplications including IQSEC2, TSPYL2 and KDM5C genes in patients with neurodevelopmental disorders

PubMed Central

Moey, Ching; Hinze, Susan J; Brueton, Louise; Morton, Jenny; McMullan, Dominic J; Kamien, Benjamin; Barnett, Christopher P; Brunetti-Pierri, Nicola; Nicholl, Jillian; Gecz, Jozef; Shoubridge, Cheryl

2016-01-01

Copy number variations are a common cause of intellectual disability (ID). Determining the contribution of copy number variants (CNVs), particularly gains, to disease remains challenging. Here, we report four males with ID with sub-microscopic duplications at Xp11.2 and review the few cases with overlapping duplications reported to date. We established the extent of the duplicated regions in each case encompassing a minimum of three known disease genes TSPYL2, KDM5C and IQSEC2 with one case also duplicating the known disease gene HUWE1. Patients with a duplication encompassing TSPYL2, KDM5C and IQSEC2 without gains of nearby SMC1A and HUWE1 genes have not been reported thus far. All cases presented with ID and significant deficits of speech development. Some patients also manifested behavioral disturbances such as hyperactivity and attention-deficit/hyperactivity disorder. Lymphoblastic cell lines from patients show markedly elevated levels of TSPYL2, KDM5C and SMC1A, transcripts consistent with the extent of their CNVs. The duplicated region in our patients contains several genes known to escape X-inactivation, including KDM5C, IQSEC2 and SMC1A. In silico analysis of expression data in selected gene expression omnibus series indicates that dosage of these genes, especially IQSEC2, is similar in males and females despite the fact they escape from X-inactivation in females. Taken together, the data suggest that gains in Xp11.22 including IQSEC2 cause ID and are associated with hyperactivity and attention-deficit/hyperactivity disorder, and are likely to be dosage-sensitive in males. PMID:26059843

Xp11.2 microduplications including IQSEC2, TSPYL2 and KDM5C genes in patients with neurodevelopmental disorders.

PubMed

Moey, Ching; Hinze, Susan J; Brueton, Louise; Morton, Jenny; McMullan, Dominic J; Kamien, Benjamin; Barnett, Christopher P; Brunetti-Pierri, Nicola; Nicholl, Jillian; Gecz, Jozef; Shoubridge, Cheryl

2016-03-01

Copy number variations are a common cause of intellectual disability (ID). Determining the contribution of copy number variants (CNVs), particularly gains, to disease remains challenging. Here, we report four males with ID with sub-microscopic duplications at Xp11.2 and review the few cases with overlapping duplications reported to date. We established the extent of the duplicated regions in each case encompassing a minimum of three known disease genes TSPYL2, KDM5C and IQSEC2 with one case also duplicating the known disease gene HUWE1. Patients with a duplication encompassing TSPYL2, KDM5C and IQSEC2 without gains of nearby SMC1A and HUWE1 genes have not been reported thus far. All cases presented with ID and significant deficits of speech development. Some patients also manifested behavioral disturbances such as hyperactivity and attention-deficit/hyperactivity disorder. Lymphoblastic cell lines from patients show markedly elevated levels of TSPYL2, KDM5C and SMC1A, transcripts consistent with the extent of their CNVs. The duplicated region in our patients contains several genes known to escape X-inactivation, including KDM5C, IQSEC2 and SMC1A. In silico analysis of expression data in selected gene expression omnibus series indicates that dosage of these genes, especially IQSEC2, is similar in males and females despite the fact they escape from X-inactivation in females. Taken together, the data suggest that gains in Xp11.22 including IQSEC2 cause ID and are associated with hyperactivity and attention-deficit/hyperactivity disorder, and are likely to be dosage-sensitive in males.

An Investigation of the McDonnell XP-85 Airplane in the Ames 40- by 80-Foot Wind Tunnel: Pressure-Distribution Tests

NASA Technical Reports Server (NTRS)

Hunton, Lynn W.; James, Harry A.

1948-01-01

Pressure measurements were made during wind-tunnel tests of the McDonnell XP-85 parasite fighter. Static-pressure orifices were located over the fuselage nose, over the canopy, along the wing root, and along the upper and lower stabilizer roots. A total-pressure and static-pressure rake was located in the turbojet engine air-intake duct. It was installed at the station where the compressor face would be located. Pressure data were obtained for two airplane conditions, clean and with skyhook extended, through a range of angle of attack and a range of yaw.

Xp11.2 translocation renal cell carcinoma occurring during pregnancy with a novel translocation involving chromosome 19: a case report with review of the literature

PubMed Central

Armah, Henry B; Parwani, Anil V; Surti, Urvashi; Bastacky, Sheldon I

2009-01-01

The recently recognized renal cell carcinomas (RCCs) associated with Xp11.2 translocations (TFE3 transcription factor gene fusions) are rare tumors predominantly reported in children. They comprise at least one-third of pediatric RCCs and only few adult cases have been reported. Here, we present a case of Xp11.2 translocation RCC in 26-year-old pregnant female. Her routine antenatal ultrasonography accidentally found a complex cystic right renal mass. Further radiologic studies revealed unilocular cyst with multiple mural nodules at inferior pole of right kidney, which was suspicious for RCC. She underwent right radical nephrectomy at 15 weeks gestation. Macroscopically, the cystic tumor was well encapsulated with multiple friable mural nodules on its inner surface. Microscopically, the tumor consisted of clear and eosinophilic/oncocytic voluminous cells arranged in papillary, trabecular, and nested/alveolar patterns. Occasional hyaline nodules and numerous psammoma bodies were present. Immunohistochemically, the tumor showed strong nuclear positivity for TFE3. Epithelial membrane antigen, CD10, and E-cadherin were strongly positive. Cytokeratin AE1/AE3, cytokeratin CAM-5.2, calveolin, and parvalbumin were moderately positive. Cytokeratin 7, renal cell carcinoma antigen, and colloidal iron were focally weakly positive. BerEP4 and carbonic anhydrase IX were negative. Cytogenetically, the tumor harbored a novel variant translocation involving chromosomes X and 19, t(X;19)(p11.2;q13.1). Interphase FISH analysis performed on cultured and uncultured tumor cells using a dual-color break-apart DNA probe within the BCL3 gene on 19q13.3 was negative for the BCL3 gene rearrangement. She received no adjuvant therapy, delivered a normal term baby five months later, and is alive without evidence of disease 27 months after diagnosis and surgery. Unlike most recently reported Xp11.2 translocation RCCs in adult patients with aggressive clinical course, this adult case occurring

Xp11.2 translocation renal cell carcinoma occurring during pregnancy with a novel translocation involving chromosome 19: a case report with review of the literature.

PubMed

Armah, Henry B; Parwani, Anil V; Surti, Urvashi; Bastacky, Sheldon I

2009-05-18

The recently recognized renal cell carcinomas (RCCs) associated with Xp11.2 translocations (TFE3 transcription factor gene fusions) are rare tumors predominantly reported in children. They comprise at least one-third of pediatric RCCs and only few adult cases have been reported. Here, we present a case of Xp11.2 translocation RCC in 26-year-old pregnant female. Her routine antenatal ultrasonography accidentally found a complex cystic right renal mass. Further radiologic studies revealed unilocular cyst with multiple mural nodules at inferior pole of right kidney, which was suspicious for RCC. She underwent right radical nephrectomy at 15 weeks gestation. Macroscopically, the cystic tumor was well encapsulated with multiple friable mural nodules on its inner surface. Microscopically, the tumor consisted of clear and eosinophilic/oncocytic voluminous cells arranged in papillary, trabecular, and nested/alveolar patterns. Occasional hyaline nodules and numerous psammoma bodies were present.Immunohistochemically, the tumor showed strong nuclear positivity for TFE3. Epithelial membrane antigen, CD10, and E-cadherin were strongly positive. Cytokeratin AE1/AE3, cytokeratin CAM-5.2, calveolin, and parvalbumin were moderately positive. Cytokeratin 7, renal cell carcinoma antigen, and colloidal iron were focally weakly positive. BerEP4 and carbonic anhydrase IX were negative. Cytogenetically, the tumor harbored a novel variant translocation involving chromosomes X and 19, t(X;19)(p11.2;q13.1). Interphase FISH analysis performed on cultured and uncultured tumor cells using a dual-color break-apart DNA probe within the BCL3 gene on 19q13.3 was negative for the BCL3 gene rearrangement. She received no adjuvant therapy, delivered a normal term baby five months later, and is alive without evidence of disease 27 months after diagnosis and surgery. Unlike most recently reported Xp11.2 translocation RCCs in adult patients with aggressive clinical course, this adult case occurring

Transport systems research vehicle color display system operations manual

NASA Technical Reports Server (NTRS)

Easley, Wesley C.; Johnson, Larry E.

1989-01-01

A recent upgrade of the Transport Systems Research Vehicle operated by the Advanced Transport Operating Systems Program Office at the NASA Langley Research Center has resulted in an all-glass panel in the research flight deck. Eight ARINC-D size CRT color displays make up the panel. A major goal of the display upgrade effort was ease of operation and maintenance of the hardware while maintaining versatility needed for flight research. Software is the key to this required versatility and will be the area demanding the most detailed technical design expertise. This document is is intended to serve as a single source of quick reference information needed for routine operation and system level maintenance. Detailed maintenance and modification of the display system will require specific design documentation and must be accomplished by individuals with specialized knowledge and experience.

Network operating system focus technology

NASA Technical Reports Server (NTRS)

1985-01-01

An activity structured to provide specific design requirements and specifications for the Space Station Data Management System (DMS) Network Operating System (NOS) is outlined. Examples are given of the types of supporting studies and implementation tasks presently underway to realize a DMS test bed capability to develop hands-on understanding of NOS requirements as driven by actual subsystem test beds participating in the overall Johnson Space Center test bed program. Classical operating system elements and principal NOS functions are listed.

Investigation of the Flying Mock-Up of Consolidated Vultee XP-92 Airplane in the Ames 40- by 80-Foot Wind Tunnel: Pressure Distributions

NASA Technical Reports Server (NTRS)

Graham, David

1948-01-01

This report contains the results of the wind tunnel investigation of the pressure distribution on the flying mock-up of the Consolidated Vultee XP-92 airplane. Data are presented for the pressure distribution over the wing, vertical tail and the fuselage, and for the pressure loss and rate of flow through the ducted fuselage. Data are also presented for the calibration of two airspeed indicators, and for the calibration of angle-of-attack and sideslip-angle indicator vanes.

High-Performance Computing Systems and Operations | Computational Science |

Science.gov Websites

NREL Systems and Operations High-Performance Computing Systems and Operations NREL operates high-performance computing (HPC) systems dedicated to advancing energy efficiency and renewable energy technologies. Capabilities NREL's HPC capabilities include: High-Performance Computing Systems We operate

Bird Vision System

NASA Technical Reports Server (NTRS)

2008-01-01

The Bird Vision system is a multicamera photogrammerty software application that runs on a Microsoft Windows XP platform and was developed at Kennedy Space Center by ASRC Aerospace. This software system collects data about the locations of birds within a volume centered on the Space Shuttle and transmits it in real time to the laptop computer of a test director in the Launch Control Center (LCC) Firing Room.

Prognostic Analysis System and Methods of Operation

NASA Technical Reports Server (NTRS)

MacKey, Ryan M. E. (Inventor); Sneddon, Robert (Inventor)

2014-01-01

A prognostic analysis system and methods of operating the system are provided. In particular, a prognostic analysis system for the analysis of physical system health applicable to mechanical, electrical, chemical and optical systems and methods of operating the system are described herein.

System Shock: The Archetype of Operational Shock

DTIC Science & Technology

2017-05-25

System Shock: The Archetype of Operational Shock A Monograph by MAJ Benjamen A. McDaniel United States Army School of Advanced Military...Mongraphs 3. DATES COVERED (From - To) JUN 2016- MAY 2017 4. TITLE AND SUBTITLE System Shock: The Archetype of Operational Shock 5a. CONTRACT NUMBER...is whether the US can use the concept of ‘ system shock’ to better operate in the Grey Zone. ‘ System shock’ combines ideas from systems theory, chaos

Science Orders Systems and Operations Manual.

ERIC Educational Resources Information Center

Kriz, Harry M.

This manual describes the implementation and operation of SCIENCE ORDERS, an online orders management system used by the Science and Technology Department of Newman Library at Virginia Polytechnic Institute and State University. Operational since January 1985, the system is implemented using the SPIRES database management system and is used to (1)…

Computer-aided operations engineering with integrated models of systems and operations

NASA Technical Reports Server (NTRS)

Malin, Jane T.; Ryan, Dan; Fleming, Land

1994-01-01

CONFIG 3 is a prototype software tool that supports integrated conceptual design evaluation from early in the product life cycle, by supporting isolated or integrated modeling, simulation, and analysis of the function, structure, behavior, failures and operation of system designs. Integration and reuse of models is supported in an object-oriented environment providing capabilities for graph analysis and discrete event simulation. Integration is supported among diverse modeling approaches (component view, configuration or flow path view, and procedure view) and diverse simulation and analysis approaches. Support is provided for integrated engineering in diverse design domains, including mechanical and electro-mechanical systems, distributed computer systems, and chemical processing and transport systems. CONFIG supports abstracted qualitative and symbolic modeling, for early conceptual design. System models are component structure models with operating modes, with embedded time-related behavior models. CONFIG supports failure modeling and modeling of state or configuration changes that result in dynamic changes in dependencies among components. Operations and procedure models are activity structure models that interact with system models. CONFIG is designed to support evaluation of system operability, diagnosability and fault tolerance, and analysis of the development of system effects of problems over time, including faults, failures, and procedural or environmental difficulties.

The Advanced Technology Operations System: ATOS

NASA Technical Reports Server (NTRS)

Kaufeler, J.-F.; Laue, H. A.; Poulter, K.; Smith, H.

1993-01-01

Mission control systems supporting new space missions face ever-increasing requirements in terms of functionality, performance, reliability and efficiency. Modern data processing technology is providing the means to meet these requirements in new systems under development. During the past few years the European Space Operations Centre (ESOC) of the European Space Agency (ESA) has carried out a number of projects to demonstrate the feasibility of using advanced software technology, in particular, knowledge based systems, to support mission operations. A number of advances must be achieved before these techniques can be moved towards operational use in future missions, namely, integration of the applications into a single system framework and generalization of the applications so that they are mission independent. In order to achieve this goal, ESA initiated the Advanced Technology Operations System (ATOS) program, which will develop the infrastructure to support advanced software technology in mission operations, and provide applications modules to initially support: Mission Preparation, Mission Planning, Computer Assisted Operations, and Advanced Training. The first phase of the ATOS program is tasked with the goal of designing and prototyping the necessary system infrastructure to support the rest of the program. The major components of the ATOS architecture is presented. This architecture relies on the concept of a Mission Information Base (MIB) as the repository for all information and knowledge which will be used by the advanced application modules in future mission control systems. The MIB is being designed to exploit the latest in database and knowledge representation technology in an open and distributed system. In conclusion the technological and implementation challenges expected to be encountered, as well as the future plans and time scale of the project, are presented.

Application of System Operational Effectiveness Methodology to Space Launch Vehicle Development and Operations

NASA Technical Reports Server (NTRS)

Watson, Michael D.; Kelley, Gary W.

2012-01-01

The Department of Defense (DoD) defined System Operational Effectiveness (SOE) model provides an exceptional framework for an affordable approach to the development and operation of space launch vehicles and their supporting infrastructure. The SOE model provides a focal point from which to direct and measure technical effectiveness and process efficiencies of space launch vehicles. The application of the SOE model to a space launch vehicle's development and operation effort leads to very specific approaches and measures that require consideration during the design phase. This paper provides a mapping of the SOE model to the development of space launch vehicles for human exploration by addressing the SOE model key points of measurement including System Performance, System Availability, Technical Effectiveness, Process Efficiency, System Effectiveness, Life Cycle Cost, and Affordable Operational Effectiveness. In addition, the application of the SOE model to the launch vehicle development process is defined providing the unique aspects of space launch vehicle production and operations in lieu of the traditional broader SOE context that examines large quantities of fielded systems. The tailoring and application of the SOE model to space launch vehicles provides some key insights into the operational design drivers, capability phasing, and operational support systems.

Skylab hardware report operational bioinstrumentation system

NASA Technical Reports Server (NTRS)

Luczkowski, S.

1977-01-01

The Skylab Operational Bioinstrumentation System is a personal, individually adjustable biomedical system designed to monitor the basic physiological functions of each suited crewman during specified periods of a manned space mission. The basic physiological functions of this system include electrocardiogram, respiration by impedance pneumogram, body temperature, cardiotachometer, and subject identification. The Operational Bioinstrumentation System was scheduled to monitor each crewman during launch, extravehicular activities, suited intravehicular experiments, and undocking and return.

Terminal-area STOL operating systems experiments program

NASA Technical Reports Server (NTRS)

Smith, D. W.; Watson, D.; Christensen, J. V.

1973-01-01

Information which will aid in the choice by the U.S. Government and industry of system concepts, design criteria, operating procedures for STOL aircraft and STOL ports, STOL landing guidance systems, air traffic control systems, and airborne avionics and flight control systems. Ames has developed a terminal-area STOL operating systems experiments program which is a part of the joint DOT/NASA effort is discussed. The Ames operating systems experiments program, its objectives, the program approach, the program schedule, typical experiments, the research facilities to be used, and the program status are described.

Advanced Autonomous Systems for Space Operations

NASA Astrophysics Data System (ADS)

Gross, A. R.; Smith, B. D.; Muscettola, N.; Barrett, A.; Mjolssness, E.; Clancy, D. J.

2002-01-01

New missions of exploration and space operations will require unprecedented levels of autonomy to successfully accomplish their objectives. Inherently high levels of complexity, cost, and communication distances will preclude the degree of human involvement common to current and previous space flight missions. With exponentially increasing capabilities of computer hardware and software, including networks and communication systems, a new balance of work is being developed between humans and machines. This new balance holds the promise of not only meeting the greatly increased space exploration requirements, but simultaneously dramatically reducing the design, development, test, and operating costs. New information technologies, which take advantage of knowledge-based software, model-based reasoning, and high performance computer systems, will enable the development of a new generation of design and development tools, schedulers, and vehicle and system health management capabilities. Such tools will provide a degree of machine intelligence and associated autonomy that has previously been unavailable. These capabilities are critical to the future of advanced space operations, since the science and operational requirements specified by such missions, as well as the budgetary constraints will limit the current practice of monitoring and controlling missions by a standing army of ground-based controllers. System autonomy capabilities have made great strides in recent years, for both ground and space flight applications. Autonomous systems have flown on advanced spacecraft, providing new levels of spacecraft capability and mission safety. Such on-board systems operate by utilizing model-based reasoning that provides the capability to work from high-level mission goals, while deriving the detailed system commands internally, rather than having to have such commands transmitted from Earth. This enables missions of such complexity and communication` distances as are not

Multiple operating system rotation environment moving target defense

DOE Office of Scientific and Technical Information (OSTI.GOV)

Evans, Nathaniel; Thompson, Michael

Systems and methods for providing a multiple operating system rotation environment ("MORE") moving target defense ("MTD") computing system are described. The MORE-MTD system provides enhanced computer system security through a rotation of multiple operating systems. The MORE-MTD system increases attacker uncertainty, increases the cost of attacking the system, reduces the likelihood of an attacker locating a vulnerability, and reduces the exposure time of any located vulnerability. The MORE-MTD environment is effectuated by rotation of the operating systems at a given interval. The rotating operating systems create a consistently changing attack surface for remote attackers.

ORATOS: ESA's future flight dynamics operations system

NASA Astrophysics Data System (ADS)

Dreger, Frank; Fertig, Juergen; Muench, Rolf

The Orbit and Attitude Operations System (ORATOS -- the European Space Agency's future orbit and attitude operations system -- will be in use from the mid-nineties until well beyond the year 2000. The ORATOS design is based on the experience from flight dynamics support to all past ESA missions. The ORATOS computer hardware consists of a network of powerful UNIX workstations. ORATOS resides on several hardware platforms, each comprising one or more fileservers, several client workstations and the associated communications interface hardware. The ORATOS software is structured into three layers. The flight dynamics applications layer, the support layer and the operating system layer. This architectural design separates the flight dynamics application software from the support tools and operating system facilities. It allows upgrading and replacement of operating system facilities with a minimum (or no) effect on the application layer.

OFMTutor: An operator function model intelligent tutoring system

NASA Technical Reports Server (NTRS)

Jones, Patricia M.

1989-01-01

The design, implementation, and evaluation of an Operator Function Model intelligent tutoring system (OFMTutor) is presented. OFMTutor is intended to provide intelligent tutoring in the context of complex dynamic systems for which an operator function model (OFM) can be constructed. The human operator's role in such complex, dynamic, and highly automated systems is that of a supervisory controller whose primary responsibilities are routine monitoring and fine-tuning of system parameters and occasional compensation for system abnormalities. The automated systems must support the human operator. One potentially useful form of support is the use of intelligent tutoring systems to teach the operator about the system and how to function within that system. Previous research on intelligent tutoring systems (ITS) is considered. The proposed design for OFMTutor is presented, and an experimental evaluation is described.

Vibrational spectra of Mg2KH(XO4)2·15H2O (X=P, As) containing dimer units [H(XO4)2].

PubMed

Stefov, V; Koleva, V; Najdoski, M; Abdija, Z; Cahil, A; Šoptrajanov, B

2017-08-05

Infrared and Raman spectra of Mg 2 KH(PO 4 ) 2 ·15H 2 O and Mg 2 KH(AsO 4 ) 2 ·15H 2 O and a series of their partially deuterated analogues were recorded and analyzed. Compounds of the type Mg 2 KH(XO 4 ) 2 ·15H 2 O (X=P, As) are little-known and a rare case of phosphate and arsenate salts containing dimer units [H(XO 4 ) 2 ] in the crystal structure. The analysis of their IR spectra (recorded at room and liquid nitrogen temperature) and Raman spectra showed that the spectral characteristics of the XO 4 groups connected in a dimer through a proton are not consistent with the presence of X-O-H covalent linkage and C 1 crystallographic symmetry of the XO 4 groups. The observation of a singlet Raman band for the ν 1 (XO 4 ) mode as well as the absence of substantial splitting of the ν 3 (XO 4 ) modes and IR activation of the ν 1 (XO 4 ) mode suggest that the dimer units [H(XO 4 ) 2 ] are most probably symmetric rather than non-symmetric ones. It was found that, in the vibrational spectra of Mg 2 KH(AsO 4 ) 2 ·15H 2 O, both ν 1 (AsО 4 ) and ν 3 (AsО 4 ) modes have practically the same wavenumber around 830cm -1 . It was also established that the ν 4 (PО 4 ) modes in the deuterated hydrogendiphosphate compound are strongly coupled, most probably with HDO and/or D 2 O librations. As a whole, the spectral picture of Mg 2 KH(XO 4 ) 2 ·15H 2 O (X=P, As) very much resembles that observed for the struvite type compounds with the formula KMgXO 4 ·6H 2 O (X=P, As) which do not contain X-OH groups. This means that vibrations of the dimers [H(XO 4 ) 2 ] play a relatively small part in the general spectral appearance. Copyright © 2017 Elsevier B.V. All rights reserved.

Can HN[double bond, length as m-dash]NH, FN[double bond, length as m-dash]NH, or HN[double bond, length as m-dash]CHOH bridge the σ-hole and the lone pair at P in binary complexes with H2XP, for X = F, Cl, NC, OH, CN, CCH, CH3, and H?

PubMed

Del Bene, Janet E; Alkorta, Ibon; Elguero, José

2015-11-11

Ab initio MP2/aug'-cc-pVTZ calculations have been carried out to investigate the properties of complexes formed between H2XP, for X = F, Cl, NC, OH, CN, CCH, CH3, and H, and the possible bridging molecules HN[double bond, length as m-dash]NH, FN[double bond, length as m-dash]NH, and HN[double bond, length as m-dash]CHOH. H2XP:HNNH and H2XP:FNNH complexes are stabilized by PN pnicogen bonds, except for H2(CH3)P:FNNH and H3P:FNNH which are stabilized by N-HP hydrogen bonds. H2XP:HNCHOH complexes are stabilized by PN pnicogen bonds and nonlinear O-HP hydrogen bonds. For a fixed H2XP molecule, binding energies decrease in the order HNCHOH > HNNH > FNNH, except for the binding energies of H2(CH3)P and H3P with HNNH and FNNH. Binding energies of complexes with HNCHOH and HNNH increase as the P-N1 distance decreases, but binding energies of complexes with FNNH show little dependence on this distance. The large binding energies of H2XP:HNCHOH complexes arise from a cooperative effect involving electron-pair acceptance by P to form a pnicogen bond, and electron-pair donation by P to form a hydrogen bond. The dominant charge-transfer interaction in these complexes involves electron-pair donation by N across the pnicogen bond, except for complexes in which X is one of the more electropositive substituents, CCH, CH3, and H. For these, lone-pair donation by P across the hydrogen bond dominates. AIM and NBO data for these complexes are consistent with their bonding characteristics, showing molecular graphs with bond critical points and charge-transfer interactions associated with hydrogen and pnicogen bonds. EOM-CCSD spin-spin coupling constants (1p)J(P-N) across the pnicogen bond for each series of complexes correlate with the P-N distance. In contrast, (2h)J(O-P) values for complexes H2XP:HNCHOH do not correlate with the O-P distance, a consequence of the nonlinearity of these hydrogen bonds.

47 CFR 101.1009 - System operations.

Code of Federal Regulations, 2012 CFR

2012-10-01

... 47 Telecommunication 5 2012-10-01 2012-10-01 false System operations. 101.1009 Section 101.1009 Telecommunication FEDERAL COMMUNICATIONS COMMISSION (CONTINUED) SAFETY AND SPECIAL RADIO SERVICES FIXED MICROWAVE SERVICES Local Multipoint Distribution Service § 101.1009 System operations. (a) The licensee may construct...

47 CFR 101.1009 - System operations.

Code of Federal Regulations, 2011 CFR

2011-10-01

... 47 Telecommunication 5 2011-10-01 2011-10-01 false System operations. 101.1009 Section 101.1009 Telecommunication FEDERAL COMMUNICATIONS COMMISSION (CONTINUED) SAFETY AND SPECIAL RADIO SERVICES FIXED MICROWAVE SERVICES Local Multipoint Distribution Service § 101.1009 System operations. (a) The licensee may construct...

47 CFR 101.1009 - System operations.

Code of Federal Regulations, 2013 CFR

2013-10-01

... 47 Telecommunication 5 2013-10-01 2013-10-01 false System operations. 101.1009 Section 101.1009 Telecommunication FEDERAL COMMUNICATIONS COMMISSION (CONTINUED) SAFETY AND SPECIAL RADIO SERVICES FIXED MICROWAVE SERVICES Local Multipoint Distribution Service § 101.1009 System operations. (a) The licensee may construct...

47 CFR 101.1009 - System operations.

Code of Federal Regulations, 2014 CFR

2014-10-01

... 47 Telecommunication 5 2014-10-01 2014-10-01 false System operations. 101.1009 Section 101.1009 Telecommunication FEDERAL COMMUNICATIONS COMMISSION (CONTINUED) SAFETY AND SPECIAL RADIO SERVICES FIXED MICROWAVE SERVICES Local Multipoint Distribution Service § 101.1009 System operations. (a) The licensee may construct...

47 CFR 101.1009 - System operations.

Code of Federal Regulations, 2010 CFR

2010-10-01

... 47 Telecommunication 5 2010-10-01 2010-10-01 false System operations. 101.1009 Section 101.1009 Telecommunication FEDERAL COMMUNICATIONS COMMISSION (CONTINUED) SAFETY AND SPECIAL RADIO SERVICES FIXED MICROWAVE SERVICES Local Multipoint Distribution Service § 101.1009 System operations. (a) The licensee may construct...

Operationally Efficient Propulsion System Study (OEPSS) data book. Volume 1: Generic ground operations data

NASA Technical Reports Server (NTRS)

Byrd, Raymond J.

1990-01-01

This study was initiated to identify operations problems and cost drivers for current propulsion systems and to identify technology and design approaches to increase the operational efficiency and reduce operations costs for future propulsion systems. To provide readily usable data for the Advance Launch System (ALS) program, the results of the Operationally Efficient Propulsion System Study (OEPSS) were organized into a series of OEPSS Data Books as follows: Volume 1, Generic Ground Operations Data; Volume 2, Ground Operations Problems; Volume 3, Operations Technology; Volume 4, OEPSS Design Concepts; and Volume 5, OEPSS Final Review Briefing, which summarizes the activities and results of the study. This volume presents ground processing data for a generic LOX/LH2 booster and core propulsion system based on current STS experience. The data presented includes: top logic diagram, process flow, activities bar-chart, loaded timelines, manpower requirements in terms of duration, headcount and skill mix per operations and maintenance instruction (OMI), and critical path tasks and durations.

Pediatric renal cell carcinoma associated with Xp11.2 translocation/TFE3 gene fusion.

PubMed

Winarti, Ni Wayan; Argani, Pedram; De Marzo, Angelo M; Hicks, Jessica; Mulyadi, Ketut

2008-01-01

Renal cell carcinoma (RCC) in children and young adults is rare and pathologically problematic. RCC can be either hereditary or sporadic and has a guarded prognosis because appropriate management has not been established. A case of RCC in an 11-year-old is reported. The clinical presentation was a right abdominal mass, hematuria, urinary tract infection, and wasting. Radio-logically, the mass was found within the right kidney with calcification and paraaortic lymphadenopathy. The postsurgical diagnosis was Wilms' tumor stage T4N2M0. On gross inspection, the tumor was ill defined, extending across Gerota's fascia and into the ureter lumina. Microscopically, the tumor consisted of malignant epithelial cells with clear and eosinophilic cytoplasm in nested, papillary, and alveolar configuration. Hyaline nodules, psammoma bodies, vascular invasion, capsular invasion, and extension into the ureter were also found. Immunohistochemically, the cells showed strong nuclear immunoreactivity for TFE3. We concluded that this case was an RCC associated with Xp11.2 translocation/TFE3 fusion, Fuhrman grade 3, stage IV.

The effect of PO 4 doping on the luminescent properties of Sr 3-3zEu 2zV 2-xP xO 8

NASA Astrophysics Data System (ADS)

Cao, S.; Ma, Y. Q.; Yang, K.; Zhu, W. L.; Yin, W. J.; Zheng, G. H.; Wu, M. Z.; Sun, Z. Q.

2010-07-01

The luminescent properties of Sr 3V 2-xP xO 8 (0 ⩽ x ⩽ 2), Eu 3+ doped Sr 2.7Eu 0.2V 2-yP yO 8 (0 ⩽ y ⩽ 2) and Sr 3-3zEu 2zV 0.8P 1.2O 8 (0 < z ⩽ 0.3) have been investigated. For the Sr 3V 2-xP xO 8 (0 ⩽ x ⩽ 2) samples, the VO43- activation and emission intensity reaches the strongest as x = 1.6. For the Sr 2.7Eu 0.2V 2-yP yO 8 (0 ⩽ y ⩽ 2) samples, an appropriate amount of phosphorus doping enhances the Eu 3+ emission with the strongest emission occurring at y = 1.2. For the Sr 3-3zEu 2zV 0.8P 1.2O 8 (0 < z ⩽ 0.3) sample with the phosphorus content fixed at 1.2, it exhibits the most intense emission as Eu 3+ concentration reaches at z = 0.2. Our results indicate that the introduction of the PO43- plays an important role in the photoluminescence properties of the studied samples and the relevant mechanism has been discussed.

Operational Characteristics of an Accelerator Driven Fissile Solution System

DOE Office of Scientific and Technical Information (OSTI.GOV)

Kimpland, Robert Herbert

Operational characteristics represent the set of responses that a nuclear system exhibits during normal operation. Operators rely on this behavior to assess the status of the system and to predict the consequences of off-normal events. These characteristics largely refer to the relationship between power and system operating conditions. The static and dynamic behavior of a chain-reacting system, operating at sufficient power, is primarily governed by reactivity effects. The science of reactor physics has identified and evaluated a number of such effects, including Doppler broadening and shifts in the thermal neutron spectrum. Often these reactivity effects are quantified in the formmore » of feedback coefficients that serve as coupling coefficients relating the neutron population and the physical mechanisms that drive reactivity effects, such as fissile material temperature and density changes. The operational characteristics of such nuclear systems usually manifest themselves when perturbations between system power (neutron population) and system operating conditions arise. Successful operation of such systems requires the establishment of steady equilibrium conditions. However, prior to obtaining the desired equilibrium (steady-state) conditions, an approach from zero-power (startup) must occur. This operational regime may possess certain limiting system conditions that must be maintained to achieve effective startup. Once steady-state is achieved, a key characteristic of this operational regime is the level of stability that the system possesses. Finally, a third operational regime, shutdown, may also possess limiting conditions of operation that must be maintained. This report documents the operational characteristics of a “generic” Accelerator Driven Fissile Solution (ADFS) system during the various operational regimes of startup, steady-state operation, and shutdown. Typical time-dependent behavior for each operational regime will be illustrated, and

Spatial Operator Algebra for multibody system dynamics

NASA Technical Reports Server (NTRS)

Rodriguez, G.; Jain, A.; Kreutz-Delgado, K.

1992-01-01

The Spatial Operator Algebra framework for the dynamics of general multibody systems is described. The use of a spatial operator-based methodology permits the formulation of the dynamical equations of motion of multibody systems in a concise and systematic way. The dynamical equations of progressively more complex grid multibody systems are developed in an evolutionary manner beginning with a serial chain system, followed by a tree topology system and finally, systems with arbitrary closed loops. Operator factorizations and identities are used to develop novel recursive algorithms for the forward dynamics of systems with closed loops. Extensions required to deal with flexible elements are also discussed.

From Operating-System Correctness to Pervasively Verified Applications

NASA Astrophysics Data System (ADS)

Daum, Matthias; Schirmer, Norbert W.; Schmidt, Mareike

Though program verification is known and has been used for decades, the verification of a complete computer system still remains a grand challenge. Part of this challenge is the interaction of application programs with the operating system, which is usually entrusted with retrieving input data from and transferring output data to peripheral devices. In this scenario, the correct operation of the applications inherently relies on operating-system correctness. Based on the formal correctness of our real-time operating system Olos, this paper describes an approach to pervasively verify applications running on top of the operating system.

49 CFR 234.257 - Warning system operation.

Code of Federal Regulations, 2012 CFR

2012-10-01

... 49 Transportation 4 2012-10-01 2012-10-01 false Warning system operation. 234.257 Section 234.257... EMERGENCY NOTIFICATION SYSTEMS Maintenance, Inspection, and Testing Inspections and Tests § 234.257 Warning system operation. (a) Each highway-rail crossing warning system shall be tested to determine that it...

49 CFR 234.257 - Warning system operation.

Code of Federal Regulations, 2014 CFR

2014-10-01

... 49 Transportation 4 2014-10-01 2014-10-01 false Warning system operation. 234.257 Section 234.257... EMERGENCY NOTIFICATION SYSTEMS Maintenance, Inspection, and Testing Inspections and Tests § 234.257 Warning system operation. (a) Each highway-rail crossing warning system shall be tested to determine that it...

49 CFR 234.257 - Warning system operation.

Code of Federal Regulations, 2013 CFR

2013-10-01

... 49 Transportation 4 2013-10-01 2013-10-01 false Warning system operation. 234.257 Section 234.257... EMERGENCY NOTIFICATION SYSTEMS Maintenance, Inspection, and Testing Inspections and Tests § 234.257 Warning system operation. (a) Each highway-rail crossing warning system shall be tested to determine that it...

An approach to operating system testing

NASA Technical Reports Server (NTRS)

Sum, R. N., Jr.; Campbell, R. H.; Kubitz, W. J.

1984-01-01

To ensure the reliability and performance of a new system, it must be verified or validated in some manner. Currently, testing is the only resonable technique available for doing this. Part of this testing process is the high level system test. System testing is considered with respect to operating systems and in particular UNIX. This consideration results in the development and presentation of a good method for performing the system test. The method includes derivations from the system specifications and ideas for management of the system testing project. Results of applying the method to the IBM System/9000 XENIX operating system test and the development of a UNIX test suite are presented.

Investigation of the Flying Mock-Up of the Consolidated Vultee XP-92 Airplane in the Ames 40- by 80-Foot Wind Tunnel. Force and Moment Characteristics

NASA Technical Reports Server (NTRS)

Wick, Bradford, H.; Graham, David

1948-01-01

This report contains the results of the investigation of the aerodynamic characteristics of the flying mock-up of the Consolidated Vultee XP-92 airplane as conducted in the Ames 40- by 80-foot wind tunnel, Data are presented for test conditions which would give information as to the limits of stability and controllability, and also, the effect of Reynolds number. No analysis of the data has been made.

U.S. Electric System Operating Data

EIA Publications

EIA provides hourly electricity operating data, including actual and forecast demand, net generation, and the power flowing between electric systems. EIA's new U.S. Electric System Operating Data tool provides nearly real-time demand data, plus analysis and visualizations of hourly, daily, and weekly electricity supply and demand on a national and regional level for all of the 66 electric system balancing authorities that make up the U.S. electric grid.

PILOT: An intelligent distributed operations support system

NASA Technical Reports Server (NTRS)

Rasmussen, Arthur N.

1993-01-01

The Real-Time Data System (RTDS) project is exploring the application of advanced technologies to the real-time flight operations environment of the Mission Control Centers at NASA's Johnson Space Center. The system, based on a network of engineering workstations, provides services such as delivery of real time telemetry data to flight control applications. To automate the operation of this complex distributed environment, a facility called PILOT (Process Integrity Level and Operation Tracker) is being developed. PILOT comprises a set of distributed agents cooperating with a rule-based expert system; together they monitor process operation and data flows throughout the RTDS network. The goal of PILOT is to provide unattended management and automated operation under user control.

49 CFR 325.37 - Location and operation of sound level measurement system; highway operations.

Code of Federal Regulations, 2013 CFR

2013-10-01

... system; highway operations. 325.37 Section 325.37 Transportation Other Regulations Relating to...; Highway Operations § 325.37 Location and operation of sound level measurement system; highway operations..., the holder must orient himself/herself relative to the highway in a manner consistent with the...

49 CFR 325.37 - Location and operation of sound level measurement system; highway operations.

Code of Federal Regulations, 2012 CFR

2012-10-01

... system; highway operations. 325.37 Section 325.37 Transportation Other Regulations Relating to...; Highway Operations § 325.37 Location and operation of sound level measurement system; highway operations..., the holder must orient himself/herself relative to the highway in a manner consistent with the...

49 CFR 325.37 - Location and operation of sound level measurement system; highway operations.

Code of Federal Regulations, 2014 CFR

2014-10-01

... system; highway operations. 325.37 Section 325.37 Transportation Other Regulations Relating to...; Highway Operations § 325.37 Location and operation of sound level measurement system; highway operations..., the holder must orient himself/herself relative to the highway in a manner consistent with the...

Communications Processor Operating System Study. Executive Summary,

DTIC Science & Technology

1980-11-01

AD-A095 b36 ROME AIR DEVELOPMENT CENTER GRIFFISS AFB NY F/e 17/2 COMMUNICATIONS PROCESSOR OPERATING SYSTEM STUDY. EXECUTIVE SUMM—ETC(U) NOV 80 J...COMMUNICATIONS PROCESSOR OPERATING SYSTEM STUDY Julian Gitlih SPTIC ELECTE«^ FEfi 2 6 1981^ - E APPROVED FOR PUBLIC RELEASE; DISTRIBUTION UNLIMITED "a O...Subtitle) EXECUTIVE^SUMMARY 0F> COMMUNICATIONS PROCESSOR OPERATING SYSTEM $t - • >X W tdLl - ’•• • 7 AUTHORf«! ! , Julian

System Operations Studies : Feeder System Model. User's Manual.

DOT National Transportation Integrated Search

1982-11-01

The Feeder System Model (FSM) is one of the analytic models included in the System Operations Studies (SOS) software package developed for urban transit systems analysis. The objective of the model is to assign a proportion of the zone-to-zone travel...

Methodology for Designing Operational Banking Risks Monitoring System

NASA Astrophysics Data System (ADS)

Kostjunina, T. N.

2018-05-01

The research looks at principles of designing an information system for monitoring operational banking risks. A proposed design methodology enables one to automate processes of collecting data on information security incidents in the banking network, serving as the basis for an integrated approach to the creation of an operational risk management system. The system can operate remotely ensuring tracking and forecasting of various operational events in the bank network. A structure of a content management system is described.

Software fault tolerance in computer operating systems

NASA Technical Reports Server (NTRS)

Iyer, Ravishankar K.; Lee, Inhwan

1994-01-01

This chapter provides data and analysis of the dependability and fault tolerance for three operating systems: the Tandem/GUARDIAN fault-tolerant system, the VAX/VMS distributed system, and the IBM/MVS system. Based on measurements from these systems, basic software error characteristics are investigated. Fault tolerance in operating systems resulting from the use of process pairs and recovery routines is evaluated. Two levels of models are developed to analyze error and recovery processes inside an operating system and interactions among multiple instances of an operating system running in a distributed environment. The measurements show that the use of process pairs in Tandem systems, which was originally intended for tolerating hardware faults, allows the system to tolerate about 70% of defects in system software that result in processor failures. The loose coupling between processors which results in the backup execution (the processor state and the sequence of events occurring) being different from the original execution is a major reason for the measured software fault tolerance. The IBM/MVS system fault tolerance almost doubles when recovery routines are provided, in comparison to the case in which no recovery routines are available. However, even when recovery routines are provided, there is almost a 50% chance of system failure when critical system jobs are involved.

Perfect commuting-operator strategies for linear system games

NASA Astrophysics Data System (ADS)

Cleve, Richard; Liu, Li; Slofstra, William

2017-01-01

Linear system games are a generalization of Mermin's magic square game introduced by Cleve and Mittal. They show that perfect strategies for linear system games in the tensor-product model of entanglement correspond to finite-dimensional operator solutions of a certain set of non-commutative equations. We investigate linear system games in the commuting-operator model of entanglement, where Alice and Bob's measurement operators act on a joint Hilbert space, and Alice's operators must commute with Bob's operators. We show that perfect strategies in this model correspond to possibly infinite-dimensional operator solutions of the non-commutative equations. The proof is based around a finitely presented group associated with the linear system which arises from the non-commutative equations.

Design and Effectiveness of Intelligent Tutors for Operators of Complex Dynamic Systems: A Tutor Implementation for Satellite System Operators.

ERIC Educational Resources Information Center

Mitchell, Christine M.; Govindaraj, T.

1990-01-01

Discusses the use of intelligent tutoring systems as opposed to traditional on-the-job training for training operators of complex dynamic systems and describes the computer architecture for a system for operators of a NASA (National Aeronautics and Space Administration) satellite control system. An experimental evaluation with college students is…

The Launch Systems Operations Cost Model

NASA Technical Reports Server (NTRS)

Prince, Frank A.; Hamaker, Joseph W. (Technical Monitor)

2001-01-01

One of NASA's primary missions is to reduce the cost of access to space while simultaneously increasing safety. A key component, and one of the least understood, is the recurring operations and support cost for reusable launch systems. In order to predict these costs, NASA, under the leadership of the Independent Program Assessment Office (IPAO), has commissioned the development of a Launch Systems Operations Cost Model (LSOCM). LSOCM is a tool to predict the operations & support (O&S) cost of new and modified reusable (and partially reusable) launch systems. The requirements are to predict the non-recurring cost for the ground infrastructure and the recurring cost of maintaining that infrastructure, performing vehicle logistics, and performing the O&S actions to return the vehicle to flight. In addition, the model must estimate the time required to cycle the vehicle through all of the ground processing activities. The current version of LSOCM is an amalgamation of existing tools, leveraging our understanding of shuttle operations cost with a means of predicting how the maintenance burden will change as the vehicle becomes more aircraft like. The use of the Conceptual Operations Manpower Estimating Tool/Operations Cost Model (COMET/OCM) provides a solid point of departure based on shuttle and expendable launch vehicle (ELV) experience. The incorporation of the Reliability and Maintainability Analysis Tool (RMAT) as expressed by a set of response surface model equations gives a method for estimating how changing launch system characteristics affects cost and cycle time as compared to today's shuttle system. Plans are being made to improve the model. The development team will be spending the next few months devising a structured methodology that will enable verified and validated algorithms to give accurate cost estimates. To assist in this endeavor the LSOCM team is part of an Agency wide effort to combine resources with other cost and operations professionals to

SIRTF Science Operations System Design

NASA Technical Reports Server (NTRS)

Green, William

1999-01-01

SIRTF Science Operations System Design William B. Green Manager, SIRTF Science Center California Institute of Technology M/S 310-6 1200 E. California Blvd., Pasadena CA 91125 (626) 395 8572 Fax (626) 568 0673 bgreen@ipac.caltech.edu. The Space Infrared Telescope Facility (SIRTF) will be launched in December 2001, and perform an extended series of science observations at wavelengths ranging from 20 to 160 microns for five years or more. The California Institute of Technology has been selected as the home for the SIRTF Science Center (SSC). The SSC will be responsible for evaluating and selecting observation proposals, providing technical support to the science community, performing mission planning and science observation scheduling activities, instrument calibration during operations and instrument health monitoring, production of archival quality data products, and management of science research grants. The science payload consists of three instruments delivered by instrument Principal Investigators located at University of Arizona, Cornell, and Harvard Smithsonian Astrophysical Observatory. The SSC is responsible for design, development, and operation of the Science Operations System (SOS) which will support the functions assigned to the SSC by NASA. The SIRTF spacecraft, mission profile, and science instrument design have undergone almost ten years of refinement. SIRTF development and operations activities are highly cost constrained. The cost constraints have impacted the design of the SOS in several ways. The Science Operations System has been designed to incorporate a set of efficient, easy to use tools which will make it possible for scientists to propose observation sequences in a rapid and automated manner. The use of highly automated tools for requesting observations will simplify the long range observatory scheduling process, and the short term scheduling of science observations. Pipeline data processing will be highly automated and data

Remapping of the RP15 Locus for X-Linked Cone-Rod Degeneration to Xp11.4-p21.1, and Identification of a De Novo Insertion in the RPGR Exon ORF15

PubMed Central

Mears, Alan J.; Hiriyanna, Suja; Vervoort, Raf; Yashar, Beverly; Gieser, Linn; Fahrner, Stacey; Daiger, Stephen P.; Heckenlively, John R.; Sieving, Paul A.; Wright, Alan F.; Swaroop, Anand

2000-01-01

X-linked forms of retinitis pigmentosa (XLRP) are among the most severe, because of their early onset, often leading to significant vision loss before the 4th decade. Previously, the RP15 locus was assigned to Xp22, by linkage analysis of a single pedigree with “X-linked dominant cone-rod degeneration.” After clinical reevaluation of a female in this pedigree identified her as affected, we remapped the disease to a 19.5-cM interval (DXS1219–DXS993) at Xp11.4-p21.1. This new interval overlapped both RP3 (RPGR) and COD1. Sequencing of the previously published exons of RPGR revealed no mutations, but a de novo insertion was detected in the new RPGR exon, ORF15. The identification of an RPGR mutation in a family with a severe form of cone and rod degeneration suggests that RPGR mutations may encompass a broader phenotypic spectrum than has previously been recognized in “typical” retinitis pigmentosa. PMID:10970770

NASA Customer Data and Operations System

NASA Technical Reports Server (NTRS)

Butler, Madeline J.; Stallings, William H.

1991-01-01

In addition to the currently provided NASA services such as Communications and Tracking and Data Relay Satellite System services, the NASA's Customer Data and Operations System (CDOS) will provide the following services to the user: Data Delivery Service, Data Archive Service, and CDOS Operations Management Service. This paper describes these services in detail and presents respective block diagrams. The CDOS services will support a variety of multipurpose missions simultaneously with centralized and common hardware and software data-driven systems.

49 CFR 325.37 - Location and operation of sound level measurement system; highway operations.

Code of Federal Regulations, 2011 CFR

2011-10-01

... 49 Transportation 5 2011-10-01 2011-10-01 false Location and operation of sound level measurement...; Highway Operations § 325.37 Location and operation of sound level measurement system; highway operations. (a) The microphone of a sound level measurement system that conforms to the rules in § 325.23 of this...

Operation quality assessment model for video conference system

NASA Astrophysics Data System (ADS)

Du, Bangshi; Qi, Feng; Shao, Sujie; Wang, Ying; Li, Weijian

2018-01-01

Video conference system has become an important support platform for smart grid operation and management, its operation quality is gradually concerning grid enterprise. First, the evaluation indicator system covering network, business and operation maintenance aspects was established on basis of video conference system's operation statistics. Then, the operation quality assessment model combining genetic algorithm with regularized BP neural network was proposed, which outputs operation quality level of the system within a time period and provides company manager with some optimization advice. The simulation results show that the proposed evaluation model offers the advantages of fast convergence and high prediction accuracy in contrast with regularized BP neural network, and its generalization ability is superior to LM-BP neural network and Bayesian BP neural network.

Test, Control and Monitor System (TCMS) operations plan

NASA Technical Reports Server (NTRS)

Macfarlane, C. K.; Conroy, M. P.

1993-01-01

The purpose is to provide a clear understanding of the Test, Control and Monitor System (TCMS) operating environment and to describe the method of operations for TCMS. TCMS is a complex and sophisticated checkout system focused on support of the Space Station Freedom Program (SSFP) and related activities. An understanding of the TCMS operating environment is provided and operational responsibilities are defined. NASA and the Payload Ground Operations Contractor (PGOC) will use it as a guide to manage the operation of the TCMS computer systems and associated networks and workstations. All TCMS operational functions are examined. Other plans and detailed operating procedures relating to an individual operational function are referenced within this plan. This plan augments existing Technical Support Management Directives (TSMD's), Standard Practices, and other management documentation which will be followed where applicable.

Compound haplotypes at Xp11.23 and human population growth in Eurasia.

PubMed

Alonso, S; Armour, J A L

2004-09-01

To investigate patterns of diversity and the evolutionary history of Eurasians, we have sequenced a 2.8 kb region at Xp11.23 in a sample of African and Eurasian chromosomes. This region is in a long intron of CLCN5 and is immediately flanked by a highly variable minisatellite, DXS255, and a human-specific Ta0 LINE. Compared to Africans, Eurasians showed a marked reduction in sequence diversity. The main Euro-Asiatic haplotype seems to be the ancestral haplotype for the whole sample. Coalescent simulations, including recombination and exponential growth, indicate a median length of strong linkage disequilibrium, up to approximately 9 kb for this area. The Ka/Ks ratio between the coding sequence of human CLCN5 and its mouse orthologue is much less than 1. This implies that the region sequenced is unlikely to be under the strong influence of positive selective processes on CLCN5, mutations in which have been associated with disorders such as Dent's disease. In contrast, a scenario based on a population bottleneck and exponential growth seems a more likely explanation for the reduced diversity observed in Eurasians. Coalescent analysis and linked minisatellite diversity (which reaches a gene diversity value greater than 98% in Eurasians) suggest an estimated age of origin of the Euro-Asiatic diversity compatible with a recent out-of-Africa model for colonization of Eurasia by modern Homo sapiens.

Reactor Operations Monitoring System

DOE Office of Scientific and Technical Information (OSTI.GOV)

Hart, M.M.

1989-01-01

The Reactor Operations Monitoring System (ROMS) is a VME based, parallel processor data acquisition and safety action system designed by the Equipment Engineering Section and Reactor Engineering Department of the Savannah River Site. The ROMS will be analyzing over 8 million signal samples per minute. Sixty-eight microprocessors are used in the ROMS in order to achieve a real-time data analysis. The ROMS is composed of multiple computer subsystems. Four redundant computer subsystems monitor 600 temperatures with 2400 thermocouples. Two computer subsystems share the monitoring of 600 reactor coolant flows. Additional computer subsystems are dedicated to monitoring 400 signals from assortedmore » process sensors. Data from these computer subsystems are transferred to two redundant process display computer subsystems which present process information to reactor operators and to reactor control computers. The ROMS is also designed to carry out safety functions based on its analysis of process data. The safety functions include initiating a reactor scram (shutdown), the injection of neutron poison, and the loadshed of selected equipment. A complete development Reactor Operations Monitoring System has been built. It is located in the Program Development Center at the Savannah River Site and is currently being used by the Reactor Engineering Department in software development. The Equipment Engineering Section is designing and fabricating the process interface hardware. Upon proof of hardware and design concept, orders will be placed for the final five systems located in the three reactor areas, the reactor training simulator, and the hardware maintenance center.« less

Evaluation of Microcomputer-Based Operation and Maintenance Management Systems for Army Water/Wastewater Treatment Plant Operation.

DTIC Science & Technology

1986-07-01

COMPUTER-AIDED OPERATION MANAGEMENT SYSTEM ................. 29 Functions of an Off-Line Computer-Aided Operation Management System Applications of...System Comparisons 85 DISTRIBUTION 5V J. • 0. FIGURES Number Page 1 Hardware Components 21 2 Basic Functions of a Computer-Aided Operation Management System...Plant Visits 26 4 Computer-Aided Operation Management Systems Reviewed for Analysis of Basic Functions 29 5 Progress of Software System Installation and

Mission operations concepts for Earth Observing System (EOS)

NASA Technical Reports Server (NTRS)

Kelly, Angelita C.; Taylor, Thomas D.; Hawkins, Frederick J.

1991-01-01

Mission operation concepts are described which are being used to evaluate and influence space and ground system designs and architectures with the goal of achieving successful, efficient, and cost-effective Earth Observing System (EOS) operations. Emphasis is given to the general characteristics and concepts developed for the EOS Space Measurement System, which uses a new series of polar-orbiting observatories. Data rates are given for various instruments. Some of the operations concepts which require a total system view are also examined, including command operations, data processing, data accountability, data archival, prelaunch testing and readiness, launch, performance monitoring and assessment, contingency operations, flight software maintenance, and security.

Fine-needle aspiration of a Xp11.2 translocation/TFE3 fusion renal cell carcinoma metastatic to the lung: report of a case and review of the literature.

PubMed

Schinstine, Malcolm; Filie, Armando C; Torres-Cabala, Carlos; Abati, Andrea; Linehan, W Marston; Merino, Maria

2006-11-01

A 57-yr-old woman presented to the National Cancer Institute (NCI) with a history of nephrectomy for a clear cell renal cell carcinoma (RCC), Fuhrman grade 3 of 4 diagnosed 1 yr prior to admission to the NCI. A CT scan done upon admission revealed multiple bilateral lung masses. A CT-guided fine-needle aspiration (FNA) of one of the lung masses revealed a cellular specimen composed primarily of follicular structures surrounding dense hyalinized central cores. The cells in the follicular structures displayed bland nuclei and had granular to vacuolated cytoplasm. Papillary structures were also appreciated. Immunocytochemical studies showed tumor cells that were strongly vimentin and TFE3 positive. Focal staining for AE1/AE3 and CD10 was observed, as was negative staining for EMA. A surgical biopsy specimen reflected the FNA findings and demonstrated a similar immunoprofile. These findings correspond to the recently described Xp11.2 translocation/TFE3 fusion renal cell carcinoma. To our knowledge, this is the first report describing the cytologic features of an Xp11.2 translocation/TFE3 fusion RCC. (C) 2006 Wiley-Liss, Inc.

PC-based automation system streamlines operations

DOE Office of Scientific and Technical Information (OSTI.GOV)

Bowman, J.

1995-10-01

The continued emergence of PC-based automation systems in the modern compressor station is driving the need for personnel who have the special skills need to support them. However, the dilemma is that operating budget restraints limit the overall number of people available to operate and maintain compressor stations. An ideal solution is to deploy automation systems which can be easily understood and supported by existing compressor station personnel. This paper reviews such a system developed by Waukesha-Pearce Industries, Inc.

Planning Systems for Distributed Operations

NASA Technical Reports Server (NTRS)

Maxwell, Theresa G.

2002-01-01

This viewgraph representation presents an overview of the mission planning process involving distributed operations (such as the International Space Station (ISS)) and the computer hardware and software systems needed to support such an effort. Topics considered include: evolution of distributed planning systems, ISS distributed planning, the Payload Planning System (PPS), future developments in distributed planning systems, Request Oriented Scheduling Engine (ROSE) and Next Generation distributed planning systems.

Selecting a Cable System Operator.

ERIC Educational Resources Information Center

Cable Television Information Center, Washington, DC.

Intended to assist franchising authorities with the process of selecting a cable television system operator from franchise applicants, this document provides a framework for analysis of individual applications. Section 1 deals with various methods which can be used to select an operator. The next section covers the application form, the vehicle a…

Interactive orbital proximity operations planning system

NASA Technical Reports Server (NTRS)

Grunwald, Arthur J.; Ellis, Stephen R.

1990-01-01

An interactive graphical planning system for on-site planning of proximity operations in the congested multispacecraft environment about the space station is presented. The system shows the astronaut a bird's eye perspective of the space station, the orbital plane, and the co-orbiting spacecraft. The system operates in two operational modes: (1) a viewpoint mode, in which the astronaut is able to move the viewpoint around in the orbital plane to range in on areas of interest; and (2) a trajectory design mode, in which the trajectory is planned. Trajectory design involves the composition of a set of waypoints which result in a fuel-optimal trajectory which satisfies all operational constraints, such as departure and arrival constraints, plume impingement constraints, and structural constraints. The main purpose of the system is to present the trajectory and the constraints in an easily interpretable graphical format. Through a graphical interactive process, the trajectory waypoints are edited until all operational constraints are satisfied. A series of experiments was conducted to evaluate the system. Eight airline pilots with no prior background in orbital mechanics participated in the experiments. Subject training included a stand-alone training session of about 6 hours duration, in which the subjects became familiar with orbital mechanics concepts and performed a series of exercises to familiarize themselves with the control and display features of the system. They then carried out a series of production runs in which 90 different trajectory design situations were randomly addressed. The purpose of these experiments was to investigate how the planning time, planning efforts, and fuel expenditures were affected by the planning difficulty. Some results of these experiments are presented.

PSF/SFPQ is a very common gene fusion partner in TFE3 rearrangement-associated perivascular epithelioid cell tumors (PEComas) and melanotic Xp11 translocation renal cancers: clinicopathologic, immunohistochemical, and molecular characteristics suggesting classification as a distinct entity.

PubMed

Rao, Qiu; Shen, Qin; Xia, Qiu-yuan; Wang, Zi-yu; Liu, Biao; Shi, Shan-shan; Shi, Qun-li; Yin, Hong-lin; Wu, Bo; Ye, Sheng-bing; Li, Li; Chen, Jie-Yu; Pan, Min-hong; Li, Qing; Li, Rui; Wang, Xuan; Zhang, Ru-song; Yu, Bo; Ma, Heng-hui; Lu, Zhen-feng; Zhou, Xiao-jun

2015-09-01

An increasing number of TFE3 rearrangement-associated tumors, such as TFE3 rearrangement-associated perivascular epithelioid cell tumors (PEComas), melanotic Xp11 translocation renal cancers, and melanotic Xp11 neoplasms, have recently been reported. We examined 12 such cases, including 5 TFE3 rearrangement-associated PEComas located in the pancreas, cervix, or pelvis and 7 melanotic Xp11 translocation renal cancers, using clinicopathologic, immunohistochemical, and molecular analyses. All the tumors shared a similar morphology, including a purely nested or sheet-like architecture separated by a delicate vascular network, purely epithelioid cells displaying a clear or granular eosinophilic cytoplasm, a lack of papillary structures and spindle cell or fat components, uniform round or oval nuclei containing small visible nucleoli, and, in most cases (11/12), melanin pigmentation. The levels of mitotic activity and necrosis varied. All 12 cases displayed moderately (2+) or strongly (3+) positive immunoreactivity for TFE3 and cathepsin K. One case labeled focally for HMB45 and Melan-A, whereas the others typically labeled moderately (2+) or strongly (3+) for 1 of these markers. None of the cases were immunoreactive for smooth muscle actin, desmin, CKpan, S100, or PAX8. PSF-TFE3 fusion genes were confirmed by reverse transcription polymerase chain reaction in cases (7/7) in which a novel PSF-TFE3 fusion point was identified. All of the cases displayed TFE3 rearrangement associated with Xp11 translocation. Furthermore, we developed a PSF-TFE3 fusion fluorescence in situ hybridization assay for the detection of the PSF-TFE3 fusion gene and detected it in all 12 cases. Clinical follow-up data were available for 7 patients. Three patients died, and 2 patients (cases 1 and 3) remained alive with no evidence of disease after initial resection. Case 2 experienced recurrence and remained alive with disease. Case 5, a recent case, remained alive with extensive abdominal cavity

Measurement and analysis of operating system fault tolerance

NASA Technical Reports Server (NTRS)

Lee, I.; Tang, D.; Iyer, R. K.

1992-01-01

This paper demonstrates a methodology to model and evaluate the fault tolerance characteristics of operational software. The methodology is illustrated through case studies on three different operating systems: the Tandem GUARDIAN fault-tolerant system, the VAX/VMS distributed system, and the IBM/MVS system. Measurements are made on these systems for substantial periods to collect software error and recovery data. In addition to investigating basic dependability characteristics such as major software problems and error distributions, we develop two levels of models to describe error and recovery processes inside an operating system and on multiple instances of an operating system running in a distributed environment. Based on the models, reward analysis is conducted to evaluate the loss of service due to software errors and the effect of the fault-tolerance techniques implemented in the systems. Software error correlation in multicomputer systems is also investigated.

49 CFR 234.257 - Warning system operation.

Code of Federal Regulations, 2011 CFR

2011-10-01

... 49 Transportation 4 2011-10-01 2011-10-01 false Warning system operation. 234.257 Section 234.257..., Inspection, and Testing Inspections and Tests § 234.257 Warning system operation. (a) Each highway-rail crossing warning system shall be tested to determine that it functions as intended when it is placed in...

49 CFR 234.257 - Warning system operation.

Code of Federal Regulations, 2010 CFR

2010-10-01

... 49 Transportation 4 2010-10-01 2010-10-01 false Warning system operation. 234.257 Section 234.257..., Inspection, and Testing Inspections and Tests § 234.257 Warning system operation. (a) Each highway-rail crossing warning system shall be tested to determine that it functions as intended when it is placed in...

A Brief Investigation of the Hydrodynamic Characteristics of a 1/13.33-Scale Powered Dynamic Model of a Preliminary Design of the Martin XP6M-1 Flying Boat, TED No. NACA DE-385

NASA Technical Reports Server (NTRS)

Blanchard, Ulysse J.

1953-01-01

The hydrodynamic characteristics of a preliminary design of the Martin XP6M-1 flying boat have been determined. Longitudinal stability during take-off and landing, resistance of the complete model, and behavior during taxiing and landing in rough water are presented.

The Triangle of the Space Launch System Operations

NASA Astrophysics Data System (ADS)

Fayolle, Eric

2010-09-01

Firemen know it as “fire triangle”, mathematicians know it as “golden triangle”, sailormen know it as “Bermuda triangle”, politicians know it as “Weimar triangle”… This article aims to present a new aspect of that shape geometry in the space launch system world: “the triangle of the space launch system operations”. This triangle is composed of these three following topics, which have to be taken into account for any space launch system operation processing: design, safety and operational use. Design performance is of course taking into account since the early preliminary phase of a system development. This design performance is matured all along the development phases, thanks to consecutives iterations in order to respect the financial and timing constraints imposed to the development of the system. This process leads to a detailed and precise design to assess the required performance. Then, the operational use phase brings its batch of constraints during the use of the system. This phase is conducted by specific procedures for each operation. Each procedure has sequences for each sub-system, which have to be conducted in a very precise chronological way. These procedures can be processed by automatic way or manual way, with the necessity or not of the implication of operators, and in a determined environment. Safeguard aims to verify the respect of the specific constraints imposed to guarantee the safety of persons and property, the protection of public health and the environment. Safeguard has to be taken into account above the operational constraints of any space operation, without forgetting the highest safety level for the operators of the space operation, and of course without damaging the facilities or without disturbing the external environment. All space operations are the result of a “win-win” compromise between these three topics. Contrary to the fire triangle where one of the topics has to be suppressed in order to avoid the

Automated Operations Development for Advanced Exploration Systems

NASA Technical Reports Server (NTRS)

Haddock, Angie T.; Stetson, Howard

2012-01-01

Automated space operations command and control software development and its implementation must be an integral part of the vehicle design effort. The software design must encompass autonomous fault detection, isolation, recovery capabilities and also provide "single button" intelligent functions for the crew. Development, operations and safety approval experience with the Timeliner system onboard the International Space Station (ISS), which provided autonomous monitoring with response and single command functionality of payload systems, can be built upon for future automated operations as the ISS Payload effort was the first and only autonomous command and control system to be in continuous execution (6 years), 24 hours a day, 7 days a week within a crewed spacecraft environment. Utilizing proven capabilities from the ISS Higher Active Logic (HAL) System, along with the execution component design from within the HAL 9000 Space Operating System, this design paper will detail the initial HAL System software architecture and interfaces as applied to NASA's Habitat Demonstration Unit (HDU) in support of the Advanced Exploration Systems, Autonomous Mission Operations project. The development and implementation of integrated simulators within this development effort will also be detailed and is the first step in verifying the HAL 9000 Integrated Test-Bed Component [2] designs effectiveness. This design paper will conclude with a summary of the current development status and future development goals as it pertains to automated command and control for the HDU.

Automated Instructional Management Systems (AIMS) Version III, Operator's Guide.

ERIC Educational Resources Information Center

New York Inst. of Tech., Old Westbury.

This manual gives the instructions necessary to understand and operate the Automated Instructional Management System (AIMS), utilizing IBM System 360, Model 30/Release 20 Disk Operating System, and the OpScan 100 System Reader and Tape Unit. It covers the AIMS III system initialization, system and operational input, requirements, master response…

Terminal-area STOL operating systems experiments program

NASA Technical Reports Server (NTRS)

Smith, D. W.; Watson, D.; Christensen, J. V.

1972-01-01

A system study to determine the application of short takeoff aircraft for a high speed, short haul air transportation service was conducted. The study focused on developing information which will aid in choosing system concepts, design criteria, operating procedures, landing guidance systems, air traffic control systems, and airborne avionics and flight control systems. A terminal area STOL operating system experiments program was developed. The objectives, program approach, program schedule, typical experiments, research facilities to be used, and program status are discussed.

[Controlling systems for operating room managers].

PubMed

Schüpfer, G; Bauer, M; Scherzinger, B; Schleppers, A

2005-08-01

Management means developing, shaping and controlling of complex, productive and social systems. Therefore, operating room managers also need to develop basic skills in financial and managerial accounting as a basis for operative and strategic controlling which is an essential part of their work. A good measurement system should include financial and strategic concepts for market position, innovation performance, productivity, attractiveness, liquidity/cash flow and profitability. Since hospitals need to implement a strategy to reach their business objectives, the performance measurement system has to be individually adapted to the strategy of the hospital. In this respect the navigation system developed by Gälweiler is compared to the "balanced score card" system of Kaplan and Norton.

Pyrolaser Operating System

NASA Technical Reports Server (NTRS)

Roberts, Floyd E., III

1994-01-01

Software provides for control and acquisition of data from optical pyrometer. There are six individual programs in PYROLASER package. Provides quick and easy way to set up, control, and program standard Pyrolaser. Temperature and emisivity measurements either collected as if Pyrolaser in manual operating mode or displayed on real-time strip charts and stored in standard spreadsheet format for posttest analysis. Shell supplied to allow macros, which are test-specific, added to system easily. Written using Labview software for use on Macintosh-series computers running System 6.0.3 or later, Sun Sparc-series computers running Open-Windows 3.0 or MIT's X Window System (X11R4 or X11R5), and IBM PC or compatible computers running Microsoft Windows 3.1 or later.

Virtualization of Legacy Instrumentation Control Computers for Improved Reliability, Operational Life, and Management.

PubMed

Katz, Jonathan E

2017-01-01

Laboratories tend to be amenable environments for long-term reliable operation of scientific measurement equipment. Indeed, it is not uncommon to find equipment 5, 10, or even 20+ years old still being routinely used in labs. Unfortunately, the Achilles heel for many of these devices is the control/data acquisition computer. Often these computers run older operating systems (e.g., Windows XP) and, while they might only use standard network, USB or serial ports, they require proprietary software to be installed. Even if the original installation disks can be found, it is a burdensome process to reinstall and is fraught with "gotchas" that can derail the process-lost license keys, incompatible hardware, forgotten configuration settings, etc. If you have running legacy instrumentation, the computer is the ticking time bomb waiting to put a halt to your operation.In this chapter, I describe how to virtualize your currently running control computer. This virtualized computer "image" is easy to maintain, easy to back up and easy to redeploy. I have used this multiple times in my own lab to greatly improve the robustness of my legacy devices.After completing the steps in this chapter, you will have your original control computer as well as a virtual instance of that computer with all the software installed ready to control your hardware should your original computer ever be decommissioned.

A Framework for Adaptable Operating and Runtime Systems

DOE Office of Scientific and Technical Information (OSTI.GOV)

Sterling, Thomas

The emergence of new classes of HPC systems where performance improvement is enabled by Moore’s Law for technology is manifest through multi-core-based architectures including specialized GPU structures. Operating systems were originally designed for control of uniprocessor systems. By the 1980s multiprogramming, virtual memory, and network interconnection were integral services incorporated as part of most modern computers. HPC operating systems were primarily derivatives of the Unix model with Linux dominating the Top-500 list. The use of Linux for commodity clusters was first pioneered by the NASA Beowulf Project. However, the rapid increase in number of cores to achieve performance gain throughmore » technology advances has exposed the limitations of POSIX general-purpose operating systems in scaling and efficiency. This project was undertaken through the leadership of Sandia National Laboratories and in partnership of the University of New Mexico to investigate the alternative of composable lightweight kernels on scalable HPC architectures to achieve superior performance for a wide range of applications. The use of composable operating systems is intended to provide a minimalist set of services specifically required by a given application to preclude overheads and operational uncertainties (“OS noise”) that have been demonstrated to degrade efficiency and operational consistency. This project was undertaken as an exploration to investigate possible strategies and methods for composable lightweight kernel operating systems towards support for extreme scale systems.« less

Operating health analysis of electric power systems

NASA Astrophysics Data System (ADS)

Fotuhi-Firuzabad, Mahmud

The required level of operating reserve to be maintained by an electric power system can be determined using both deterministic and probabilistic techniques. Despite the obvious disadvantages of deterministic approaches there is still considerable reluctance to apply probabilistic techniques due to the difficulty of interpreting a single numerical risk index and the lack of sufficient information provided by a single index. A practical way to overcome difficulties is to embed deterministic considerations in the probabilistic indices in order to monitor the system well-being. The system well-being can be designated as healthy, marginal and at risk. The concept of system well-being is examined and extended in this thesis to cover the overall area of operating reserve assessment. Operating reserve evaluation involves the two distinctly different aspects of unit commitment and the dispatch of the committed units. Unit commitment health analysis involves the determination of which unit should be committed to satisfy the operating criteria. The concepts developed for unit commitment health, margin and risk are extended in this thesis to evaluate the response well-being of a generating system. A procedure is presented to determine the optimum dispatch of the committed units to satisfy the response criteria. The impact on the response wellbeing being of variations in the margin time, required regulating margin and load forecast uncertainty are illustrated. The effects on the response well-being of rapid start units, interruptible loads and postponable outages are also illustrated. System well-being is, in general, greatly improved by interconnection with other power systems. The well-being concepts are extended to evaluate the spinning reserve requirements in interconnected systems. The interconnected system unit commitment problem is decomposed into two subproblems in which unit scheduling is performed in each isolated system followed by interconnected system evaluation

Multitasking Operating Systems for the IBM PC.

ERIC Educational Resources Information Center

Owen, G. Scott

1985-01-01

The ability of a microcomputer to execute several programs at the same time is called "multitasking." The nature and use of one multitasking operating system Concurrent PC-DOS from Digital Research (the developers of the CP/M operating system) are discussed. (JN)

[Development of medical emergency response system for accidents due to chemicals in Chongqing municipality].

PubMed

Ning, Xu; Dong, Zhao-jun; Mu, Ling; Zhai, Jian-cai

2006-12-01

To plan and develop a Chongqing chemical accident rescue command system. Based on the modes of leakage and diffusion of various poisonous gases and chemicals, different modes of injuries produced, and their appropriate rescue and treatments, also taking the following factors such as the condition of storage of chemicals, meteorological and geographic conditions, medical institutions and equipment, and their rescuing capacity into consideration, a plan was drafted to establish the rescue system. Real-time simulation technology, data analysis, evaluation technology and database technology were employed in the planning. Using Visual Studio 6.0 as the software development platform, this project aimed to design the software of an emergency command system for chemical accidents in Chongqing which could be operated with the Windows 2000/XP operating system. This system provided a dynamic scope of the endangered area, casualty number estimates, and recommendation of measures and a rescue plan for various chemical accidents. Furthermore, the system helped retrieve comprehensive information regarding the physical and chemical characteristics of more than 4 200 dangerous poisonous chemicals and their appropriate treatment modalities. This system is easy to operate with a friendly interface, functions rapidly and can provide real-time analysis with comparatively precise results. This system could satisfy the requirements of executing the command and the rescue of a chemical accident with good prospects of application.

Automated Operations Development for Advanced Exploration Systems

NASA Technical Reports Server (NTRS)

Haddock, Angie; Stetson, Howard K.

2012-01-01

Automated space operations command and control software development and its implementation must be an integral part of the vehicle design effort. The software design must encompass autonomous fault detection, isolation, recovery capabilities and also provide single button intelligent functions for the crew. Development, operations and safety approval experience with the Timeliner system on-board the International Space Station (ISS), which provided autonomous monitoring with response and single command functionality of payload systems, can be built upon for future automated operations as the ISS Payload effort was the first and only autonomous command and control system to be in continuous execution (6 years), 24 hours a day, 7 days a week within a crewed spacecraft environment. Utilizing proven capabilities from the ISS Higher Active Logic (HAL) System [1] , along with the execution component design from within the HAL 9000 Space Operating System [2] , this design paper will detail the initial HAL System software architecture and interfaces as applied to NASA s Habitat Demonstration Unit (HDU) in support of the Advanced Exploration Systems, Autonomous Mission Operations project. The development and implementation of integrated simulators within this development effort will also be detailed and is the first step in verifying the HAL 9000 Integrated Test-Bed Component [2] designs effectiveness. This design paper will conclude with a summary of the current development status and future development goals as it pertains to automated command and control for the HDU.

A Distributed Operating System for BMD Applications.

DTIC Science & Technology

1982-01-01

Defense) applications executing on distributed hardware with local and shared memories. The objective was to develop real - time operating system functions...make the Basic Real - Time Operating System , and the set of new EPL language primitives that provide BMD application processes with efficient mechanisms

Intelligent transportation systems field operational test cross-cutting study : commercial vehicle operations -- roadside

DOT National Transportation Integrated Search

1998-11-01

Commercial Vehicle Operations - Roadside report discusses the findings and conclusions exclusively from Field Operational Tests (FOTs) of roadside Intelligent Transportation Systems (ITS) for Commercial Vehicle Operations (CVO). The FOTs considered i...

Operation of staged membrane oxidation reactor systems

DOEpatents

Repasky, John Michael

2012-10-16

A method of operating a multi-stage ion transport membrane oxidation system. The method comprises providing a multi-stage ion transport membrane oxidation system with at least a first membrane oxidation stage and a second membrane oxidation stage, operating the ion transport membrane oxidation system at operating conditions including a characteristic temperature of the first membrane oxidation stage and a characteristic temperature of the second membrane oxidation stage; and controlling the production capacity and/or the product quality by changing the characteristic temperature of the first membrane oxidation stage and/or changing the characteristic temperature of the second membrane oxidation stage.

Spatial operator algebra framework for multibody system dynamics

NASA Technical Reports Server (NTRS)

Rodriguez, G.; Jain, Abhinandan; Kreutz, K.

1989-01-01

The Spatial Operator Algebra framework for the dynamics of general multibody systems is described. The use of a spatial operator-based methodology permits the formulation of the dynamical equations of motion of multibody systems in a concise and systematic way. The dynamical equations of progressively more complex grid multibody systems are developed in an evolutionary manner beginning with a serial chain system, followed by a tree topology system and finally, systems with arbitrary closed loops. Operator factorizations and identities are used to develop novel recursive algorithms for the forward dynamics of systems with closed loops. Extensions required to deal with flexible elements are also discussed.

A Proposed Operational Concept for the Defense Communications Operations Support System.

DTIC Science & Technology

1986-01-01

Artificial Intelligence AMA Automatic Message Accounting AMIE AUTODIN Management Index System AMPE Automated Message Processing Exchange ANCS AUTOVON Network...Support IMPRESS Inpact/Restoral System INFORM Information Retrieval System 1OC Initial Operational Capability IRU Intellegent Remote Unit I-S/A AMPE

14 CFR 25.961 - Fuel system hot weather operation.

Code of Federal Regulations, 2010 CFR

2010-01-01

... AIRCRAFT AIRWORTHINESS STANDARDS: TRANSPORT CATEGORY AIRPLANES Powerplant Fuel System § 25.961 Fuel system hot weather operation. (a) The fuel system must perform satisfactorily in hot weather operation. This... 14 Aeronautics and Space 1 2010-01-01 2010-01-01 false Fuel system hot weather operation. 25.961...

14 CFR 25.961 - Fuel system hot weather operation.

Code of Federal Regulations, 2013 CFR

2013-01-01

... AIRCRAFT AIRWORTHINESS STANDARDS: TRANSPORT CATEGORY AIRPLANES Powerplant Fuel System § 25.961 Fuel system hot weather operation. (a) The fuel system must perform satisfactorily in hot weather operation. This... 14 Aeronautics and Space 1 2013-01-01 2013-01-01 false Fuel system hot weather operation. 25.961...

14 CFR 25.961 - Fuel system hot weather operation.

Code of Federal Regulations, 2012 CFR

2012-01-01

... AIRCRAFT AIRWORTHINESS STANDARDS: TRANSPORT CATEGORY AIRPLANES Powerplant Fuel System § 25.961 Fuel system hot weather operation. (a) The fuel system must perform satisfactorily in hot weather operation. This... 14 Aeronautics and Space 1 2012-01-01 2012-01-01 false Fuel system hot weather operation. 25.961...

14 CFR 25.961 - Fuel system hot weather operation.

Code of Federal Regulations, 2011 CFR

2011-01-01

... AIRCRAFT AIRWORTHINESS STANDARDS: TRANSPORT CATEGORY AIRPLANES Powerplant Fuel System § 25.961 Fuel system hot weather operation. (a) The fuel system must perform satisfactorily in hot weather operation. This... 14 Aeronautics and Space 1 2011-01-01 2011-01-01 false Fuel system hot weather operation. 25.961...

14 CFR 25.961 - Fuel system hot weather operation.

Code of Federal Regulations, 2014 CFR

2014-01-01

... AIRCRAFT AIRWORTHINESS STANDARDS: TRANSPORT CATEGORY AIRPLANES Powerplant Fuel System § 25.961 Fuel system hot weather operation. (a) The fuel system must perform satisfactorily in hot weather operation. This... 14 Aeronautics and Space 1 2014-01-01 2014-01-01 false Fuel system hot weather operation. 25.961...

Operating system for a real-time multiprocessor propulsion system simulator

NASA Technical Reports Server (NTRS)

Cole, G. L.

1984-01-01

The success of the Real Time Multiprocessor Operating System (RTMPOS) in the development and evaluation of experimental hardware and software systems for real time interactive simulation of air breathing propulsion systems was evaluated. The Real Time Multiprocessor Operating System (RTMPOS) provides the user with a versatile, interactive means for loading, running, debugging and obtaining results from a multiprocessor based simulator. A front end processor (FEP) serves as the simulator controller and interface between the user and the simulator. These functions are facilitated by the RTMPOS which resides on the FEP. The RTMPOS acts in conjunction with the FEP's manufacturer supplied disk operating system that provides typical utilities like an assembler, linkage editor, text editor, file handling services, etc. Once a simulation is formulated, the RTMPOS provides for engineering level, run time operations such as loading, modifying and specifying computation flow of programs, simulator mode control, data handling and run time monitoring. Run time monitoring is a powerful feature of RTMPOS that allows the user to record all actions taken during a simulation session and to receive advisories from the simulator via the FEP. The RTMPOS is programmed mainly in PASCAL along with some assembly language routines. The RTMPOS software is easily modified to be applicable to hardware from different manufacturers.

ASTER system operating achievement for 15 years on orbit

NASA Astrophysics Data System (ADS)

Inada, Hitomi; Ito, Yoshiyuki; Kikuchi, Masakuni; Sakuma, Fumihiro; Tatsumi, Kenji; Akagi, Shigeki; Ono, Hidehiko

2015-10-01

ASTER (Advanced Spaceborne Thermal Emission and Reflection Radiometer) System is operating more than 15 years since launched on board of NASA's Terra spacecraft in December 1999. ASTER System is composed of 3 radiometers (VNIR (Visible and Near Infrared Radiometer), SWIR (Short-Wave Infrared Radiometer), and TIR (Thermal Infrared Radiometer)), CSP (Common Signal Processor) and MSP (Master Power Supply). This paper describes the ASTER System operating history and the achievement of ASTER System long term operation since the initial checkout operation, the normal operation, and the continuous operation. Through the 15 years operation, ASTER system had totally checked the all subsystems (MPS, VNIR, TIR, SWIR, and CSP) health and safety check using telemetry data trend evaluation, and executed the necessary action. The watch items are monitored as the life control items. The pointing mechanics for VNIR, SWIR and TIR, and the cooler for SWIR and TIR are all operating with any problem for over 15 years. In 2003, ASTER was successfully operated for the lunar calibration. As the future plan, ASTER team is proposing the 2nd lunar calibration before the end of mission.

Interactive orbital proximity operations planning system

NASA Technical Reports Server (NTRS)

Grunwald, Arthur J.; Ellis, Stephen R.

1988-01-01

An interactive graphical proximity operations planning system was developed, which allows on-site design of efficient, complex, multiburn maneuvers in a dynamic multispacecraft environment. Maneuvering takes place in and out of the orbital plane. The difficulty in planning such missions results from the unusual and counterintuitive character of orbital dynamics and complex time-varying operational constraints. This difficulty is greatly overcome by visualizing the relative trajectories and the relevant constraints in an easily interpretable graphical format, which provides the operator with immediate feedback on design actions. The display shows a perspective bird's-eye view of a Space Station and co-orbiting spacecraft on the background of the Station's orbital plane. The operator has control over the two modes of operation: a viewing system mode, which enables the exporation of the spatial situation about the Space Station and thus the ability to choose and zoom in on areas of interest; and a trajectory design mode, which allows the interactive editing of a series of way points and maneuvering burns to obtain a trajectory that complies with all operational constraints. A first version of this display was completed. An experimental program is planned in which operators will carry out a series of design missions which vary in complexity and constraints.

Operationally Efficient Propulsion System Study (OEPSS) data book. Volume 2: Ground operations problems

NASA Technical Reports Server (NTRS)

Waldrop, Glen S.

1990-01-01

Operations problems and cost drivers were identified for current propulsion systems and design and technology approaches were identified to increase the operational efficiency and to reduce operations costs for future propulsion systems. To provide readily usable data for the ALS program, the results of the OEPSS study were organized into a series of OEPSS Data Books. This volume presents a detailed description of 25 major problems encountered during launch processing of current expendable and reusable launch vehicles. A concise description of each problem and its operational impact on launch processing is presented, along with potential solutions and technology recommendation.

Operations Monitoring Assistant System Design

DTIC Science & Technology

1986-07-01

Logic. Artificial Inteligence 25(1)::75-94. January.18. 41 -Nils J. Nilsson. Problem-Solving Methods In Artificli Intelligence. .klcG raw-Hill B3ook...operations monitoring assistant (OMA) system is designed that combines operations research, artificial intelligence, and human reasoning techniques and...KnowledgeCraft (from Carnegie Group), and 5.1 (from Teknowledze). These tools incorporate the best methods of applied artificial intelligence, and

Expert system verification concerns in an operations environment

NASA Technical Reports Server (NTRS)

Goodwin, Mary Ann; Robertson, Charles C.

1987-01-01

The Space Shuttle community is currently developing a number of knowledge-based tools, primarily expert systems, to support Space Shuttle operations. It is proposed that anticipating and responding to the requirements of the operations environment will contribute to a rapid and smooth transition of expert systems from development to operations, and that the requirements for verification are critical to this transition. The paper identifies the requirements of expert systems to be used for flight planning and support and compares them to those of existing procedural software used for flight planning and support. It then explores software engineering concepts and methodology that can be used to satisfy these requirements, to aid the transition from development to operations and to support the operations environment during the lifetime of expert systems. Many of these are similar to those used for procedural hardware.

Alpha: A real-time decentralized operating system for mission-oriented system integration and operation

NASA Technical Reports Server (NTRS)

Jensen, E. Douglas

1988-01-01

Alpha is a new kind of operating system that is unique in two highly significant ways. First, it is decentralized transparently providing reliable resource management across physically dispersed nodes, so that distributed applications programming can be done largely as though it were centralized. And second, it provides comprehensive, high technology support for real-time system integration and operation, an application area which consists predominately of aperiodic activities having critical time constraints such as deadlines. Alpha is extremely adaptable so that it can be easily optimized for a wide range of problem-specific functionality, performance, and cost. Alpha is the first systems effort of the Archons Project, and the prototype was created at Carnegie-Mellon University directly on modified Sun multiprocessor workstation hardware. It has been demonstrated with a real-time C(sup 2) application. Continuing research is leading to a series of enhanced follow-ons to Alpha; these are portable but initially hosted on Concurrent's MASSCOMP line of multiprocessor products.

47 CFR 73.1350 - Transmission system operation.

Code of Federal Regulations, 2010 CFR

2010-10-01

... interference. (e) If a broadcast station is operating in a manner that poses a threat to life or property or... 47 Telecommunication 4 2010-10-01 2010-10-01 false Transmission system operation. 73.1350 Section... RADIO BROADCAST SERVICES Rules Applicable to All Broadcast Stations § 73.1350 Transmission system...

The structure of the clouds distributed operating system

NASA Technical Reports Server (NTRS)

Dasgupta, Partha; Leblanc, Richard J., Jr.

1989-01-01

A novel system architecture, based on the object model, is the central structuring concept used in the Clouds distributed operating system. This architecture makes Clouds attractive over a wide class of machines and environments. Clouds is a native operating system, designed and implemented at Georgia Tech. and runs on a set of generated purpose computers connected via a local area network. The system architecture of Clouds is composed of a system-wide global set of persistent (long-lived) virtual address spaces, called objects that contain persistent data and code. The object concept is implemented at the operating system level, thus presenting a single level storage view to the user. Lightweight treads carry computational activity through the code stored in the objects. The persistent objects and threads gives rise to a programming environment composed of shared permanent memory, dispensing with the need for hardware-derived concepts such as the file systems and message systems. Though the hardware may be distributed and may have disks and networks, the Clouds provides the applications with a logically centralized system, based on a shared, structured, single level store. The current design of Clouds uses a minimalist philosophy with respect to both the kernel and the operating system. That is, the kernel and the operating system support a bare minimum of functionality. Clouds also adheres to the concept of separation of policy and mechanism. Most low-level operating system services are implemented above the kernel and most high level services are implemented at the user level. From the measured performance of using the kernel mechanisms, we are able to demonstrate that efficient implementations are feasible for the object model on commercially available hardware. Clouds provides a rich environment for conducting research in distributed systems. Some of the topics addressed in this paper include distributed programming environments, consistency of persistent data

Electronic Performance Support for Operational Systems: A Case Study of the Link Monitor and Control Operator Assistant

NASA Technical Reports Server (NTRS)

Hill, Randall W., Jr.; Cooper, Lynne P.

1993-01-01

For complex operational systems, help needs to come from the inside out. It is often not realistic to call a help desk for problems that need immediate attention, especially for tasks that put a heavy cognitive load on the system operator. This session addresses the issues associated with providing electronic performance support for operational systems, including situations where the system is already fielded and can only change through evolution rather than revolution. We present a case study based on our experiences in developing the Link Monitor and Control Operator Assistant for NASA's Deep Space Network (DSN). The goals of the Operator Assistant are to improve the operability of the system and increase the efficiency of mission operations.

49 CFR 234.205 - Operating characteristics of warning system apparatus.

Code of Federal Regulations, 2014 CFR

2014-10-01

... 49 Transportation 4 2014-10-01 2014-10-01 false Operating characteristics of warning system... Maintenance Standards § 234.205 Operating characteristics of warning system apparatus. Operating... system shall be maintained in accordance with the limits within which the system is designed to operate. ...

49 CFR 234.205 - Operating characteristics of warning system apparatus.

Code of Federal Regulations, 2012 CFR

2012-10-01

... 49 Transportation 4 2012-10-01 2012-10-01 false Operating characteristics of warning system... Maintenance Standards § 234.205 Operating characteristics of warning system apparatus. Operating... system shall be maintained in accordance with the limits within which the system is designed to operate. ...

49 CFR 234.205 - Operating characteristics of warning system apparatus.

Code of Federal Regulations, 2013 CFR

2013-10-01

... 49 Transportation 4 2013-10-01 2013-10-01 false Operating characteristics of warning system... Maintenance Standards § 234.205 Operating characteristics of warning system apparatus. Operating... system shall be maintained in accordance with the limits within which the system is designed to operate. ...

Surface Operations Systems Improve Airport Efficiency

NASA Technical Reports Server (NTRS)

2009-01-01

With Small Business Innovation Research (SBIR) contracts from Ames Research Center, Mosaic ATM of Leesburg, Virginia created software to analyze surface operations at airports. Surface surveillance systems, which report locations every second for thousands of air and ground vehicles, generate massive amounts of data, making gathering and analyzing this information difficult. Mosaic?s Surface Operations Data Analysis and Adaptation (SODAA) tool is an off-line support tool that can analyze how well the airport surface operation is working and can help redesign procedures to improve operations. SODAA helps researchers pinpoint trends and correlations in vast amounts of recorded airport operations data.

Systemic Operational Design: Improving Operational Planning for the Netherlands Armed Forces

DTIC Science & Technology

2006-05-25

This methodology is called Soft Systems Methodology . His methodology is a structured way of thinking in which not only a perceived problematic...Many similarities exist between Systemic Operational Design and Soft Systems Methodology , their epistemology is related. Furthermore, they both have...Systems Thinking: Managing Chaos and Complexity. Boston: Butterworth Heinemann, 1999. Checkland, Peter, and Jim Scholes. Soft Systems Methodology in

Implementation of NASTRAN on the IBM/370 CMS operating system

NASA Technical Reports Server (NTRS)

Britten, S. S.; Schumacker, B.

1980-01-01

The NASA Structural Analysis (NASTRAN) computer program is operational on the IBM 360/370 series computers. While execution of NASTRAN has been described and implemented under the virtual storage operating systems of the IBM 370 models, the IBM 370/168 computer can also operate in a time-sharing mode under the virtual machine operating system using the Conversational Monitor System (CMS) subset. The changes required to make NASTRAN operational under the CMS operating system are described.

Redesigning the District Operating System

ERIC Educational Resources Information Center

Hodas, Steven

2015-01-01

In this paper, we look at the inner workings of a school district through the lens of the "district operating system (DOS)," a set of interlocking mutually-reinforcing modules that includes functions like procurement, contracting, data and IT policy, the general counsel's office, human resources, and the systems for employee and family…

Experimental Assessment of Two Exothermic Systems to Neutralize Landmines

DTIC Science & Technology

2006-02-01

S M 21 A T/ st ee l 4. 9 2 kg (5 lb ) 6” 1’ 0 5” D et on at io n of re si du al e xp lo si ve , cr at er 1 .0 m x...P T- M i-B a III A T/ ba ke lit e 7. 2 5. 0 kg (1 1 lb ) 40 ” 14 ’ 2 8” S m al l D et on at io n of re si du al e xp lo si ve... S m al l c ra te r

[Comprehensive system integration and networking in operating rooms].

PubMed

Feußner, H; Ostler, D; Kohn, N; Vogel, T; Wilhelm, D; Koller, S; Kranzfelder, M

2016-12-01

A comprehensive surveillance and control system integrating all devices and functions is a precondition for realization of the operating room of the future. Multiple proprietary integrated operation room systems are currently available with a central user interface; however, they only cover a relatively small part of all functionalities. Internationally, there are at least three different initiatives to promote a comprehensive systems integration and networking in the operating room: the Japanese smart cyber operating theater (SCOT), the American medical device plug-and-play interoperability program (MDPnP) and the German secure and dynamic networking in operating room and hospital (OR.NET) project supported by the Federal Ministry of Education and Research. Within the framework of the internationally advanced OR.NET project, prototype solution approaches were realized, which make short-term and mid-term comprehensive data retrieval systems probable. An active and even autonomous control of the medical devices by the surveillance and control system (closed loop) is expected only in the long run due to strict regulatory barriers.

Interactive orbital proximity operations planning system

NASA Technical Reports Server (NTRS)

Grunwald, Arthur J.; Ellis, Stephen R.

1989-01-01

An interactive, graphical proximity operations planning system was developed which allows on-site design of efficient, complex, multiburn maneuvers in the dynamic multispacecraft environment about the space station. Maneuvering takes place in, as well as out of, the orbital plane. The difficulty in planning such missions results from the unusual and counterintuitive character of relative orbital motion trajectories and complex operational constraints, which are both time varying and highly dependent on the mission scenario. This difficulty is greatly overcome by visualizing the relative trajectories and the relative constraints in an easily interpretable, graphical format, which provides the operator with immediate feedback on design actions. The display shows a perspective bird's-eye view of the space station and co-orbiting spacecraft on the background of the station's orbital plane. The operator has control over two modes of operation: (1) a viewing system mode, which enables him or her to explore the spatial situation about the space station and thus choose and frame in on areas of interest; and (2) a trajectory design mode, which allows the interactive editing of a series of way-points and maneuvering burns to obtain a trajectory which complies with all operational constraints. Through a graphical interactive process, the operator will continue to modify the trajectory design until all operational constraints are met. The effectiveness of this display format in complex trajectory design is presently being evaluated in an ongoing experimental program.

A unifying framework for systems modeling, control systems design, and system operation

NASA Technical Reports Server (NTRS)

Dvorak, Daniel L.; Indictor, Mark B.; Ingham, Michel D.; Rasmussen, Robert D.; Stringfellow, Margaret V.

2005-01-01

Current engineering practice in the analysis and design of large-scale multi-disciplinary control systems is typified by some form of decomposition- whether functional or physical or discipline-based-that enables multiple teams to work in parallel and in relative isolation. Too often, the resulting system after integration is an awkward marriage of different control and data mechanisms with poor end-to-end accountability. System of systems engineering, which faces this problem on a large scale, cries out for a unifying framework to guide analysis, design, and operation. This paper describes such a framework based on a state-, model-, and goal-based architecture for semi-autonomous control systems that guides analysis and modeling, shapes control system software design, and directly specifies operational intent. This paper illustrates the key concepts in the context of a large-scale, concurrent, globally distributed system of systems: NASA's proposed Array-based Deep Space Network.

Structural and optical properties of GaxIn1-xP layers grown by chemical beam epitaxy

NASA Astrophysics Data System (ADS)

Seong, Tae-Yeon; Yang, Jung-Ja; Ryu, Mee Yi; Song, Jong-In; Yu, Phil W.

1998-05-01

Chemical beam epitaxial (CBE) GaxIn1-xP layers (x≈0.5) grown on (001) GaAs substrates at temperatures ranging from 490 to 580°C have been investigated using transmission electron diffraction (TED), transmission electron microscopy, and photoluminescence (PL). TED examination revealed the presence of diffuse scattering 1/2{111}B positions, indicating the occurrence of typical CuPt-type ordering in the GaInP CBE layers. As the growth temperature decreased from 580 to 490°C, maxima in the intensity of the diffuse scattering moved from ½{111}B to ½{-1+δ,1-δ,0} positions, where δ is a positive value. As the growth temperature increased from 490 to 550°C, the maxima in the diffuse scattering intensity progressively approached positions of 1/2\\{bar 110\\} , i.e., the value of δ decreased from 0.25 to 0.17. Bandgap reduction (˜45 meV) was observed in the CBE GaInP layers and was attributed to the presence of ordered structures.

Appraisal of the Operation Respond Emergency Information System (OREIS)

DOT National Transportation Integrated Search

1998-04-01

The Operation Respond Institute has been instrumental in developing the Operation Respond Emergency Information System (OREIS) for first responders to hazardous material incidents in transportation. The Operation Respond system aims to facilitate rap...

InkTag: Secure Applications on an Untrusted Operating System.

PubMed

Hofmann, Owen S; Kim, Sangman; Dunn, Alan M; Lee, Michael Z; Witchel, Emmett

2013-01-01

InkTag is a virtualization-based architecture that gives strong safety guarantees to high-assurance processes even in the presence of a malicious operating system. InkTag advances the state of the art in untrusted operating systems in both the design of its hypervisor and in the ability to run useful applications without trusting the operating system. We introduce paraverification , a technique that simplifies the InkTag hypervisor by forcing the untrusted operating system to participate in its own verification. Attribute-based access control allows trusted applications to create decentralized access control policies. InkTag is also the first system of its kind to ensure consistency between secure data and metadata, ensuring recoverability in the face of system crashes.

The University of Wisconsin OAO operating system

NASA Technical Reports Server (NTRS)

Heacox, H. C.; Mcnall, J. F.

1972-01-01

The Wisconsin OAO operating system is presented which consists of two parts: a computer program called HARUSPEX, which makes possible reasonably efficient and convenient operation of the package and ground operations equipment which provides real-time status monitoring, commanding and a quick-look at the data.

An operational computer program to control Self Defense Surface Missile System operations

DOE Office of Scientific and Technical Information (OSTI.GOV)

Roe, C.L.

1991-12-01

An account is given of the system architecture and operational protocols of the NATO Seasparrow Surface Missile System (NSSMS) Operational Computer Program (OCP) which has been developed, and is being deployed multinationally, to respond against antiship missiles. Flowcharts are presented for the target detection and tracking, control, and engagement phases of the Self Defense Surface Missile System that is controlled by the OCP. USN and other NATO vessels will carry the NSSMS well into the next century; the OCP presently described will be deployed in the course of 1992 to enhance the self-defense capabilities of the NSSMS-equipped fleet. 8 refs.

Operating rules for multireservoir systems

NASA Astrophysics Data System (ADS)

Oliveira, Rodrigo; Loucks, Daniel P.

1997-04-01

Multireservoir operating policies are usually defined by rules that specify either individual reservoir desired (target) storage volumes or desired (target) releases based on the time of year and the existing total storage volume in all reservoirs. This paper focuses on the use of genetic search algorithms to derive these multireservoir operating policies. The genetic algorithms use real-valued vectors containing information needed to define both system release and individual reservoir storage volume targets as functions of total storage in each of multiple within-year periods. Elitism, arithmetic crossover, mutation, and "en bloc" replacement are used in the algorithms to generate successive sets of possible operating policies. Each policy is then evaluated using simulation to compute a performance index for a given flow series. The better performing policies are then used as a basis for generating new sets of possible policies. The process of improved policy generation and evaluation is repeated until no further improvement in performance is obtained. The proposed algorithm is applied to example reservoir systems used for water supply and hydropower.

Describing Elementary Teachers' Operative Systems: A Case Study

ERIC Educational Resources Information Center

Dotger, Sharon; McQuitty, Vicki

2014-01-01

This case study introduces the notion of an operative system to describe elementary teachers' knowledge and practice. Drawing from complex systems theory, the operative system is defined as the network of knowledge and practices that constituted teachers' work within a lesson study cycle. Data were gathered throughout a lesson study cycle in which…

A flexible telerobotic system for space operations

NASA Technical Reports Server (NTRS)

Sliwa, N. O.; Will, R. W.

1987-01-01

The objective and design of a proposed goal-oriented knowledge-based telerobotic system for space operations is described. This design effort encompasses the elements of the system executive and user interface and the distribution and general structure of the knowledge base, the displays, and the task sequencing. The objective of the design effort is to provide an expandable structure for a telerobotic system that provides cooperative interaction between the human operator and computer control. The initial phase of the implementation provides a rule-based, goal-oriented script generator to interface to the existing control modes of a telerobotic research system, in the Intelligent Systems Research Lab at NASA Research Center.

Head-mounted display systems and the special operations soldier

NASA Astrophysics Data System (ADS)

Loyd, Rodney B.

1998-08-01

In 1997, the Boeing Company, working with DARPA under the Smart Modules program and the US Army Soldier Systems Command, embarked on an advanced research and development program to develop a wearable computer system tailored for use with soldiers of the US Special Operations Command. The 'special operations combat management system' is a rugged advanced wearable tactical computer, designed to provide the special operations soldier with enhanced situation awareness and battlefield information capabilities. Many issues must be considered during the design of wearable computers for a combat soldier, including the system weight, placement on the body with respect to other equipment, user interfaces and display system characteristics. During the initial feasibility study for the system, the operational environment was examined and potential users were interviewed to establish the proper display solution for the system. Many display system requirements resulted, such as head or helmet mounting, Night Vision Goggle compatibility, minimal visible light emissions, environmental performance and even the need for handheld or other 'off the head' type display systems. This paper will address these issues and other end user requirements for display systems for applications in the harsh and demanding environment of the Special Operations soldier.

Recursive flexible multibody system dynamics using spatial operators

NASA Technical Reports Server (NTRS)

Jain, A.; Rodriguez, G.

1992-01-01

This paper uses spatial operators to develop new spatially recursive dynamics algorithms for flexible multibody systems. The operator description of the dynamics is identical to that for rigid multibody systems. Assumed-mode models are used for the deformation of each individual body. The algorithms are based on two spatial operator factorizations of the system mass matrix. The first (Newton-Euler) factorization of the mass matrix leads to recursive algorithms for the inverse dynamics, mass matrix evaluation, and composite-body forward dynamics for the systems. The second (innovations) factorization of the mass matrix, leads to an operator expression for the mass matrix inverse and to a recursive articulated-body forward dynamics algorithm. The primary focus is on serial chains, but extensions to general topologies are also described. A comparison of computational costs shows that the articulated-body, forward dynamics algorithm is much more efficient than the composite-body algorithm for most flexible multibody systems.

Expert operator's associate: A knowledge based system for spacecraft control

NASA Technical Reports Server (NTRS)

Nielsen, Mogens; Grue, Klaus; Lecouat, Francois

1991-01-01

The Expert Operator's Associate (EOA) project is presented which studies the applicability of expert systems for day-to-day space operations. A prototype expert system is developed, which operates on-line with an existing spacecraft control system at the European Space Operations Centre, and functions as an 'operator's assistant' in controlling satellites. The prototype is demonstrated using an existing real-time simulation model of the MARECS-B2 telecommunication satellite. By developing a prototype system, the extent to which reliability and effectivens of operations can be enhanced by AI based support is examined. In addition the study examines the questions of acquisition and representation of the 'knowledge' for such systems, and the feasibility of 'migration' of some (currently) ground-based functions into future spaceborne autonomous systems.

InkTag: Secure Applications on an Untrusted Operating System

PubMed Central

Hofmann, Owen S.; Kim, Sangman; Dunn, Alan M.; Lee, Michael Z.; Witchel, Emmett

2014-01-01

InkTag is a virtualization-based architecture that gives strong safety guarantees to high-assurance processes even in the presence of a malicious operating system. InkTag advances the state of the art in untrusted operating systems in both the design of its hypervisor and in the ability to run useful applications without trusting the operating system. We introduce paraverification, a technique that simplifies the InkTag hypervisor by forcing the untrusted operating system to participate in its own verification. Attribute-based access control allows trusted applications to create decentralized access control policies. InkTag is also the first system of its kind to ensure consistency between secure data and metadata, ensuring recoverability in the face of system crashes. PMID:24429939

Development of lung cancer CT screening operating support system

NASA Astrophysics Data System (ADS)

Ishigaki, Rikuta; Hanai, Kozou; Suzuki, Masahiro; Kawata, Yoshiki; Niki, Noboru; Eguchi, Kenji; Kakinuma, Ryutaro; Moriyama, Noriyuki

2009-02-01

In Japan, lung cancer death ranks first among men and third among women. Lung cancer death is increasing yearly, thus early detection and treatment are needed. For this reason, CT screening for lung cancer has been introduced. The CT screening services are roughly divided into three sections: office, radiology and diagnosis sections. These operations have been performed through paper-based or a combination of paper-based and an existing electronic health recording system. This paper describes an operating support system for lung cancer CT screening in order to make the screening services efficient. This operating support system is developed on the basis of 1) analysis of operating processes, 2) digitalization of operating information, and 3) visualization of operating information. The utilization of the system is evaluated through an actual application and users' survey questionnaire obtained from CT screening centers.

Autonomous System Technologies for Resilient Airspace Operations

NASA Technical Reports Server (NTRS)

Houston, Vincent E.; Le Vie, Lisa R.

2017-01-01

Increasing autonomous systems within the aircraft cockpit begins with an effort to understand what autonomy is and developing the technology that encompasses it. Autonomy allows an agent, human or machine, to act independently within a circumscribed set of goals; delegating responsibility to the agent(s) to achieve overall system objective(s). Increasingly Autonomous Systems (IAS) are the highly sophisticated progression of current automated systems toward full autonomy. Working in concert with humans, these types of technologies are expected to improve the safety, reliability, costs, and operational efficiency of aviation. IAS implementation is imminent, which makes the development and the proper performance of such technologies, with respect to cockpit operation efficiency, the management of air traffic and data communication information, vital. A prototype IAS agent that attempts to optimize the identification and distribution of "relevant" air traffic data to be utilized by human crews during complex airspace operations has been developed.

Smart Operations in Distributed Energy Resources System

NASA Astrophysics Data System (ADS)

Wei, Li; Jie, Shu; Zhang-XianYong; Qing, Zhou

Smart grid capabilities are being proposed to help solve the challenges concerning system operations due to that the trade-offs between energy and environmental needs will be constantly negotiated while a reliable supply of electricity needs even greater assurance in case of that threats of disruption have risen. This paper mainly explores models for distributed energy resources system (DG, storage, and load),and also reviews the evolving nature of electricity markets to deal with this complexity and a change of emphasis on signals from these markets to affect power system control. Smart grid capabilities will also impact reliable operations, while cyber security issues must be solved as a culture change that influences all system design, implementation, and maintenance. Lastly, the paper explores significant questions for further research and the need for a simulation environment that supports such investigation and informs deployments to mitigate operational issues as they arise.

A new taxonomy for distributed computer systems based upon operating system structure

NASA Technical Reports Server (NTRS)

Foudriat, E. C.

1985-01-01

Characteristics of the resource structure found in the operating system are considered as a mechanism for classifying distributed computer systems. Since the operating system resources, themselves, are too diversified to provide a consistent classification, the structure upon which resources are built and shared are examined. The location and control character of this indivisibility provides the taxonomy for separating uniprocessors, computer networks, network computers (fully distributed processing systems or decentralized computers) and algorithm and/or data control multiprocessors. The taxonomy is important because it divides machines into a classification that is relevant or important to the client and not the hardware architect. It also defines the character of the kernel O/S structure needed for future computer systems. What constitutes an operating system for a fully distributed processor is discussed in detail.

A novel interactive educational system in the operating room--the IE system.

PubMed

Nakayama, Takayuki; Numao, Noboru; Yoshida, Soichiro; Ishioka, Junichiro; Matsuoka, Yoh; Saito, Kazutaka; Fujii, Yasuhisa; Kihara, Kazunori

2016-02-02

The shortage of surgeon is one of the serious problems in Japan. To solve the problem, various efforts have been undertaken to improve surgical education and training. However, appropriate teaching methods in the operating room have not been well established. The aim of this study is to assess the utility of a novel interactive educational (IE) system for surgical education on urologic surgeries in the operating room. A total of 20 Japanese medical students were educated on urologic surgery using the IE system in the operating room. The IE system consists of two parts. The first is three-dimensional (3D) magnified vision of the operative field using a 3D head-mounted display and a 3D endoscope. The second is interactive educative communication between medical students and surgeons using a small-sized wireless communication device. The satisfaction level with the IE system and the physical burden on medical students was examined via questionnaire. All students utilized the IE system in urologic surgery and responded to the survey. Most students were satisfied with the IE system. They also felt more welcomed by the surgeon when using the IE system than when not using it. No major unpleasant symptoms were observed but five students (25 %) experienced mild eye fatigue as a result of viewing the medical images. The IE system has the potential to motivate students to become interested in surgery and could be an efficient method of surgical education in the operating room.

Deep phenotyping of 89 xeroderma pigmentosum patients reveals unexpected heterogeneity dependent on the precise molecular defect

PubMed Central

Fassihi, Hiva; Sethi, Mieran; Fawcett, Heather; Wing, Jonathan; Chandler, Natalie; Mohammed, Shehla; Craythorne, Emma; Morley, Ana M. S.; Lim, Rongxuan; Turner, Sally; Henshaw, Tanya; Garrood, Isabel; Giunti, Paola; Hedderly, Tammy; Abiona, Adesoji; Naik, Harsha; Harrop, Gemma; McGibbon, David; Jaspers, Nicolaas G. J.; Botta, Elena; Nardo, Tiziana; Stefanini, Miria; Young, Antony R.; Sarkany, Robert P. E.; Lehmann, Alan R.

2016-01-01

Xeroderma pigmentosum (XP) is a rare DNA repair disorder characterized by increased susceptibility to UV radiation (UVR)-induced skin pigmentation, skin cancers, ocular surface disease, and, in some patients, sunburn and neurological degeneration. Genetically, it is assigned to eight complementation groups (XP-A to -G and variant). For the last 5 y, the UK national multidisciplinary XP service has provided follow-up for 89 XP patients, representing most of the XP patients in the United Kingdom. Causative mutations, DNA repair levels, and more than 60 clinical variables relating to dermatology, ophthalmology, and neurology have been measured, using scoring systems to categorize disease severity. This deep phenotyping has revealed unanticipated heterogeneity of clinical features, between and within complementation groups. Skin cancer is most common in XP-C, XP-E, and XP-V patients, previously considered to be the milder groups based on cellular analyses. These patients have normal sunburn reactions and are therefore diagnosed later and are less likely to adhere to UVR protection. XP-C patients are specifically hypersensitive to ocular damage, and XP-F and XP-G patients appear to be much less susceptible to skin cancer than other XP groups. Within XP groups, different mutations confer susceptibility or resistance to neurological damage. Our findings on this large cohort of XP patients under long-term follow-up reveal that XP is more heterogeneous than has previously been appreciated. Our data now enable provision of personalized prognostic information and management advice for each XP patient, as well as providing new insights into the functions of the XP proteins. PMID:26884178

Hanford Environmental Information System (HEIS) Operator`s Manual. Volume 1

DOE Office of Scientific and Technical Information (OSTI.GOV)

Schreck, R.I.

1991-10-01

The Hanford Environmental Information System (HEIS) is a consolidated set of automated resources that effectively manage the data gathered during environmental monitoring and restoration of the Hanford Site. The HEIS includes an integrated database that provides consistent and current data to all users and promotes sharing of data by the entire user community. This manual describes the facilities available to the operational user who is responsible for data entry, processing, scheduling, reporting, and quality assurance. A companion manual, the HEIS User`s Manual, describes the facilities available-to the scientist, engineer, or manager who uses the system for environmental monitoring, assessment, andmore » restoration planning; and to the regulator who is responsible for reviewing Hanford Site operations against regulatory requirements and guidelines.« less

Time warp operating system version 2.7 internals manual

NASA Technical Reports Server (NTRS)

1992-01-01

The Time Warp Operating System (TWOS) is an implementation of the Time Warp synchronization method proposed by David Jefferson. In addition, it serves as an actual platform for running discrete event simulations. The code comprising TWOS can be divided into several different sections. TWOS typically relies on an existing operating system to furnish some very basic services. This existing operating system is referred to as the Base OS. The existing operating system varies depending on the hardware TWOS is running on. It is Unix on the Sun workstations, Chrysalis or Mach on the Butterfly, and Mercury on the Mark 3 Hypercube. The base OS could be an entirely new operating system, written to meet the special needs of TWOS, but, to this point, existing systems have been used instead. The base OS's used for TWOS on various platforms are not discussed in detail in this manual, as they are well covered in their own manuals. Appendix G discusses the interface between one such OS, Mach, and TWOS.

Prototype operational earthquake prediction system

USGS Publications Warehouse

Spall, Henry

1986-01-01

An objective if the U.S. Earthquake Hazards Reduction Act of 1977 is to introduce into all regions of the country that are subject to large and moderate earthquakes, systems for predicting earthquakes and assessing earthquake risk. In 1985, the USGS developed for the Secretary of the Interior a program for implementation of a prototype operational earthquake prediction system in southern California.

Naturalistic Decision Making For Power System Operators

DOE Office of Scientific and Technical Information (OSTI.GOV)

Greitzer, Frank L.; Podmore, Robin; Robinson, Marck

2009-06-23

Abstract: Motivation -- As indicated by the Blackout of 2003, the North American interconnected electric system is vulnerable to cascading outages and widespread blackouts. Investigations of large scale outages often attribute the causes to the three T’s: Trees, Training and Tools. A systematic approach has been developed to document and understand the mental processes that an expert power system operator uses when making critical decisions. The approach has been developed and refined as part of a capability demonstration of a high-fidelity real-time power system simulator under normal and emergency conditions. To examine naturalistic decision making (NDM) processes, transcripts of operator-to-operatormore » conversations are analyzed to reveal and assess NDM-based performance criteria. Findings/Design -- The results of the study indicate that we can map the Situation Awareness Level of the operators at each point in the scenario. We can also identify clearly what mental models and mental simulations are being performed at different points in the scenario. As a result of this research we expect that we can identify improved training methods and improved analytical and visualization tools for power system operators. Originality/Value -- The research applies for the first time, the concepts of Recognition Primed Decision Making, Situation Awareness Levels and Cognitive Task Analysis to training of electric power system operators. Take away message -- The NDM approach provides an ideal framework for systematic training management and mitigation to accelerate learning in team-based training scenarios with high-fidelity power grid simulators.« less

14 CFR 27.961 - Fuel system hot weather operation.

Code of Federal Regulations, 2010 CFR

2010-01-01

... AIRCRAFT AIRWORTHINESS STANDARDS: NORMAL CATEGORY ROTORCRAFT Powerplant Fuel System § 27.961 Fuel system hot weather operation. Each suction lift fuel system and other fuel systems with features conducive to... 14 Aeronautics and Space 1 2010-01-01 2010-01-01 false Fuel system hot weather operation. 27.961...

14 CFR 27.961 - Fuel system hot weather operation.

Code of Federal Regulations, 2011 CFR

2011-01-01

... AIRCRAFT AIRWORTHINESS STANDARDS: NORMAL CATEGORY ROTORCRAFT Powerplant Fuel System § 27.961 Fuel system hot weather operation. Each suction lift fuel system and other fuel systems with features conducive to... 14 Aeronautics and Space 1 2011-01-01 2011-01-01 false Fuel system hot weather operation. 27.961...

14 CFR 29.961 - Fuel system hot weather operation.

Code of Federal Regulations, 2013 CFR

2013-01-01

... AIRCRAFT AIRWORTHINESS STANDARDS: TRANSPORT CATEGORY ROTORCRAFT Powerplant Fuel System § 29.961 Fuel system hot weather operation. Each suction lift fuel system and other fuel systems conducive to vapor... 14 Aeronautics and Space 1 2013-01-01 2013-01-01 false Fuel system hot weather operation. 29.961...

14 CFR 27.961 - Fuel system hot weather operation.

Code of Federal Regulations, 2014 CFR

2014-01-01

... AIRCRAFT AIRWORTHINESS STANDARDS: NORMAL CATEGORY ROTORCRAFT Powerplant Fuel System § 27.961 Fuel system hot weather operation. Each suction lift fuel system and other fuel systems with features conducive to... 14 Aeronautics and Space 1 2014-01-01 2014-01-01 false Fuel system hot weather operation. 27.961...

14 CFR 27.961 - Fuel system hot weather operation.

Code of Federal Regulations, 2012 CFR

2012-01-01

... AIRCRAFT AIRWORTHINESS STANDARDS: NORMAL CATEGORY ROTORCRAFT Powerplant Fuel System § 27.961 Fuel system hot weather operation. Each suction lift fuel system and other fuel systems with features conducive to... 14 Aeronautics and Space 1 2012-01-01 2012-01-01 false Fuel system hot weather operation. 27.961...

14 CFR 29.961 - Fuel system hot weather operation.

Code of Federal Regulations, 2011 CFR

2011-01-01

... AIRCRAFT AIRWORTHINESS STANDARDS: TRANSPORT CATEGORY ROTORCRAFT Powerplant Fuel System § 29.961 Fuel system hot weather operation. Each suction lift fuel system and other fuel systems conducive to vapor... 14 Aeronautics and Space 1 2011-01-01 2011-01-01 false Fuel system hot weather operation. 29.961...

14 CFR 27.961 - Fuel system hot weather operation.

Code of Federal Regulations, 2013 CFR

2013-01-01

... AIRCRAFT AIRWORTHINESS STANDARDS: NORMAL CATEGORY ROTORCRAFT Powerplant Fuel System § 27.961 Fuel system hot weather operation. Each suction lift fuel system and other fuel systems with features conducive to... 14 Aeronautics and Space 1 2013-01-01 2013-01-01 false Fuel system hot weather operation. 27.961...

14 CFR 29.961 - Fuel system hot weather operation.

Code of Federal Regulations, 2014 CFR

2014-01-01

... AIRCRAFT AIRWORTHINESS STANDARDS: TRANSPORT CATEGORY ROTORCRAFT Powerplant Fuel System § 29.961 Fuel system hot weather operation. Each suction lift fuel system and other fuel systems conducive to vapor... 14 Aeronautics and Space 1 2014-01-01 2014-01-01 false Fuel system hot weather operation. 29.961...

14 CFR 29.961 - Fuel system hot weather operation.

Code of Federal Regulations, 2010 CFR

2010-01-01

... AIRCRAFT AIRWORTHINESS STANDARDS: TRANSPORT CATEGORY ROTORCRAFT Powerplant Fuel System § 29.961 Fuel system hot weather operation. Each suction lift fuel system and other fuel systems conducive to vapor... 14 Aeronautics and Space 1 2010-01-01 2010-01-01 false Fuel system hot weather operation. 29.961...

14 CFR 29.961 - Fuel system hot weather operation.

Code of Federal Regulations, 2012 CFR

2012-01-01

... AIRCRAFT AIRWORTHINESS STANDARDS: TRANSPORT CATEGORY ROTORCRAFT Powerplant Fuel System § 29.961 Fuel system hot weather operation. Each suction lift fuel system and other fuel systems conducive to vapor... 14 Aeronautics and Space 1 2012-01-01 2012-01-01 false Fuel system hot weather operation. 29.961...

Making adaptable systems work for mission operations: A case study

NASA Technical Reports Server (NTRS)

Holder, Barbara E.; Levesque, Michael E.

1993-01-01

The Advanced Multimission Operations System (AMMOS) at NASA's Jet Propulsion Laboratory is based on a highly adaptable multimission ground data system (MGDS) for mission operations. The goal for MGDS is to support current flight project science and engineering personnel and to meet the demands of future missions while reducing associated operations and software development costs. MGDS has become a powerful and flexible mission operations system by using a network of heterogeneous workstations, emerging open system standards, and selecting an adaptable tools-based architecture. Challenges in developing adaptable systems for mission operations and the benefits of this approach are described.

The HAL 9000 Space Operating System Real-Time Planning Engine Design and Operations Requirements

NASA Technical Reports Server (NTRS)

Stetson, Howard; Watson, Michael D.; Shaughnessy, Ray

2012-01-01

In support of future deep space manned missions, an autonomous/automated vehicle, providing crew autonomy and an autonomous response planning system, will be required due to the light time delays in communication. Vehicle capabilities as a whole must provide for tactical response to vehicle system failures and space environmental effects induced failures, for risk mitigation of permanent loss of communication with Earth, and for assured crew return capabilities. The complexity of human rated space systems and the limited crew sizes and crew skills mix drive the need for a robust autonomous capability on-board the vehicle. The HAL 9000 Space Operating System[2] designed for such missions and space craft includes the first distributed real-time planning / re-planning system. This paper will detail the software architecture of the multiple planning engine system, and the interface design for plan changes, approval and implementation that is performed autonomously. Operations scenarios will be defined for analysis of the planning engines operations and its requirements for nominal / off nominal activities. An assessment of the distributed realtime re-planning system, in the defined operations environment, will be provided as well as findings as it pertains to the vehicle, crew, and mission control requirements needed for implementation.

A Framework for Enterprise Operating Systems Based on Zachman Framework

NASA Astrophysics Data System (ADS)

Ostadzadeh, S. Shervin; Rahmani, Amir Masoud

Nowadays, the Operating System (OS) isn't only the software that runs your computer. In the typical information-driven organization, the operating system is part of a much larger platform for applications and data that extends across the LAN, WAN and Internet. An OS cannot be an island unto itself; it must work with the rest of the enterprise. Enterprise wide applications require an Enterprise Operating System (EOS). Enterprise operating systems used in an enterprise have brought about an inevitable tendency to lunge towards organizing their information activities in a comprehensive way. In this respect, Enterprise Architecture (EA) has proven to be the leading option for development and maintenance of enterprise operating systems. EA clearly provides a thorough outline of the whole information system comprising an enterprise. To establish such an outline, a logical framework needs to be laid upon the entire information system. Zachman Framework (ZF) has been widely accepted as a standard scheme for identifying and organizing descriptive representations that have prominent roles in enterprise-wide system development. In this paper, we propose a framework based on ZF for enterprise operating systems. The presented framework helps developers to design and justify completely integrated business, IT systems, and operating systems which results in improved project success rate.

The IUE Science Operations Ground System

NASA Technical Reports Server (NTRS)

Pitts, Ronald E.; Arquilla, Richard

1994-01-01

The International Ultraviolet Explorer (IUE) Science Operations System provides full realtime operations capabilities and support to the operations staff and astronomer users. The components of this very diverse and extremely flexible hardware and software system have played a major role in maintaining the scientific efficiency and productivity of the IUE. The software provides the staff and user with all the tools necessary for pre-visit and real-time planning and operations analysis for any day of the year. Examples of such tools include the effects of spacecraft constraints on target availability, maneuver times between targets, availability of guide stars, target identification, coordinate transforms, e-mail transfer of Observatory forms and messages, and quick-look analysis of image data. Most of this extensive software package can also be accessed remotely by individual users for information, scheduling of shifts, pre-visit planning, and actual observing program execution. Astronomers, with a modest investment in hardware and software, may establish remote observing sites. We currently have over 20 such sites in our remote observers' network.

An approach to the design of operations systems

NASA Technical Reports Server (NTRS)

Chafin, Roy L.; Curran, Patrick S.

1993-01-01

The MultiMission Control Team (MMCT) consists of mission controllers which provides Real-Time operations support for the Mars Observer project. The Real-Time Operations task is to insure the integrity of the ground data system, to insure that the configuration is correct to support the mission, and to monitor the spacecraft for the Spacecraft Team. Operations systems are typically developed by adapting operations systems from previous projects. Problems tend to be solved empirically when they are either anticipated or observed in testing. This development method has worked in the past when time was available for extensive Ops testing. In the present NASA budget environment, a more cost conscious design approach has become necessary. Cost is a concern because operations is an ongoing, continuous activity. Reducing costs entails reducing staff. Reducing staffing levels potentially increases the risk of mission failure. Therefore, keeping track of the risk level is necessary.

Launch processing system transition from development to operation

NASA Technical Reports Server (NTRS)

Paul, H. C.

1977-01-01

The Launch Processing System has been under development at Kennedy Space Center since 1973. A prototype system was developed and delivered to Marshall Space Flight Center for Solid Rocket Booster checkout in July 1976. The first production hardware arrived in late 1976. The System uses a distributed computer network for command and monitoring and is supported by a dual large scale computer system for 'off line' processing. A high level of automation is anticipated for Shuttle and Payload testing and launch operations to gain the advantages of short turnaround capability, repeatability of operations, and minimization of operations and maintenance (O&M) manpower. Learning how to efficiently apply the system is our current problem. We are searching for more effective ways to convey LPS system performance characteristics from the designer to a large number of users. Once we have done this, we can realize the advantages of LPS system design.

EXODUS: Integrating intelligent systems for launch operations support

NASA Technical Reports Server (NTRS)

Adler, Richard M.; Cottman, Bruce H.

1991-01-01

Kennedy Space Center (KSC) is developing knowledge-based systems to automate critical operations functions for the space shuttle fleet. Intelligent systems will monitor vehicle and ground support subsystems for anomalies, assist in isolating and managing faults, and plan and schedule shuttle operations activities. These applications are being developed independently of one another, using different representation schemes, reasoning and control models, and hardware platforms. KSC has recently initiated the EXODUS project to integrate these stand alone applications into a unified, coordinated intelligent operations support system. EXODUS will be constructed using SOCIAL, a tool for developing distributed intelligent systems. EXODUS, SOCIAL, and initial prototyping efforts using SOCIAL to integrate and coordinate selected EXODUS applications are described.

Modeling and Simulation for Mission Operations Work System Design

NASA Technical Reports Server (NTRS)

Sierhuis, Maarten; Clancey, William J.; Seah, Chin; Trimble, Jay P.; Sims, Michael H.

2003-01-01

Work System analysis and design is complex and non-deterministic. In this paper we describe Brahms, a multiagent modeling and simulation environment for designing complex interactions in human-machine systems. Brahms was originally conceived as a business process design tool that simulates work practices, including social systems of work. We describe our modeling and simulation method for mission operations work systems design, based on a research case study in which we used Brahms to design mission operations for a proposed discovery mission to the Moon. We then describe the results of an actual method application project-the Brahms Mars Exploration Rover. Space mission operations are similar to operations of traditional organizations; we show that the application of Brahms for space mission operations design is relevant and transferable to other types of business processes in organizations.

Information processing of motion in facial expression and the geometry of dynamical systems

NASA Astrophysics Data System (ADS)

Assadi, Amir H.; Eghbalnia, Hamid; McMenamin, Brenton W.

2005-01-01

An interesting problem in analysis of video data concerns design of algorithms that detect perceptually significant features in an unsupervised manner, for instance methods of machine learning for automatic classification of human expression. A geometric formulation of this genre of problems could be modeled with help of perceptual psychology. In this article, we outline one approach for a special case where video segments are to be classified according to expression of emotion or other similar facial motions. The encoding of realistic facial motions that convey expression of emotions for a particular person P forms a parameter space XP whose study reveals the "objective geometry" for the problem of unsupervised feature detection from video. The geometric features and discrete representation of the space XP are independent of subjective evaluations by observers. While the "subjective geometry" of XP varies from observer to observer, levels of sensitivity and variation in perception of facial expressions appear to share a certain level of universality among members of similar cultures. Therefore, statistical geometry of invariants of XP for a sample of population could provide effective algorithms for extraction of such features. In cases where frequency of events is sufficiently large in the sample data, a suitable framework could be provided to facilitate the information-theoretic organization and study of statistical invariants of such features. This article provides a general approach to encode motion in terms of a particular genre of dynamical systems and the geometry of their flow. An example is provided to illustrate the general theory.

A Study on the Deriving Requirements of ARGO Operation System

NASA Astrophysics Data System (ADS)

Seo, Yoon-Kyung; Rew, Dong-Young; Lim, Hyung-Chul; Park, In-Kwan; Yim, Hong-Suh; Jo, Jung Hyun; Park, Jong-Uk

2009-12-01

Korea Astronomy and Space Science Institute (KASI) has been developing one mobile and one stationary SLR system since 2008 named as ARGO-M and ARGO-F, respectively. KASI finished the step of deriving the system requirements of ARGO. The requirements include definitions and scopes of various software and hardware components which are necessary for developing the ARGO-M operation system. And the requirements define function, performance, and interface requirements. The operation system consisting of ARGO-M site, ARGO-F site, and Remote Operation Center (ROC) inside KASI is designed for remote access and the automatic tracking and control system which are the main operation concept of ARGO system. To accomplish remote operation, we are considering remote access to ARGO-F and ARGO-M from ROC. The mobile-phone service allows us to access the ARGO-F remotely and to control the system in an emergency. To implement fully automatic tracking and control function in ARGO-F, we have investigated and described the requirements about the automatic aircraft detection system and the various meteorological sensors. This paper addresses the requirements of ARGO Operation System.

Shuttle remote manipulator system mission preparation and operations

NASA Technical Reports Server (NTRS)

Smith, Ernest E., Jr.

1989-01-01

The preflight planning, analysis, procedures development, and operations support for the Space Transportation System payload deployment and retrieval missions utilizing the Shuttle Remote Manipulator System are summarized. Analysis of the normal operational loads and failure induced loads and motion are factored into all procedures. Both the astronaut flight crews and the Mission Control Center flight control teams receive considerable training for standard and mission specific operations. The real time flight control team activities are described.

PYROLASER - PYROLASER OPTICAL PYROMETER OPERATING SYSTEM

NASA Technical Reports Server (NTRS)

Roberts, F. E.

1994-01-01

The PYROLASER package is an operating system for the Pyrometer Instrument Company's Pyrolaser. There are 6 individual programs in the PYROLASER package: two main programs, two lower level subprograms, and two programs which, although independent, function predominantly as macros. The package provides a quick and easy way to setup, control, and program a standard Pyrolaser. Temperature and emissivity measurements may be either collected as if the Pyrolaser were in the manual operations mode, or displayed on real time strip charts and stored in standard spreadsheet format for post-test analysis. A shell is supplied to allow macros, which are test-specific, to be easily added to the system. The Pyrolaser Simple Operation program provides full on-screen remote operation capabilities, thus allowing the user to operate the Pyrolaser from the computer just as it would be operated manually. The Pyrolaser Simple Operation program also allows the use of "quick starts". Quick starts provide an easy way to permit routines to be used as setup macros for specific applications or tests. The specific procedures required for a test may be ordered in a sequence structure and then the sequence structure can be started with a simple button in the cluster structure provided. One quick start macro is provided for continuous Pyrolaser operation. A subprogram, Display Continuous Pyr Data, is used to display and store the resulting data output. Using this macro, the system is set up for continuous operation and the subprogram is called to display the data in real time on strip charts. The data is simultaneously stored in a spreadsheet format. The resulting spreadsheet file can be opened in any one of a number of commercially available spreadsheet programs. The Read Continuous Pyrometer program is provided as a continuously run subprogram for incorporation of the Pyrolaser software into a process control or feedback control scheme in a multi-component system. The program requires the

Operationally Efficient Propulsion System Study (OEPSS) data book. Executive summary

NASA Technical Reports Server (NTRS)

Wong, George S.

1990-01-01

The study was initiated to identify operations problems and cost drivers for current propulsion systems and to identify technology and design approaches to increase the operational efficiency and reduce operations costs for future propulsion systems. To provide readily usable data for the Advanced Launch System (ALS) program, the results of the Operationally Efficient Propulsion System Study (OEPSS) were organized into a series of OEPSS Data Books as follows: Volume 1, Generic Ground Operations Data; Volume 2, Ground Operations Problems; Volume 3, Operations Technology; Volume 4, OEPSS Design Concepts; and Volume 5, OEPSS Final Review Briefing, which summarizes the activities and results of the study. Summarized here are the salient results of the first year. A synopsis of each volume listed above is presented.

Advancing satellite operations with intelligent graphical monitoring systems

NASA Technical Reports Server (NTRS)

Hughes, Peter M.; Shirah, Gregory W.; Luczak, Edward C.

1993-01-01

For nearly twenty-five years, spacecraft missions have been operated in essentially the same manner: human operators monitor displays filled with alphanumeric text watching for limit violations or other indicators that signal a problem. The task is performed predominately by humans. Only in recent years have graphical user interfaces and expert systems been accepted within the control center environment to help reduce operator workloads. Unfortunately, the development of these systems is often time consuming and costly. At the NASA Goddard Space Flight Center (GSFC), a new domain specific expert system development tool called the Generic Spacecraft Analyst Assistant (GenSAA) has been developed. Through the use of a highly graphical user interface and point-and-click operation, GenSAA facilitates the rapid, 'programming-free' construction of intelligent graphical monitoring systems to serve as real-time, fault-isolation assistants for spacecraft analysts. Although specifically developed to support real-time satellite monitoring, GenSAA can support the development of intelligent graphical monitoring systems in a variety of space and commercial applications.

Unix becoming healthcare's standard operating system.

PubMed

Gardner, E

1991-02-11

An unfamiliar buzzword is making its way into healthcare executives' vocabulary, as well as their computer systems. Unix is being touted by many industry observers as the most likely candidate to be a standard operating system for minicomputers, mainframes and computer networks.

Clinical, biochemical, and neuropsychiatric evaluation of a patient with a contiguous gene syndrome due to a microdeletion Xp11.3 including the Norrie disease locus and monoamine oxidase (MAOA and MAOB) genes.

PubMed

Collins, F A; Murphy, D L; Reiss, A L; Sims, K B; Lewis, J G; Freund, L; Karoum, F; Zhu, D; Maumenee, I H; Antonarakis, S E

1992-01-01

Norrie disease is a rare X-linked recessive disorder characterized by blindness from infancy. The gene for Norrie disease has been localized to Xp11.3. More recently, the genes for monoamine oxidase (MAOA, MAOB) have been mapped to the same region. This study evaluates the clinical, biochemical, and neuropsychiatric data in an affected male and 2 obligate heterozygote females from a single family with a submicroscopic deletion involving Norrie disease and MAO genes. The propositus was a profoundly retarded, blind male; he also had neurologic abnormalities including myoclonus and stereotopy-habit disorder. Both obligate carrier females had a normal IQ. The propositus' mother met diagnostic criteria for "chronic hypomania and schizotypal features." The propositus' MAO activity was undetectable and the female heterozygotes had reduced levels comparable to patients receiving MAO inhibiting antidepressants. MAO substrate and metabolite abnormalities were found in the propositus' plasma and CSF. This study indicates that subtle biochemical and possibly neuropsychiatric abnormalities may be detected in some heterozygotes with the microdeletion in Xp11.3 due to loss of the gene product for the MAO genes; this deletion can also explain some of the complex phenotype of this contiguous gene syndrome in the propositus.

14 CFR 23.961 - Fuel system hot weather operation.

Code of Federal Regulations, 2011 CFR

2011-01-01

... 14 Aeronautics and Space 1 2011-01-01 2011-01-01 false Fuel system hot weather operation. 23.961... AIRCRAFT AIRWORTHINESS STANDARDS: NORMAL, UTILITY, ACROBATIC, AND COMMUTER CATEGORY AIRPLANES Powerplant Fuel System § 23.961 Fuel system hot weather operation. Each fuel system must be free from vapor lock...

14 CFR 23.961 - Fuel system hot weather operation.

Code of Federal Regulations, 2012 CFR

2012-01-01

... 14 Aeronautics and Space 1 2012-01-01 2012-01-01 false Fuel system hot weather operation. 23.961... AIRCRAFT AIRWORTHINESS STANDARDS: NORMAL, UTILITY, ACROBATIC, AND COMMUTER CATEGORY AIRPLANES Powerplant Fuel System § 23.961 Fuel system hot weather operation. Each fuel system must be free from vapor lock...

14 CFR 23.961 - Fuel system hot weather operation.

Code of Federal Regulations, 2010 CFR

2010-01-01

... 14 Aeronautics and Space 1 2010-01-01 2010-01-01 false Fuel system hot weather operation. 23.961... AIRCRAFT AIRWORTHINESS STANDARDS: NORMAL, UTILITY, ACROBATIC, AND COMMUTER CATEGORY AIRPLANES Powerplant Fuel System § 23.961 Fuel system hot weather operation. Each fuel system must be free from vapor lock...

14 CFR 23.961 - Fuel system hot weather operation.

Code of Federal Regulations, 2014 CFR

2014-01-01

... 14 Aeronautics and Space 1 2014-01-01 2014-01-01 false Fuel system hot weather operation. 23.961... AIRCRAFT AIRWORTHINESS STANDARDS: NORMAL, UTILITY, ACROBATIC, AND COMMUTER CATEGORY AIRPLANES Powerplant Fuel System § 23.961 Fuel system hot weather operation. Each fuel system must be free from vapor lock...

14 CFR 23.961 - Fuel system hot weather operation.

Code of Federal Regulations, 2013 CFR

2013-01-01

... 14 Aeronautics and Space 1 2013-01-01 2013-01-01 false Fuel system hot weather operation. 23.961... AIRCRAFT AIRWORTHINESS STANDARDS: NORMAL, UTILITY, ACROBATIC, AND COMMUTER CATEGORY AIRPLANES Powerplant Fuel System § 23.961 Fuel system hot weather operation. Each fuel system must be free from vapor lock...