Cystogenesis and elongated primary cilia in Tsc1-deficient distal convoluted tubules.

Science.gov (United States)

Armour, Eric A; Carson, Robert P; Ess, Kevin C

2012-08-15

Tuberous sclerosis complex (TSC) is a multiorgan hamartomatous disease caused by loss of function mutations of either the TSC1 or TSC2 genes. Neurological symptoms of TSC predominate in younger patients, but renal pathologies are a serious aspect of the disease in older children and adults. To study TSC pathogenesis in the kidney, we inactivated the mouse Tsc1 gene in the distal convoluted tubules (DCT). At young ages, Tsc1 conditional knockout (CKO) mice have enlarged kidneys and mild cystogenesis with increased mammalian target of rapamycin complex (mTORC)1 but decreased mTORC2 signaling. Treatment with the mTORC1 inhibitor rapamycin reduces kidney size and cystogenesis. Rapamycin withdrawal led to massive cystogenesis involving both distal as well as proximal tubules. To assess the contribution of decreased mTORC2 signaling in kidney pathogenesis, we also generated Rictor CKO mice. These animals did not have any detectable kidney pathology. Finally, we examined primary cilia in the DCT. Cilia were longer in Tsc1 CKO mice, and rapamycin treatment returned cilia length to normal. Rictor CKO mice had normal cilia in the DCT. Overall, our findings suggest that loss of the Tsc1 gene in the DCT is sufficient for renal cystogenesis. This cytogenesis appears to be mTORC1 but not mTORC2 dependent. Intriguingly, the mechanism may be cell autonomous as well as non-cell autonomous and possibly involves the length and function of primary cilia.

Cystogenesis and elongated primary cilia in Tsc1-deficient distal convoluted tubules

Science.gov (United States)

Armour, Eric A.; Carson, Robert P.

2012-01-01

Tuberous sclerosis complex (TSC) is a multiorgan hamartomatous disease caused by loss of function mutations of either the TSC1 or TSC2 genes. Neurological symptoms of TSC predominate in younger patients, but renal pathologies are a serious aspect of the disease in older children and adults. To study TSC pathogenesis in the kidney, we inactivated the mouse Tsc1 gene in the distal convoluted tubules (DCT). At young ages, Tsc1 conditional knockout (CKO) mice have enlarged kidneys and mild cystogenesis with increased mammalian target of rapamycin complex (mTORC)1 but decreased mTORC2 signaling. Treatment with the mTORC1 inhibitor rapamycin reduces kidney size and cystogenesis. Rapamycin withdrawal led to massive cystogenesis involving both distal as well as proximal tubules. To assess the contribution of decreased mTORC2 signaling in kidney pathogenesis, we also generated Rictor CKO mice. These animals did not have any detectable kidney pathology. Finally, we examined primary cilia in the DCT. Cilia were longer in Tsc1 CKO mice, and rapamycin treatment returned cilia length to normal. Rictor CKO mice had normal cilia in the DCT. Overall, our findings suggest that loss of the Tsc1 gene in the DCT is sufficient for renal cystogenesis. This cytogenesis appears to be mTORC1 but not mTORC2 dependent. Intriguingly, the mechanism may be cell autonomous as well as non-cell autonomous and possibly involves the length and function of primary cilia. PMID:22674026

Characterisation of TSC1 promoter deletions in tuberous sclerosis complex patients

NARCIS (Netherlands)

A.M.W. van den Ouweland (Ans)

2011-01-01

textabstractTuberous sclerosis complex (TSC), an autosomal dominant disorder, is a multisystem disease with manifestations in the central nervous system, kidneys, skin and/or heart. Most TSC patients carry a pathogenic mutation in either TSC1 or TSC2. All types of mutations, including large

Characterisation of TSC1 promoter deletions in tuberous sclerosis complex patients

NARCIS (Netherlands)

Ouweland, A.M. van den; Elfferich, P.; Zonnenberg, B.A.; Arts, W.F.M.; Kleefstra, T.; Nellist, M.D.; Millan, J.M.; Withagen-Hermans, C.; Maat-Kievit, A.J.; Halley, D.J.

2011-01-01

Tuberous sclerosis complex (TSC), an autosomal dominant disorder, is a multisystem disease with manifestations in the central nervous system, kidneys, skin and/or heart. Most TSC patients carry a pathogenic mutation in either TSC1 or TSC2. All types of mutations, including large rearrangements,

Cell Type-Specific Contributions to the TSC Neuropathology

Science.gov (United States)

2017-08-01

AWARD NUMBER: W81XWH-16-1-0415 TITLE: Cell Type-Specific Contributions to the TSC Neuropathology PRINCIPAL INVESTIGATOR: Gabriella D’Arcangelo...AND SUBTITLE Cell Type-Specific Contributions to the TSC Neuropathology 5a. CONTRACT NUMBER 5b. GRANT NUMBER W81XWH-16-1-0415 5c. PROGRAM...how heterozygous and homozygous Tsc2 mutations affect the development of mutant excitatory neurons as well as other surrounding brain cells , in vivo

Heterozygous inactivation of tsc2 enhances tumorigenesis in p53 mutant zebrafish

Directory of Open Access Journals (Sweden)

Seok-Hyung Kim

2013-07-01

Tuberous sclerosis complex (TSC is a multi-organ disorder caused by mutations of the TSC1 or TSC2 genes. A key function of these genes is to inhibit mTORC1 (mechanistic target of rapamycin complex 1 kinase signaling. Cells deficient for TSC1 or TSC2 have increased mTORC1 signaling and give rise to benign tumors, although, as a rule, true malignancies are rarely seen. In contrast, other disorders with increased mTOR signaling typically have overt malignancies. A better understanding of genetic mechanisms that govern the transformation of benign cells to malignant ones is crucial to understand cancer pathogenesis. We generated a zebrafish model of TSC and cancer progression by placing a heterozygous mutation of the tsc2 gene in a p53 mutant background. Unlike tsc2 heterozygous mutant zebrafish, which never exhibited cancers, compound tsc2;p53 mutants had malignant tumors in multiple organs. Tumorigenesis was enhanced compared with p53 mutant zebrafish. p53 mutants also had increased mTORC1 signaling that was further enhanced in tsc2;p53 compound mutants. We found increased expression of Hif1-α, Hif2-α and Vegf-c in tsc2;p53 compound mutant zebrafish compared with p53 mutant zebrafish. Expression of these proteins probably underlies the increased angiogenesis seen in compound mutant zebrafish compared with p53 mutants and might further drive cancer progression. Treatment of p53 and compound mutant zebrafish with the mTORC1 inhibitor rapamycin caused rapid shrinkage of tumor size and decreased caliber of tumor-associated blood vessels. This is the first report using an animal model to show interactions between tsc2, mTORC1 and p53 during tumorigenesis. These results might explain why individuals with TSC rarely have malignant tumors, but also suggest that cancer arising in individuals without TSC might be influenced by the status of TSC1 and/or TSC2 mutations and be potentially treatable with mTORC1 inhibitors.

TSC mobile mining and extraction technology

Energy Technology Data Exchange (ETDEWEB)

Lavender, W.J. [TSC Company Ltd., Calgary, AB (Canada)

2001-11-01

This Power-Point presentation described an innovative mining and extraction technology developed by Calgary-based TSC Company Ltd. that has provided a major breakthrough in bitumen production from mineable oil sands. The presentation described the process and key mechanical components as demonstrated on oil sands leases. It also described the step change in cost structure and profitability. Oil sands mining provide a hugh resource base with no exploration costs and no decline in production. Despite these advantages, oil sands mining faces the challenge of high capital and operating costs and materials handling. Other challenges include the variability of the ore and environmental impacts. This paper described the fundamentals of the new technology called the Tar Sand Combine (TSC), a continuous mining machine, crusher, cyclone, tailings filter and stacker all in one mobile module. Several viewgraphs were included with the presentation to depict the recovery process as successfully demonstrated at a pilot project. Patent is pending on the process and components. The advantages of the TSC are reduced materials handling, and no tailings ponds are generated since tailings remain where they are mined. The final product is clean bitumen. The specifications of a commercial TSC are: 2000 ton/stream hour mining produce 25,000 bpsd bitumen at 12 per cent ore grade; mined ore bitumen recovery is greater than 95 per cent and the availability factor is 85 per cent. It was concluded that the TSC can maximize oil sands reserves, while providing significant cost savings and environmental benefits. 2 tabs., 24 figs.

Eye Involvement in TSC

Science.gov (United States)

... eye involvement. Nonretinal and Retinal Eye Findings Facial angiofibromas may involve the eyelids of individuals with TSC, ... the hamartomas have many blood vessels (as are angiofibromas of the skin). Less than half of the ...

Primary intestinal lymphangiectasia treated with rapamycin in a child with tuberous sclerosis complex (TSC).

Science.gov (United States)

Pollack, Sarah F; Geffrey, Alexandra L; Thiele, Elizabeth A; Shah, Uzma

2015-09-01

Primary intestinal lymphangiectasia (PIL) is a rare protein-losing enteropathy characterized by a congenital malformation of the lymphatic vessels of the small intestine causing insufficient drainage and leakage of lymph fluid. Tuberous sclerosis complex (TSC) is an autosomal dominant genetic disorder characterized by benign hamartomas in multiple organ systems. While the lymphatic system has been implicated in TSC through lymphangioleiomyomatosis (LAM) and lymphedema, this paper reports the first case of PIL in TSC, a female patient with a TSC2 mutation. She developed persistent and significant abdominal distension with chronic diarrhea during her first year of life. Due to lack of treatment options and the involvement of the mTOR pathway in TSC, a trial of an mTOR inhibitor, rapamycin, was initiated. This treatment was highly effective, with improvement in clinical symptoms of PIL as well as abnormal laboratory values including VEGF-C, which was elevated to over seven times the normal upper limit before treatment. This case suggests that PIL is a rare manifestation of TSC, warranting the use of mTOR inhibitors in future studies. © 2015 Wiley Periodicals, Inc.

A novel TSC2 mutation in a Chinese family with tuberous sclerosis ...

Indian Academy of Sciences (India)

1Department of Medical Genetics, College of Basic Medical Science and 2Undergraduate Student Brigade, ... Recent studies in the TSC patients indicated that mutations ... Because of the low back pain he ... tein of 946 amino acids instead of a normal tuberin protein ... In the TSC cohort, more than 90% had skin lesions,.

Assisting a child with tuberous sclerosis complex (TSC): a qualitative deep analysis of parents' experience and caring needs.

Science.gov (United States)

Graffigna, Guendalina; Bosio, Caterina; Cecchini, Isabella

2013-12-06

This study was aimed to explore parents' experience of assisting children affected by tuberous sclerosis complex (TSC) with subependymal giant-cell astrocytoma (SEGA) manifestation, in order to understand their caring needs and expectation of support. The study was designed according to the qualitative method of interpretative description and implied two sequential phases of data collection. The first phase was based on in-depth interviews with 18 Italian caregivers of children living with TSC. The second phase of the research was based on an online forum with 30 caregivers of children living with TSC. 48 Italian caregivers, assisting patients with TSC with SEGA manifestation have been involved in the study. When a TSC diagnosis is made, the whole family is affected psychologically. TSC has a great impact on families' quality of life and on their ability to cope with the disease and support the child's ability to recover and reach an acceptable level of well-being. Caregivers report the experience of losing control and the feeling of loneliness and abandon from the healthcare system. Families of children affected by TSC need targeted psychosocial assistance in order to support patients and caregivers and facilitate their social integration.

Epilepsy in Adults with TSC

Science.gov (United States)

... individual with TSC deserves the best quality of life possible, so they should work with the caregivers to reach a balance between seizure control and side effects of the medication. Treatment Options There are many ...

High-throughput drug screen identifies chelerythrine as a selective inducer of death in a TSC2-null setting.

Science.gov (United States)

Medvetz, Doug; Sun, Yang; Li, Chenggang; Khabibullin, Damir; Balan, Murugabaskar; Parkhitko, Andrey; Priolo, Carmen; Asara, John M; Pal, Soumitro; Yu, Jane; Henske, Elizabeth P

2015-01-01

Tuberous sclerosis complex (TSC) is an autosomal dominant syndrome associated with tumors of the brain, heart, kidney, and lung. The TSC protein complex inhibits the mammalian or mechanistic target of rapamycin complex 1 (mTORC1). Inhibitors of mTORC1, including rapamycin, induce a cytostatic response in TSC tumors, resulting in temporary disease stabilization and prompt regrowth when treatment is stopped. The lack of TSC-specific cytotoxic therapies represents an important unmet clinical need. Using a high-throughput chemical screen in TSC2-deficient, patient-derived cells, we identified a series of molecules antagonized by rapamycin and therefore selective for cells with mTORC1 hyperactivity. In particular, the cell-permeable alkaloid chelerythrine induced reactive oxygen species (ROS) and depleted glutathione (GSH) selectively in TSC2-null cells based on metabolic profiling. N-acetylcysteine or GSH cotreatment protected TSC2-null cells from chelerythrine's effects, indicating that chelerythrine-induced cell death is ROS dependent. Induction of heme-oxygenase-1 (HMOX1/HO-1) with hemin also blocked chelerythrine-induced cell death. In vivo, chelerythrine inhibited the growth of TSC2-null xenograft tumors with no evidence of systemic toxicity with daily treatment over an extended period of time. This study reports the results of a bioactive compound screen and the identification of a potential lead candidate that acts via a novel oxidative stress-dependent mechanism to selectively induce necroptosis in TSC2-deficient tumors. This study demonstrates that TSC2-deficient tumor cells are hypersensitive to oxidative stress-dependent cell death, and provide critical proof of concept that TSC2-deficient cells can be therapeutically targeted without the use of a rapalog to induce a cell death response. ©2014 American Association for Cancer Research.

Assisting a child with tuberous sclerosis complex (TSC): a qualitative deep analysis of parents’ experience and caring needs

Science.gov (United States)

Graffigna, Guendalina; Bosio, Caterina; Cecchini, Isabella

2013-01-01

Objective This study was aimed to explore parents’ experience of assisting children affected by tuberous sclerosis complex (TSC) with subependymal giant-cell astrocytoma (SEGA) manifestation, in order to understand their caring needs and expectation of support. Setting and procedure The study was designed according to the qualitative method of interpretative description and implied two sequential phases of data collection. The first phase was based on in-depth interviews with 18 Italian caregivers of children living with TSC. The second phase of the research was based on an online forum with 30 caregivers of children living with TSC. Participants 48 Italian caregivers, assisting patients with TSC with SEGA manifestation have been involved in the study. Results When a TSC diagnosis is made, the whole family is affected psychologically. TSC has a great impact on families’ quality of life and on their ability to cope with the disease and support the child's ability to recover and reach an acceptable level of well-being. Caregivers report the experience of losing control and the feeling of loneliness and abandon from the healthcare system. Conclusion and practice implications Families of children affected by TSC need targeted psychosocial assistance in order to support patients and caregivers and facilitate their social integration. PMID:24319280

Biomarkers of Renal Tumor Burden and Progression in TSC

Science.gov (United States)

2013-09-01

implications. Standard of care should include routine genotyping and regular creatinine measurement in all TSC patients to help guide clinical...pressure, serum chemistries, urinalysis and urine chemistries). (B) Measure soluble growth factors, angiogenesis factors and renal injury molecules in...ELISA was unacceptably limited compared to the standard curve. This limitation restricts its ability to discriminate between samples from TSC patients

TSC response of irradiated CVD diamond films

CERN Document Server

Borchi, E; Bucciolini, M; Guasti, A; Mazzocchi, S; Pirollo, S; Sciortino, S

1999-01-01

CVD diamond films have been irradiated with electrons, sup 6 sup 0 Co photons and protons in order to study the dose response to exposure to different particles and energies and to investigate linearity with dose. The Thermally Stimulated Current (TSC) has been studied as a function of the dose delivered to polymethilmetacrilate (PMMA) in the range from 1 to 12 Gy with 20 MeV electrons from a linear accelerator. The TSC spectrum has revealed the presence of two components with peak temperatures of about 470 and 520 K, corresponding to levels lying in the diamond band gap with activation energies of the order of 0.7 - 1 eV. After the subtraction of the exponential background the charge emitted during the heating scan has been evaluated and has been found to depend linearly on the dose. The thermally emitted charge of the CVD diamond films has also been studied using different particles. The samples have been irradiated with the same PMMA dose of about 2 Gy with 6 and 20 MeV electrons from a Linac, sup 6 sup 0 ...

The Role of TSC Proteins in Regulating Cell Adhesion and Motility

National Research Council Canada - National Science Library

Krymskaya, Vera P

2006-01-01

The goal of this project was to define the molecular signaling mechanisms by which TSCI and TSC2 proteins regulate cell adhesion and motility as it relates to the genetic disorder tuberous sclerosis complex (TSC...

Involvement of TSC genes and differential expression of other members of the mTOR signaling pathway in oral squamous cell carcinoma

International Nuclear Information System (INIS)

Chakraborty, Sanjukta; Mohiyuddin, SM Azeem; Gopinath, KS; Kumar, Arun

2008-01-01

Despite extensive research, the five-year survival rate of oral squamous cell carcinoma (OSCC) patients has not improved. Effective treatment of OSCC requires the identification of molecular targets and signaling pathways to design appropriate therapeutic strategies. Several genes from the mTOR signaling pathway are known to be dysregulated in a wide spectrum of cancers. However, not much is known about the involvement of this pathway in tumorigenesis of OSCC. We therefore investigated the role of the tumor suppressor genes, TSC1 and TSC2, and other members of this pathway in tumorigenesis of OSCC. Expression of genes at the RNA and protein levels was examined by semi-quantitative RT-PCR and western blot analyses, respectively. Loss of heterozygosity was studied using matched blood and tumor DNA samples and microsatellite markers from the TSC1, TSC2 and PTEN candidate regions. The effect of promoter methylation on TSC gene expression was studied by treating cells with methyltransferase inhibitor 5-azacytidine. Methylation status of the TSC2 promoter in tissue samples was examined by combined bisulfite restriction analysis (COBRA). The semi-quantitative RT-PCR analysis showed downregulation of TSC1, TSC2, EIF4EBP1 and PTEN, and upregulation of PIK3C2A, AKT1, PDPK1, RHEB, FRAP1, RPS6KB1, EIF4E and RPS6 in tumors. A similar observation was made for AKT1 and RPS6KB1 expression in tumors at the protein level. Investigation of the mechanism of downregulation of TSC genes identified LOH in 36.96% and 39.13% of the tumors at the TSC1 and TSC2 loci, respectively. No mutation was found in TSC genes. A low LOH rate of 13% was observed at the PTEN locus. Treatment of an OSCC cell line with the methyltransferase inhibitor 5-azacytidine showed a significant increase in the expression of TSC genes, suggesting methylation of their promoters. However, the 5-azacytidine treatment of non-OSCC HeLa cells showed a significant increase in the expression of the TSC2 gene only. In order

Tsc2 Haploinsufficiency Has Limited Effects on Fetal Brain Cytokine Levels during Gestational Immune Activation

Directory of Open Access Journals (Sweden)

Dan Ehninger

2014-01-01

Full Text Available Dysregulated TSC/mTOR signaling may play a pathogenetic role in forms of syndromic autism, such as autism associated with tuberous sclerosis, a genetic disorder caused by heterozygous TSC1 or TSC2 mutations. Environmental risk factors, such as gestational viral infections, may, in some cases, also contribute to the pathogenesis of autism and related neuropsychiatric disorders. We have recently found that a heterozygous Tsc2 mutation and the poly I:C model of maternal immune activation (MIA interactively perturb fetal development and adult social behavior in mice, suggesting that these factors converge on shared pathways. TSC/mTOR signaling plays an important role in the modulation of immune responses, raising the possibility that the damage caused by MIA was greater in Tsc2+/− than in wildtype fetuses because of an exacerbated immune response in the mutants. Here, cytokine antibody arrays were employed to measure relative cytokine abundances in the fetal brain and the placenta during MIA. Cytokines were induced by gestational poly I:C but there was no obvious modulatory effect of Tsc2 haploinsufficiency. The data indicate that cytokine exposure during MIA is comparable in Tsc2 haploinsufficient and wildtype control fetuses, suggesting that downstream molecular and cellular processes may account for the interactive effects of Tsc2 haploinsufficiency and MIA.

Impaired lipid accumulation in the liver of Tsc2-heterozygous mice during liver regeneration

Energy Technology Data Exchange (ETDEWEB)

Obayashi, Yoko, E-mail: youko_oobayashi@ajinomoto.com [Department of Pathology, University of Washington School of Medicine, Seattle, WA (United States); Campbell, Jean S.; Fausto, Nelson [Department of Pathology, University of Washington School of Medicine, Seattle, WA (United States); Yeung, Raymond S. [Department of Surgery, University of Washington School of Medicine, Seattle, WA (United States)

2013-07-19

Highlights: •Tuberin phosphorylation correlated with mTOR activation in early liver regeneration. •Liver regeneration in the Tsc2+/− mice was not enhanced. •The Tsc2+/− livers failed to accumulate lipid bodies during liver regeneration. •Mortality rate increased in Tsc2+/− mice after partial hepatectomy. •Tuberin plays a critical role in hepatic lipid accumulation to support regeneration. -- Abstract: Tuberin is a negative regulator of mTOR pathway. To investigate the function of tuberin during liver regeneration, we performed 70% hepatectomy on wild-type and Tsc2+/− mice. We found the tuberin phosphorylation correlated with mTOR activation during early liver regeneration in wild-type mice. However, liver regeneration in the Tsc2+/− mice was not enhanced. Instead, the Tsc2+/− livers failed to accumulate lipid bodies, and this was accompanied by increased mortality. These findings suggest that tuberin plays a critical role in liver energy balance by regulating hepatocellular lipid accumulation during early liver regeneration. These effects may influence the role of mTORC1 on cell growth and proliferation.

Deficiency of the Erc/mesothelin gene ameliorates renal carcinogenesis in Tsc2 knockout mice.

Science.gov (United States)

Zhang, Danqing; Kobayashi, Toshiyuki; Kojima, Tetsuo; Kanenishi, Kenji; Hagiwara, Yoshiaki; Abe, Masaaki; Okura, Hidehiro; Hamano, Yoshitomo; Sun, Guodong; Maeda, Masahiro; Jishage, Kou-ichi; Noda, Tetsuo; Hino, Okio

2011-04-01

Genetic crossing experiments were performed between tuberous sclerosis-2 (Tsc2) KO and expressed in renal carcinoma (Erc) KO mice to analyze the function of the Erc/mesothelin gene in renal carcinogenesis. We found the number and size of renal tumors were significantly less in Tsc2+/-;Erc-/- mice than in Tsc2+/-;Erc+/+ and Tsc2+/-;Erc+/- mice. Tumors from Tsc2+/-;Erc-/- mice exhibited reduced cell proliferation and increased apoptosis, as determined by proliferating cell nuclear antigen (Ki67) and TUNEL analysis, respectively. Adhesion to collagen-coated plates in vitro was enhanced in Erc-restored cells and decreased in Erc-suppressed cells with siRNA. Tumor formation by Tsc2-deficient cells in nude mice was remarkably suppressed by stable knockdown of Erc with shRNA. Western blot analysis showed that the phosphorylation of focal adhesion kinase, Akt and signal transducer and activator of transcription protein 3 were weaker in Erc-deficient/suppressed cells compared with Erc-expressed cells. These results indicate that deficiency of the Erc/mesothelin gene ameliorates renal carcinogenesis in Tsc2 KO mice and inhibits the phosphorylation of several kinases of cell adhesion mechanism. This suggests that Erc/mesothelin may have an important role in the promotion and/or maintenance of carcinogenesis by influencing cell-substrate adhesion via the integrin-related signal pathway. © 2011 Japanese Cancer Association.

Novel Mutation in the TSC2 Gene Associated with Prenatally Diagnosed Cardiac Rhabdomyomas and Cerebral Tuberous Sclerosis

Directory of Open Access Journals (Sweden)

Chih-Ping Chen

2006-01-01

Full Text Available Cardiac rhabdomyomas are prenatal echocardiographic markers for tuberous sclerosis complex (TSC. TSC is caused by mutations in the genes TSC1 and TSC2. We report a 28-year-old, gravida 5, para 2, woman with an uncomplicated pregnancy until prenatal ultrasound at 34 weeks' gestation revealed fetal cardiac tumors. Ultrafast magnetic resonance imaging (MRI at 36 weeks' gestation showed cardiac rhab-domyomas and small subependymal tubers. At 39 weeks' gestation, a 2262 g female infant was delivered uneventfully. Postnatal echocardiography confirmed cardiac rhabdomyomas and MRI verified small cerebral subependymal tubers. Mutational analysis of TSC1 and TSC2 genes using denaturing high-performance liquid chromatography and direct sequencing of the genes was performed and revealed that the parents had wildtype DNA, while the proband was heterozygous for a novel de novo nonsense mutation, c.4830 G > A, in exon 36 of the TSC2 gene, resulting in a change of codon 1610 TGG (tryptophan to TGA (stop codon. The mutation predicted a W1610X premature termination of the tuberin protein. These findings support an association between a TSC2 de novo nonsense mutation and prenatally detected cardiac rhabdomyomas and cerebral tuberous sclerosis. Familial molecular analysis of TSC1 and TSC2 in cases with prenatally diagnosed cardiac rhabdomyomas and cerebral tuberous sclerosis lesions is helpful in prenatal diagnosis and genetic counseling.

Identification of NPM and DDX5 as Therapeutic Targets in TSC

Science.gov (United States)

2017-12-01

Medical Research and Materiel Command Fort Detrick, Maryland 21702-5012 DISTRIBUTION STATEMENT: Approved for Public Release; Distribution Unlimited...9. SPONSORING / MONITORING AGENCY NAME(S) AND ADDRESS(ES) 10. SPONSOR/MONITOR’S ACRONYM(S) U.S. Army Medical Research and Materiel Command Fort...complexes in TSC cells. 15. SUBJECT TERMS NPM, DDX5, TSC, chemical library , split-luciferase 16. SECURITY CLASSIFICATION OF: 17. LIMITATION OF ABSTRACT

TSC1/2 mutations define a molecular subset of HCC with aggressive behaviour and treatment implication.

Science.gov (United States)

Ho, Daniel W H; Chan, Lo K; Chiu, Yung T; Xu, Iris M J; Poon, Ronnie T P; Cheung, Tan T; Tang, Chung N; Tang, Victor W L; Lo, Irene L O; Lam, Polly W Y; Yau, Derek T W; Li, Miao X; Wong, Chun M; Ng, Irene O L

2017-08-01

We investigated the mutational landscape of mammalian target of rapamycin (mTOR) signalling cascade in hepatocellular carcinomas (HCCs) with chronic HBV background, aiming to evaluate and delineate mutation-dependent mechanism of mTOR hyperactivation in hepatocarcinogenesis. We performed next-generation sequencing on human HCC samples and cell line panel. Systematic mutational screening of mTOR pathway-related genes was undertaken and mutant genes were evaluated based on their recurrence. Protein expressions of tuberous sclerosis complex (TSC)1, TSC2 and pRPS6 were assessed by immunohistochemistry in human HCC samples. Rapamycin sensitivity was estimated by colony-formation assay in HCC cell lines and the treatment was further tested using our patient-derived tumour xenograft (PDTX) models. We identified and confirmed multiple mTOR components as recurrently mutated in HBV-associated HCCs. Of significance, we detected frequent (16.2%, n=18/111) mutations of TSC1 and TSC2 genes in the HCC samples. The spectrum of TSC1/2 mutations likely disrupts the endogenous gene functions in suppressing the downstream mTOR activity through different mechanisms and leads to more aggressive tumour behaviour. Mutational disruption of TSC1 and TSC2 was also observed in HCC cell lines and our PDTX models. TSC -mutant cells exhibited reduced colony-forming ability on rapamycin treatment. With the use of biologically relevant TSC2 -mutant PDTXs, we demonstrated the therapeutic benefits of the hypersensitivity towards rapamycin treatment. Taken together, our findings suggest the significance of previously undocumented mutation-dependent mTOR hyperactivation and frequent TSC1/2 mutations in HBV-associated HCCs. They define a molecular subset of HCC having genetic aberrations in mTOR signalling, with potential significance of effective specific drug therapy. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

Comparison of three rapamycin dosing schedules in A/J Tsc2+/- mice and improved survival with angiogenesis inhibitor or asparaginase treatment in mice with subcutaneous tuberous sclerosis related tumors

Directory of Open Access Journals (Sweden)

Dabora Sandra L

2010-02-01

preclinical studies. The rapamycin dosing comparison study indicates that the duration of rapamycin treatment is more important than dose intensity. We also found that angiogenesis inhibitors and asparaginase reduce tumor growth in a TSC2 tumor mouse model and although these drugs are not as effective as rapamycin, these drug classes may have some therapeutic potential in the treatment of TSC related tumors.

Development of a TSC-Setup for the Characterization of Electron and Hole Traps in Irradiated Silicon Sensors

CERN Document Server

Stricker, Miriam

2015-01-01

For the characterization of radiation damage in silicon detectors a low-noise TSC-Setup was built to analyze charge trapping in the temperature range between 15 K and 250 K. The setup offers the possibility to perform Thermally Stimulated Current (TSC) and IV measurements and also to anneal samples at temperatures up to 180 ◦C. A first annealing study on a proton irradiated silicon pad sensor was performed. This study focuses mainly on the variation of the trap concentration and the results are compared to literature.

Results of ionospheric parameters measurements during injections of exhaust streams of TSC "Progress" OMS

Science.gov (United States)

Khakhinov, Vitaly; Alsatkin, Sergey; Medvedev, Andrey; Kushnarev, Dmitriy; Lebedev, Valentin; Potekhin, Alexander; Ratovsky, Konstantin; Shpynev, Boris

Since 2006 we have carried out active space experiments using the transport spacecraft (TSC) "Progress" and the ground-base Radio-Optic Complex of ISTP SB RAS including Irkutsk Incoherent Scatter Radar (IISR). Engine burns of TSC orbital maneuvering subsystem (OMS) were used as a source of ionospheric disturbances and changing radar signature characteristics of TSC. The flight altitudes were about 340 km. The amount of engine exhaust products was varied from 2 to 11 kg. The flow directions relative to IISR and amount of injected exhaust products were changed from flight to flight. The flows directed to IISR were almost parallel to the geomagnetic field line. For these cases the most pronounced effects were observed, the electron density depletion reached 20-40

Development of a Novel NMR-based Rheb GTPase Assay and Molecular Characterization of TSC2 GAP Activity

Science.gov (United States)

2010-05-01

GTPase) that belongs to the Ras superfamily and has homologs in yeast, fungi , slime mold, fruit fly, zebra fish, and mammals (1–3). Ge- netic and...characterization of TSC2 disease mutations affecting its GAP activity (months 9-12) While the final aspects of this task are yet to be completed, we have...domain mutants of TSC2 that we examined affected its enzymatic activ- ity. This method can now be applied to study the function and regulation of other

Characterization of Ninjurin and TSC22 induction after X-irradiation of normal human skin cells

International Nuclear Information System (INIS)

Koike, Manabu; Ninomiya, Yasuharu; Koike, Aki

2008-01-01

The skin is an external organ that is most frequently exposed to radiation. It is important to elucidate the influence of radiation exposure on the skin at the molecular level. To identify radiation-responsive genes in human skin cells, we used microarray technology to examine the effects of irradiation on 641 genes in normal human epidermal keratinocytes at 4 h and 8 h postirradiation with a cytotoxic dose of X-ray (10 Gy). We found that 18 genes were upregulated and 35 genes were downregulated in keratinocytes at 4 h and/or 8 h postirradiation. Ninjurin, whose function remains unknown in keratinocytes, was induced most strongly by X-irradiation. Several known apoptosis-related genes, such as TSC22, were also upregulated. We characterized Ninjurin and TSC22 induction after X-irradiation of normal human skin cells. The induction of the expression of Ninjurin and TSC22 mRNA in keratinocytes following high-dose X-irradiation was confirmed by northern blot analysis. In dermal fibroblasts, Ninjurin, but not TSC22, was induced after X-ray irradiation. The dependence of both gene expression on the status of an apoptosis regulator, p53, was found. In addition, the expression of both mRNA was induced upon treatment with an apoptosis inducer, etoposide. On the other hand, TSC22, but not Ninjurin, was induced and accumulated in keratinocytes upon treatment with an apoptosis inducer, anisomycin. However, in transient expression assay, EYFP-TSC22, as well as EYFP-Ninjurin or EYFP alone, did not induce apoptosis in keratinocytes in contrast to EYFP-GADD45. Taken together, these findings have important implications on the understanding of the mechanism underlying the complex response of skin cells following X-irradiation. (author)

Thermal Scanning Conductometry (TSC) as a General Method for Studying and Controlling the Phase Behavior of Conductive Physical Gels.

Science.gov (United States)

Bielejewski, Michal

2018-01-23

The thermal scanning conductometry protocol is a new approach in studying ionic gels based on low molecular weight gelators. The method is designed to follow the dynamically changing state of the ionogels, and to deliver more information and details about the subtle change of conductive properties with an increase or decrease in the temperature. Moreover, the method allows the performance of long term (i.e. days, weeks) measurements at a constant temperature to investigate the stability and durability of the system and the aging effects. The main advantage of the TSC method over classical conductometry is the ability to perform measurements during the gelation process, which was impossible with the classical method due to temperature stabilization, which usually takes a long time before the individual measurement. It is a well-known fact that to obtain the physical gel phase, the cooling stage must be fast; moreover, depending on the cooling rate, different microstructures can be achieved. The TSC method can be performed with any cooling/heating rate that can be assured by the external temperature system. In our case, we can achieve linear temperature change rates between 0.1 and approximately 10 °C/min. The thermal scanning conductometry is designed to work in cycles, continuously changing between heating and cooling stages. Such an approach allows study of the reproducibility of the thermally reversible gel-sol phase transition. Moreover, it allows the performance of different experimental protocols on the same sample, which can be refreshed to initial state (if necessary) without removal from the measuring cell. Therefore, the measurements can be performed faster, in a more efficient way, and with much higher reproducibility and accuracy. Additionally, the TSC method can be also used as a tool to manufacture the ionogels with targeted properties, like microstructure, with an instant characterization of conductive properties.

TSC plasma halo simulation of a DIII-D vertical displacement episode

International Nuclear Information System (INIS)

Sayer, R.O.; Peng, Y.K.M.; Jardin, S.C.

1993-01-01

A benchmark of the Tokamak Simulation Code (TSC) plasma halo model has been achieved by calibration against a DIII-D vertical displacement episode (VDE) consisting of vertical drift, thermal quench and current quench. With a suitable halo surrounding the main plasma, the TSC predictions are in good agreement with experimental results for the plasma current decay, plasma trajectory, toroidal and poloidal vessel currents, and for the magnetic probe and flux loop values for the entire VDE. Simulations with no plasma halo yield much faster vertical motion and significantly worse agreement with the magnetics and flux loop data than do halo simulations. (author). 12 refs, 13 figs

Early onset epileptic encephalopathy or genetically determined encephalopathy with early onset epilepsy? Lessons learned from TSC

NARCIS (Netherlands)

Curatolo, P.; Aronica, E.; Jansen, A.; Jansen, F.; Kotulska, K.; Lagae, L.; Moavero, R.; Jozwiak, S.

2016-01-01

BACKGROUND: In tuberous sclerosis complex (TSC) a relationship has been shown between early and refractory seizures and intellectual disability. However, it is uncertain whether epilepsy in TSC is simply a marker in infants who are destined to develop an encephalopathic process or if seizures play a

Early onset epileptic encephalopathy or genetically determined encephalopathy with early onset epilepsy? Lessons learned from TSC

NARCIS (Netherlands)

Curatolo, Paolo; Aronica, Eleonora; Jansen, Anna; Jansen, Floor; Kotulska, Katarzyna; Lagae, Lieven; Moavero, Romina; Jozwiak, Sergiusz

2016-01-01

In tuberous sclerosis complex (TSC) a relationship has been shown between early and refractory seizures and intellectual disability. However, it is uncertain whether epilepsy in TSC is simply a marker in infants who are destined to develop an encephalopathic process or if seizures play a causal role

Mechanism of Akt1 inhibition of breast cancer cell invasionreveals a protumorigenic role for TSC2

Energy Technology Data Exchange (ETDEWEB)

Liu, Hong; Radisky, Derek C.; Nelson, Celeste M.; Zhang, Hui; Fata, Jimmie; Roth, Richard A.; Bissell, Mina J.

2006-02-07

Akt1 is frequently upregulated in human tumors, and has been shown to accelerate cell proliferation and to suppress programmed cell death; consequently, inhibiting the activity of Akt1 has been seen as an attractive target for therapeutic intervention. Paradoxically, hyperactivation of the Akt1 oncogene can also prevent the invasive behavior that underlies progression to metastasis. Here we show that overexpression of activated myr-Akt1 in human breast cancer cells phosphorylates and thereby targets the tumor suppressor tuberous sclerosis complex 2 (TSC2) for degradation, leading to reduced Rho-GTPase activity, decreased actin stress fibers and focal adhesions, and reduced motility and invasion. Overexpression of TSC2 rescues the migration phenotype of myr-Akt1-expressing tumor cells, and high levels of TSC2 in breast cancer patients correlate with increased metastasis and reduced survival. These data indicate that the functional properties of genes designated as oncogenes or tumor suppressor genes depends on the context of the cell type and the tissues studied, and suggest the need for caution in designing therapies targeting the function of individual genes in epithelial tissues.

Specific pattern of maturation and differentiation in the formation of cortical tubers in tuberous sclerosis complex (TSC) : Evidence from layer-specific marker expression

NARCIS (Netherlands)

Mühlebner, Angelika; Iyer, Anand M.; Van Scheppingen, Jackelien; Anink, Jasper J.; Jansen, Floor E.; Veersema, Tim J.; Braun, Kees P.; Spliet, Wim G M; Van Hecke, Wim; Söylemezoǧlu, Figen; Feucht, Martha; Krsek, Pavel; Zamecnik, Josef; Bien, Christian G.; Polster, Tilman; Coras, Roland; Blümcke, Ingmar; Aronica, Eleonora

2016-01-01

Background: Tuberous sclerosis complex (TSC) is a multisystem disorder that results from mutations in the TSC1 or TSC2 genes, leading to constitutive activation of the mammalian target of rapamycin (mTOR) signaling pathway. Cortical tubers represent typical lesions of the central nervous system

Heat-Mirror Spectral Profile Optimization for TSC Hybrid Solar Conversion

Science.gov (United States)

Soule, D. E.; Wood, S. E.

1986-09-01

The efficiencies for total solar cogeneration (TSC) can be optimized by tailoring the transparent heat-mirror (HM) beam splitter spectral profile shape. The entire output is cogenerated by photovoltaic/photothermal conversions into three energy forms: electricity (PV) 18V d.c./module, high-temperature heat (HT) 400°C, and low-temperature heat (LT) 70°C. The emphasis is to maximize the high-quality energy sum PV + HT, while maintaining PV at a high level. A generalized 5-parameter double Fermi model for the HM spectral profile is investigated allowing for a wide parametric range and profile asymmetry. The projected optimized TSC conversion efficiencies are: PV = 14.3%, HT = 43.5%, and LT = 42.2% with a high-quality energy sum of PV + HT = 57.8%. The approach to this optimum HM spectral profile by experimental HM films such as ITO are discussed.

Loss of mTOR repressors Tsc1 or Pten has divergent effects on excitatory and inhibitory synaptic transmission in single hippocampal neuron cultures.

Science.gov (United States)

Weston, Matthew C; Chen, Hongmei; Swann, John W

2014-01-01

The Pten and Tsc1 genes both encode proteins that repress mechanistic target of rapamycin (mTOR) signaling. Disruption of either gene in the brain results in epilepsy and autism-like symptoms in humans and mouse models, therefore it is important to understand the molecular and physiological events that lead from gene disruption to disease phenotypes. Given the similar roles these two molecules play in the regulation of cellular growth and the overlap in the phenotypes that result from their loss, we predicted that the deletion of either the Pten or Tsc1 gene from autaptic hippocampal neurons would have similar effects on neuronal morphology and synaptic transmission. Accordingly, we found that loss of either Pten or Tsc1 caused comparable increases in soma size, dendrite length and action potential properties. However, the effects of Pten and Tsc1 loss on synaptic transmission were different. Loss of Pten lead to an increase in both excitatory and inhibitory neurotransmission, while loss of Tsc1 did not affect excitatory neurotransmission and reduced inhibitory transmission by decreasing mIPSC amplitude. Although the loss of Pten or Tsc1 both increased downstream mTORC1 signaling, phosphorylation of Akt was increased in Pten-ko and decreased in Tsc1-ko neurons, potentially accounting for the different effects on synaptic transmission. Despite the different effects at the synaptic level, our data suggest that loss of Pten or Tsc1 may both lead to an increase in the ratio of excitation to inhibition at the network level, an effect that has been proposed to underlie both epilepsy and autism.

Guidance of reactor operators and TSC personnel with the severe accident management guidance under shutdown and low power conditions

International Nuclear Information System (INIS)

Van Haesendonck, M.F.; Prior, R.P.

2000-01-01

The Westinghouse Owners Group Severe Accident Management Guidance (WOG SAMG) was developed between 1991 and 1994. The primary goals for severe accident management that form the basis of the WOG SAMG are to terminate any radioactive releases to the environment; to prevent failure of any containment fission product boundary and to return the plant to a controlled stable condition. The WOG SAMG is primarily a TSC tool for mitigation of low probability core damage events. The philosophy is that control room operators should remain focused on the prevention of core damage, whereas the TSC personnel should concentrate on the mitigation of the severe accident. The symptom based package is built up as a structured process for choosing appropriate actions based on actual plant conditions. No detailed knowledge of severe accident phenomena is required. The scope of the WOG SAMG is limited to severe accidents resulting from initiating events occurring during full power operation. However, a number of studies such as the EdF EPS 1300 Probabilistic Safety Assessment (PSA), the shutdown Probabilistic Risk Assessment (PRA) for Surry, the BERA shutdown PRA for Beznau, the EPRI/ Westinghouse ORAM methodology etc. have shown that the frequency of core damage (a severe accident) during shutdown and low power operation can be of the same order of magnitude as for full power operation. The at-power SAMG is viewed as the resolution of the severe accident issue. Similarly, it is expected that as shutdown PRAs mature, the final resolution of the severe accident issue will lie in SAMG for low power and shutdown operation. Therefore in resolution of this issue, Westinghouse has developed the Shutdown Severe Accident Management Guidance (SSAMG) which gives guidance for both control room and TSC personnel to mitigate a severe accident under shutdown or low power conditions. In the last few years, many LWR plants have been implementing SAMG. In the US, all plants have developed SAMG, and many

Modeling TSC and LAM Using Patient Derived Induced Pluripotent Stem Cells

Science.gov (United States)

2016-10-01

drug screens . We have now made TSC2 deficient human cells using patient induced pluripotent stem cells (iPSCs...Therapeutics.” Canadian Association of Research in Regenerative Medicine (CARRM), Ottawa, ON, CANADA March 7, 2015 • “ Stem cell approaches to model human... Stem cell approaches to model human development and disease.” Australian Regenerative Medicine Institute, Melbourne, Victoria, AUSTRALIA November

Evaluation of the of antibacterial efficacy of polyvinylpyrrolidone (PVP) and tri-sodium citrate (TSC) silver nanoparticles

Science.gov (United States)

Dey, Arindam; Dasgupta, Abhirup; Kumar, Vijay; Tyagi, Aakriti; Verma, Anita Kamra

2015-09-01

We present silver nanoparticles as the new age broad spectrum antibiotic. Siver nanoparticles exhibit unique physical and chemical properties that make them suitable for understanding their biological potential as antimicrobials. In this study, we explored the antibacterial activity of silver nanoparticles (TSC-AgNPs) and silver nanoparticles doped with polyvinylpyrrolidone (PVP-AgNPs) against Gram-negative and Gram-positive bacteria, Escherichia coli (DH5α) and Staphylococcus aureus, (ATCC 13709). Nucleation and growth kinetics during the synthesis process of AgNPs were precisely controlled using citrate (TSC) and further doped with polyvinylpyrrolidone (PVP). This resulted in the formation of two different sized nanoparticles 34 and 54 nm with PDI of 0.426 and 0.643. The physical characterization was done by nanoparticle tracking analysis and scanning electron microscopy, the results of which are in unison with the digital light scattering data. We found the bactericidal effect for both TSC-AgNPs and PVP-AgNPs to be dose-dependent as determined by the minimum inhibitory concentration (MIC) and minimum bactericidal concentration (MBC) against E. coli and S. aureus. Interestingly, we also observed that AgNPs showed enhanced antimicrobial activity with a MIC of 26.75 and 13.48 µg/ml for E. coli and S. aureus, respectively, while MBC for AgNPs are 53.23 and 26.75 µg/ml for E. coli and S. aureus, respectively. Moreover, AgNPs showed increased DNA degradation as observed confirming its higher efficacy as antibacterial agent than the PVP doped AgNPs.

siRNA-mediated Erc gene silencing suppresses tumor growth in Tsc2 mutant renal carcinoma model.

Science.gov (United States)

Imamura, Osamu; Okada, Hiroaki; Takashima, Yuuki; Zhang, Danqing; Kobayashi, Toshiyuki; Hino, Okio

2008-09-18

Silencing of gene expression by small interfering RNAs (siRNAs) is rapidly becoming a powerful tool for genetic analysis and represents a potential strategy for therapeutic product development. However, there are no reports of systemic delivery of siRNAs for stable treatment except short hairpin RNAs (shRNAs). On the other hand, there are many reports of systemic delivery of siRNAs for transient treatment using liposome carriers and others. With regard to shRNAs, a report showed fatality in mice due to oversaturation of cellular microRNA/short hairpin RNA pathways. Therefore, we decided to use original siRNA microspheres instead of shRNA for stable treatment of disease. In this study, we designed rat-specific siRNA sequences for Erc/mesothelin, which is a tumor-specific gene expressed in the Eker (Tsc2 mutant) rat model of hereditary renal cancer and confirmed the efficacy of gene silencing in vitro. Then, by using siRNA microspheres, we found that the suppression of Erc/mesothelin caused growth inhibition of Tsc2 mutant renal carcinoma cells in tumor implantation experiments in mice.

Simulations of ITER disruption and VDE scenarios with TSC and comparison with DINA results

International Nuclear Information System (INIS)

Bandyopadhyay, I.

2008-01-01

Vertical Displacement Events (VDEs) and plasma current disruptions pose one of the major concerns for the lifetime of in-vessel components in ITER, as well as for machine robustness, as large electromagnetic and thermal loads will induced at such events. Hence, accurate modelling of such events is crucial for estimating disruption induced forces. In the past, ITER disruption modelling has been carried out for ITER using the DINA code. However, since predictive simulations of such events depend on a large number of model assumptions, there exists chances of large error bars on the model predictions. As such it is imperative to validate the code results with other models. Towards this objective, we have carried out the VDE and Disruption simulations using the TSC code and the results are compared with the earlier DINA predictions. A detailed electromagnetic model of the ITER vessel, blankets and the first wall components has been created in TSC. In both VDE and disruption cases, the initial plasma is taken as ITER reference scenario 2 end of burn (EOB) specifications with I p = 15 MA, B t = 5 .3 T, e > 8.8 keV, e > = 1.1 x 10 20 m -3 . The plasma current disruption is initiated by dropping the plasma β in 1 msec, so that after the β crash e > = 6 eV, following which the plasma position control is switched off, resulting in a plasma current quench in about 65 msec. On the other hand, in the VDE case, the plasma control is switched off which results in either upward or downward VDE depending on the initial position of the plasma current centroid. In this case the plasma current remains close to 15 MA for a much longer time, about 700 msec in the simulations till the edge safety factor (q) becomes less than 1.5, following which the β is crashed resulting in plasma current quench. Significant differences exist in the DINA and TSC models, for example, even though the plasma current quench rate predicted by the models matches well in till the halo currents start flowing

A time-resolved current method and TSC under vacuum conditions of SEM: Trapping and detrapping processes in thermal aged XLPE insulation cables

Science.gov (United States)

Boukezzi, L.; Rondot, S.; Jbara, O.; Boubakeur, A.

2017-03-01

Thermal aging of cross-linked polyethylene (XLPE) can cause serious concerns in the safety operation in high voltage system. To get a more detailed picture on the effect of thermal aging on the trapping and detrapping process of XLPE in the melting temperature range, Thermal Stimulated Current (TSC) have been implemented in a Scanning Electron Microscope (SEM) with a specific arrangement. The XLPE specimens are molded and aged at two temperatures (120 °C and 140 °C) situated close to the melting temperature of the material. The use of SEM allows us to measure both leakage and displacement currents induced in samples under electron irradiation. The first represents the conduction process of XLPE and the second gives information on the trapping of charges in the bulk of the material. TSC associated to the SEM leads to show spectra of XLPE discharge under thermal stimulation using both currents measured after electron irradiation. It was found that leakage current in the charging process may be related to the physical defects resulting in crystallinity variation under thermal aging. However the trapped charge can be affected by the carbonyl groups resulting from the thermo-oxidation degradation and the disorder in the material. It is evidenced from the TSC spectra of unaged XLPE that there is no detrapping charge under heat stimulation. Whereas the presence of peaks in the TSC spectra of thermally aged samples indicates that there is some amount of trapped charge released by heating. The detrapping behavior of aged XLPE is supported by the supposition of the existence of two trap levels: shallow traps and deep traps. Overall, physico-chemical reactions under thermal aging at high temperatures leads to the enhancement of shallow traps density and changes in range of traps depth. These changes induce degradation of electrical properties of XLPE.

Ballistic Simulation Method for Lithium Ion Batteries (BASIMLIB) Using Thick Shell Composites (TSC) in LS-DYNA

Science.gov (United States)

2016-08-04

BAllistic SImulation Method for Lithium Ion Batteries (BASIMLIB) using Thick Shell Composites (TSC) in LS-DYNA Venkatesh Babu, Dr. Matt Castanier, Dr...and behavior of the cells through experimental and modeling at their crash worthiness laboratory • Most of the simulation work on the batteries are...at a single cell level and gap exists to simulate the batteries at their full pack capacity - Firstly, requires an enormous amount of computational

TSC measurements on proton-irradiated p-type Si-sensors

Energy Technology Data Exchange (ETDEWEB)

Donegani, Elena; Fretwurst, Eckhart; Garutti, Erika; Junkes, Alexandra [University of Hamburg (Germany)

2016-07-01

Thin n{sup +}p Si sensors are potential candidates for coping with neutron equivalent fluences up to 2.10{sup 16} n{sub eq}/cm{sup 2} and an ionizing dose in the order of a few MGy, which are expected e.g. for the HL-LHC upgrade. The aim of the present work is to provide experimental data on radiation-induced defects in order to: firstly, get a deeper understanding of the properties of hadron induced defects, and secondly develop a radiation damage model based on microscopic measurements. Therefore, the outcomes of Thermally Stimulated Current measurements on 200 μm thick Float-Zone (FZ) and Magnetic Czochralski (MCz) diodes will be shown, as a results of irradiation with 23 MeV protons and isothermal annealing. The samples were irradiated in the fluence range (0.3-1).10{sup 14} n{sub eq}/cm{sup 2}, so that the maximal temperature at which the TSC signal is still sharply distinguishable from the dark current is 200 K. In particular, special focus will be given to the defect introduction rate and to the issue of boron removal in p-type silicon. Annealing studies allow to distinguish which defects mainly contribute to the leakage current and which to the space charge, and thus correlate microscopic defects properties with macroscopic sensor properties.

Two-stage clustering (TSC: a pipeline for selecting operational taxonomic units for the high-throughput sequencing of PCR amplicons.

Directory of Open Access Journals (Sweden)

Xiao-Tao Jiang

Full Text Available Clustering 16S/18S rRNA amplicon sequences into operational taxonomic units (OTUs is a critical step for the bioinformatic analysis of microbial diversity. Here, we report a pipeline for selecting OTUs with a relatively low computational demand and a high degree of accuracy. This pipeline is referred to as two-stage clustering (TSC because it divides tags into two groups according to their abundance and clusters them sequentially. The more abundant group is clustered using a hierarchical algorithm similar to that in ESPRIT, which has a high degree of accuracy but is computationally costly for large datasets. The rarer group, which includes the majority of tags, is then heuristically clustered to improve efficiency. To further improve the computational efficiency and accuracy, two preclustering steps are implemented. To maintain clustering accuracy, all tags are grouped into an OTU depending on their pairwise Needleman-Wunsch distance. This method not only improved the computational efficiency but also mitigated the spurious OTU estimation from 'noise' sequences. In addition, OTUs clustered using TSC showed comparable or improved performance in beta-diversity comparisons compared to existing OTU selection methods. This study suggests that the distribution of sequencing datasets is a useful property for improving the computational efficiency and increasing the clustering accuracy of the high-throughput sequencing of PCR amplicons. The software and user guide are freely available at http://hwzhoulab.smu.edu.cn/paperdata/.

Synthesis, antiviral evaluation and molecular docking studies of N4-aryl substituted/unsubstituted thiosemicarbazones derived from 1-indanones as potent anti-bovine viral diarrhea virus agents.

Science.gov (United States)

Soraires Santacruz, María C; Fabiani, Matías; Castro, Eliana F; Cavallaro, Lucía V; Finkielsztein, Liliana M

2017-08-01

A series of N 4 -arylsubstituted thiosemicarbazones derived from 1-indanones and a set of compounds lacking such substitution in the N 4 position of the thiosemicarbazone moiety were synthesized and evaluated for their anti-bovine viral diarrhea virus (BVDV) activity. Among these, derivatives 2 and 15 displayed high activity (EC 50 =2.7±0.4 and 0.7±0.1µM, respectively) as inhibitors of BVDV replication. Novel key structural features related to the anti-BVDV activity were identified by structure-activity relationship (SAR) analysis. In a previous study, the thiosemicarbazone of 5,6-dimethoxy-1-indanone (5,6-TSC) was characterized as a non-nucleoside inhibitor (NNI) of the BVDV RNA-dependent RNA polymerase. In the present work, cross-resistance assays were performed with the most active compounds. Such studies were carried out on 5,6-TSC resistant BVDV (BVDV-TSC r T1) carrying mutations in the viral polymerase. This BVDV mutant was also resistant to compound 15. Molecular docking studies and MM/PBSA calculations were performed to assess the most active derivatives at the 5,6-TSC viral polymerase binding site. The differences in the interaction pattern and the binding affinity of derivative 15 either to the wild type or BVDV-TSC r T1 polymerase were key factors to define the mode of action of this compound. Copyright © 2017 Elsevier Ltd. All rights reserved.

Reassessment of the role of TSC, mTORC1 and microRNAs in amino acids-meditated translational control of TOP mRNAs.

Directory of Open Access Journals (Sweden)

Ilona Patursky-Polischuk

Full Text Available TOP mRNAs encode components of the translational apparatus, and repression of their translation comprises one mechanism, by which cells encountering amino acid deprivation downregulate the biosynthesis of the protein synthesis machinery. This mode of regulation involves TSC as knockout of TSC1 or TSC2 rescued TOP mRNAs translation in amino acid-starved cells. The involvement of mTOR in translational control of TOP mRNAs is demonstrated by the ability of constitutively active mTOR to relieve the translational repression of TOP mRNA upon amino acid deprivation. Consistently, knockdown of this kinase as well as its inhibition by pharmacological means blocked amino acid-induced translational activation of these mRNAs. The signaling of amino acids to TOP mRNAs involves RagB, as overexpression of active RagB derepressed the translation of these mRNAs in amino acid-starved cells. Nonetheless, knockdown of raptor or rictor failed to suppress translational activation of TOP mRNAs by amino acids, suggesting that mTORC1 or mTORC2 plays a minor, if any, role in this mode of regulation. Finally, miR10a has previously been suggested to positively regulate the translation of TOP mRNAs. However, we show here that titration of this microRNA failed to downregulate the basal translation efficiency of TOP mRNAs. Moreover, Drosha knockdown or Dicer knockout, which carries out the first and second processing steps in microRNAs biosynthesis, respectively, failed to block the translational activation of TOP mRNAs by amino acid or serum stimulation. Evidently, these results are questioning the positive role of microRNAs in this mode of regulation.

Experimental study on the drying of natural latex medical gloves

Science.gov (United States)

Chankrachang, Mano; Yongyingsakthavorn, Pisit; Tohsan, Atitaya; Nontakaew, Udomkiat

2018-01-01

The purpose of this research was to study latex film drying at 70 °C using a laboratory drying oven. Two different total solid content (TSC) latex compounds, which 45% TSC and 35% TSC were used. The undried latex films were prepared according to the common procedures used in latex gloves manufacturers, that is, by dry coagulant dipping process. The experimental results such as initial moisture content, the amount of moisture and drying time of latex films in each latex compound formula were determined. After that, the results were projected to calculate on the production capacity expand by 1 million piece/day of natural latex medical gloves. Finally, the rate of moisture entering the latex drying oven and the energy consumption of the drying oven were estimated. The results indicated that when the 35% TSC of latex compound was used. The initial moisture content of latex film was higher than 45% TSC of latex compound about 7%. The drying time of 35% TSC was longer than 45% TSC for 2.5 min and consume more energy about 10%. As a result, the 45% TSC latex compound was the better way to saving energy and managing humidity in the production line. Therefore, it was found to very useful to an approximate design length and size of actual of latex drying oven and the rate of moisture entering the oven as well.

Comparing Effects of TCR and TSC on MHO Distance Protection Setting in 400 kV Algerian Transmission Line

Directory of Open Access Journals (Sweden)

Mohamed ZELLAGUI

2012-11-01

Full Text Available This paper presents a study on the performances of distance relays setting in 400 kV in Eastern Algerian transmission networks at Sonelgaz Group (Algerian company of Electrical and Gas compensated by shunt Flexible AC Transmission System (FACTS. The facts are used for controlling transmission voltage, power flow, reactive power, and damping of power system oscillations in high power transfer levels. The effects of SVC devices i.e. Thyristor Controlled Reactor (TCR and the Thyristor Switched Capacitors (TSC insertion, on the total impedance of a transmission line protected by MHO distance relay are investigated. The modified setting zones protections for three forward zones (Z1, Z2 and Z3 have been calculated in order to improve the performances of distance relay protection and prevent circuit breaker nuisance tripping.

Evaluation of the TSC Dolphin Computer Assisted Instructional System in the Chapter 1 Program of the District of Columbia Public Schools. Final Report 85-9.

Science.gov (United States)

Harris, Carolyn DeMeyer; And Others

Dolphin is a computer-assisted instruction system used to teach and reinforce skills in reading, language arts, and mathematics. An evaluation of this system was conducted to provide information to TSC Division of Houghton Mifflin regarding its effectiveness and possible modifications to the system. The general design of the evaluation was to…

Profile modification computations for LHCD experiments on PBX-M using the TSC/LSC model

International Nuclear Information System (INIS)

Kaita, R.; Ignat, D.W.; Jardin, S.C.; Okabayashi, M.; Sun, Y.C.

1996-01-01

The TSC-LSC computational model of the dynamics of lower hybrid current drive has been exercised extensively in comparison with data from a Princeton Beta Experiment-Modification (PBX-M) discharge where the measured q(0) attained values slightly above unity. Several significant, but plausible, assumptions had to be introduced to keep the computation from behaving pathologically over time, producing singular profiles of plasma current density and q. Addition of a heuristic current diffusion estimate, or more exactly, a smoothing of the rf-driven current with a diffusion-like equation, greatly improved the behavior of the computation, and brought theory and measurement into reasonable agreement. The model was then extended to longer pulse lengths and higher powers to investigate performance to be expected in future PBX-M current profile modification experiments. copyright 1996 American Institute of Physics

Sun exposure causes somatic second-hit mutations and angiofibroma development in tuberous sclerosis complex

Science.gov (United States)

Tyburczy, Magdalena E.; Wang, Ji-an; Li, Shaowei; Thangapazham, Rajesh; Chekaluk, Yvonne; Moss, Joel; Kwiatkowski, David J.; Darling, Thomas N.

2014-01-01

Tuberous sclerosis complex (TSC) is characterized by the formation of tumors in multiple organs and is caused by germline mutation in one of two tumor suppressor genes, TSC1 and TSC2. As for other tumor suppressor gene syndromes, the mechanism of somatic second-hit events in TSC tumors is unknown. We grew fibroblast-like cells from 29 TSC skin tumors from 22 TSC subjects and identified germline and second-hit mutations in TSC1/TSC2 using next-generation sequencing. Eighteen of 22 (82%) subjects had a mutation identified, and 8 of the 18 (44%) subjects were mosaic with mutant allele frequencies of 0 to 19% in normal tissue DNA. Multiple tumors were available from four patients, and in each case, second-hit mutations in TSC2 were distinct indicating they arose independently. Most remarkably, 7 (50%) of the 14 somatic point mutations were CC>TT ultraviolet ‘signature’ mutations, never seen as a TSC germline mutation. These occurred exclusively in facial angiofibroma tumors from sun-exposed sites. These results implicate UV-induced DNA damage as a cause of second-hit mutations and development of TSC facial angiofibromas and suggest that measures to limit UV exposure in TSC children and adults should reduce the frequency and severity of these lesions. PMID:24271014

Different Sex-Based Responses of Gut Microbiota During the Development of Hepatocellular Carcinoma in Liver-Specific Tsc1-Knockout Mice.

Science.gov (United States)

Huang, Rong; Li, Ting; Ni, Jiajia; Bai, Xiaochun; Gao, Yi; Li, Yang; Zhang, Peng; Gong, Yan

2018-01-01

Gut microbial dysbiosis is correlated with the development of hepatocellular carcinoma (HCC). Therefore, analyzing the changing patterns in gut microbiota during HCC development, especially before HCC occurrence, is essential for the diagnosis and prevention of HCC based on gut microbial composition. However, these changing patterns in HCC are poorly understood, especially considering the sex differences in HCC incidence and mortality. Here, with an aim to determine the relationship between gut microbiota and HCC development in both sexes, and to screen potential microbial biomarkers for HCC diagnosis, we studied the changing patterns in the gut microbiota from mice of both sexes with liver-specific knockout of Tsc1 ( LTsc1KO ) that spontaneously developed HCC by 9-10 months of age and compared them to the patterns observed in their wide-type Tsc1 fl/fl cohorts using high-throughput sequencing. Using the LTsc1KO model, we were able to successfully exclude the continuing influence of diet on the gut microbiota. Based on gut microbial composition, the female LTsc1KO mice exhibited gut microbial disorder earlier than male LTsc1KO mice during the development of HCC. Our findings also indicated that the decrease in the relative abundance of anaerobic bacteria and the increase in the relative abundance of facultative anaerobic bacteria can be used as risk indexes of female HCC, but would be invalid for male HCC. Most of the changes in the gut bacteria were different between female and male LTsc1KO mice. In particular, the increased abundances of Allobaculum , Erysipelotrichaceae, Neisseriaceae, Sutterella , Burkholderiales, and Prevotella species have potential for use as risk indicators of female HCC, and the increased abundances of Paraprevotella, Paraprevotellaceae, and Prevotella can probably be applied as risk indicators of male HCC. These relationships between the gut microbiota and HCC discovered in the present study may serve as a platform for the identification

Urban summer heat load. Meteorological data as a proxy for metropolitan biodiversity

Energy Technology Data Exchange (ETDEWEB)

Kappes, Heike [Naturalis Biodiversity Center, Leiden (Netherlands); Katzschner, Lutz [Kassel Univ. (Germany). Environmental Meteorological Dept.; Nowak, Carsten [Senckenberg Gesellschaft fuer Naturforschung, Gelnhausen (Germany). Conservation Genetics Section; Biodiversity and Climate Research Centre (BiK-F), Frankfurt am Main (Germany)

2012-10-15

Regional climate models forecast that the incidence of extreme heat waves will increase in Central Europe, and the associated intensification of urban heat islands causes concerns about human health and biodiversity. We investigated species diversity of terrestrial gastropods across an urban gradient in a metropolitan area. We considered 34 sites of different degrees of urbanization and of different thermal stress classes (TSC) that were derived from a classification of urban heat load based on the physiological equivalent temperature (PET). A total of 31 species were recorded with 0-21 species per site (mean: 4.4 {+-} 5.2 species per site). Alpha diversity was best explained by TSC, bush cover and perennial vegetation cover, and it decreased with increasing thermal stress. Overall, thermal stress predicted snail and slug species richness better than urban cover (ANOVA(analysis of variance); TSC: F = 10.0, p < 0.001; urbanization: F = 3.9, p = 0.018), and the proportional loss in species richness was higher for native species than for introduced species. The results indicate that climatic stress contributes to the impoverishment of biodiversity in urban areas. We propose that TSC and/or PET are useful indicators for environmental stress levels in biodiversity studies in natural and anthropogenically transformed landscapes. (orig.)

Analysis of a Mouse Skin Model of Tuberous Sclerosis Complex.

Directory of Open Access Journals (Sweden)

Yanan Guo

Full Text Available Tuberous Sclerosis Complex (TSC is an autosomal dominant tumor suppressor gene syndrome in which patients develop several types of tumors, including facial angiofibroma, subungual fibroma, Shagreen patch, angiomyolipomas, and lymphangioleiomyomatosis. It is due to inactivating mutations in TSC1 or TSC2. We sought to generate a mouse model of one or more of these tumor types by targeting deletion of the Tsc1 gene to fibroblasts using the Fsp-Cre allele. Mutant, Tsc1ccFsp-Cre+ mice survived a median of nearly a year, and developed tumors in multiple sites but did not develop angiomyolipoma or lymphangioleiomyomatosis. They did develop a prominent skin phenotype with marked thickening of the dermis with accumulation of mast cells, that was minimally responsive to systemic rapamycin therapy, and was quite different from the pathology seen in human TSC skin lesions. Recombination and loss of Tsc1 was demonstrated in skin fibroblasts in vivo and in cultured skin fibroblasts. Loss of Tsc1 in fibroblasts in mice does not lead to a model of angiomyolipoma or lymphangioleiomyomatosis.

Distinctive MRI features of the epileptogenic zone in children with tuberous sclerosis

International Nuclear Information System (INIS)

Jahodova, A.; Krsek, P.; Kyncl, M.; Jezdik, P.; Kudr, M.; Komarek, V.; Jayakar, P.; Miller, I.; Resnick, T.

2014-01-01

Objective: Localization of the epileptogenic zone (EZ) is challenging in children with tuberous sclerosis complex (TSC). We sought to ascertain whether brain MRI could identify the EZ in TSC patients independent of the clinical and diagnostic data. Methods: Presurgical MRI's of 34 children with TSC who underwent epilepsy surgery at Miami Children's Hospital were retrospectively reevaluated by experts blinded to all other data. Changes typical of TSC (tubers, calcifications, cystic changes) and abnormalities of the perituberal cortex typical of focal cortical dysplasia (FCD) (increased cortical thickness, abnormal gyration, transmantle change, gray/white matter junction blurring) were identified and their localization was compared with the resection site. Sensitivity, specificity and accuracy of individual MRI features to localize the EZ were determined and statistically compared between postoperatively seizure-free and non-seizure-free patients as well as clusters of features typical of FCD and TSC. Results: MRI alone correctly localized the resection cavity in all 19 postoperatively seizure-free patients and 12 of 15 non-seizure-free subjects. Sensitivity, specificity and accuracy of MRI features typical of FCD to localize EZ (90%, 96% and 96%, respectively) were superior to those typical of TCS (79%, 75% and 75%, p < 0.0001). Increased cortical thickness and abnormal gyral formation outside tubers occurred only in the resection site. Resection sites were better predicted by MRI in seizure-free than in non-seizure-free patients. Conclusion: Thorough MRI evaluation identifies the EZ in a significant proportion of TSC patients. Epileptogenic regions were mostly characterized by “FCD-like” changes outside cortical tubers. The findings may have important practical consequences for surgical planning in TSC

Distinctive MRI features of the epileptogenic zone in children with tuberous sclerosis

Energy Technology Data Exchange (ETDEWEB)

Jahodova, A., E-mail: a.jagoda@email.cz [Department of Pediatric Neurology, Charles University, Second Medical School, Motol University Hospital, V Uvalu 84, Prague 5 150 06 (Czech Republic); Krsek, P., E-mail: pavel.krsek@post.cz [Department of Pediatric Neurology, Charles University, Second Medical School, Motol University Hospital, V Uvalu 84, Prague 5 150 06 (Czech Republic); Kyncl, M., E-mail: martinkyn@seznam.cz [Department of Radiology, Charles University, Second Medical School, Motol University Hospital, V Uvalu 84, Prague 5 150 06 (Czech Republic); Jezdik, P., E-mail: jezdip1@feld.cvut.cz [Department of Measurement, Faculty of Electric, Czech Technical University Prague, Technicka 2, CZ 166 27 Prague 6 (Czech Republic); Kudr, M., E-mail: mat.kudr@gmail.com [Department of Pediatric Neurology, Charles University, Second Medical School, Motol University Hospital, V Uvalu 84, Prague 5 150 06 (Czech Republic); Komarek, V., E-mail: vladimir.komarek@fnmotol.cz [Department of Pediatric Neurology, Charles University, Second Medical School, Motol University Hospital, V Uvalu 84, Prague 5 150 06 (Czech Republic); Jayakar, P., E-mail: Prasanna.Jayakar@mch.com [Department of Neurology and Comprehensive Epilepsy Program, Brain Institute, Miami Children' s Hospital, 3200 S.W. 60th Court, Miami, FL (United States); Miller, I., E-mail: ian.miller@mchdocs.com [Department of Neurology and Comprehensive Epilepsy Program, Brain Institute, Miami Children' s Hospital, 3200 S.W. 60th Court, Miami, FL (United States); Resnick, T., E-mail: trevor.resnick@mch.com [Department of Neurology and Comprehensive Epilepsy Program, Brain Institute, Miami Children' s Hospital, 3200 S.W. 60th Court, Miami, FL (United States); Department of Neurology, University of Miami Miller School of Medicine, Miami, FL (United States); and others

2014-04-15

Objective: Localization of the epileptogenic zone (EZ) is challenging in children with tuberous sclerosis complex (TSC). We sought to ascertain whether brain MRI could identify the EZ in TSC patients independent of the clinical and diagnostic data. Methods: Presurgical MRI's of 34 children with TSC who underwent epilepsy surgery at Miami Children's Hospital were retrospectively reevaluated by experts blinded to all other data. Changes typical of TSC (tubers, calcifications, cystic changes) and abnormalities of the perituberal cortex typical of focal cortical dysplasia (FCD) (increased cortical thickness, abnormal gyration, transmantle change, gray/white matter junction blurring) were identified and their localization was compared with the resection site. Sensitivity, specificity and accuracy of individual MRI features to localize the EZ were determined and statistically compared between postoperatively seizure-free and non-seizure-free patients as well as clusters of features typical of FCD and TSC. Results: MRI alone correctly localized the resection cavity in all 19 postoperatively seizure-free patients and 12 of 15 non-seizure-free subjects. Sensitivity, specificity and accuracy of MRI features typical of FCD to localize EZ (90%, 96% and 96%, respectively) were superior to those typical of TCS (79%, 75% and 75%, p < 0.0001). Increased cortical thickness and abnormal gyral formation outside tubers occurred only in the resection site. Resection sites were better predicted by MRI in seizure-free than in non-seizure-free patients. Conclusion: Thorough MRI evaluation identifies the EZ in a significant proportion of TSC patients. Epileptogenic regions were mostly characterized by “FCD-like” changes outside cortical tubers. The findings may have important practical consequences for surgical planning in TSC.

Comment on "A dynamic network model of mTOR signaling reveals TSC-independent mTORC2 regulation": building a model of the mTOR signaling network with a potentially faulty tool.

Science.gov (United States)

Manning, Brendan D

2012-07-10

In their study published in Science Signaling (Research Article, 27 March 2012, DOI: 10.1126/scisignal.2002469), Dalle Pezze et al. tackle the dynamic and complex wiring of the signaling network involving the protein kinase mTOR, which exists within two distinct protein complexes (mTORC1 and mTORC2) that differ in their regulation and function. The authors use a combination of immunoblotting for specific phosphorylation events and computational modeling. The primary experimental tool employed is to monitor the autophosphorylation of mTOR on Ser(2481) in cell lysates as a surrogate for mTOR activity, which the authors conclude is a specific readout for mTORC2. However, Ser(2481) phosphorylation occurs on both mTORC1 and mTORC2 and will dynamically change as the network through which these two complexes are connected is manipulated. Therefore, models of mTOR network regulation built using this tool are inherently imperfect and open to alternative explanations. Specific issues with the main conclusion made in this study, involving the TSC1-TSC2 (tuberous sclerosis complex 1 and 2) complex and its potential regulation of mTORC2, are discussed here. A broader goal of this Letter is to clarify to other investigators the caveats of using mTOR Ser(2481) phosphorylation in cell lysates as a specific readout for either of the two mTOR complexes.

Long-term Follow-up Assessing Renal Angiomyolipoma Treatment Patterns, Morbidity, and Mortality : An Observational Study in Tuberous Sclerosis Complex Patients in the Netherlands

NARCIS (Netherlands)

Eijkemans, Marinus J C; van der Wal, Willem; Reijnders, Leida J; Roes, Kit C B; van Waalwijk van Doorn-Khosrovani, Sahar Barjesteh; Pelletier, Corey; Magestro, Matthew; Zonnenberg, Bernard

2015-01-01

BACKGROUND: Long-term data from patients with tuberous sclerosis complex (TSC)-associated renal angiomyolipoma (angiomyolipoma) are limited. STUDY DESIGN: Retrospective observational study. SETTING & PARTICIPANTS: Adult patients with TSC treated at the University Medical Center Utrecht (the

TOSCA - first international registry to address knowledge gaps in the natural history and management of tuberous sclerosis complex.

Science.gov (United States)

Kingswood, John C; Bruzzi, Paolo; Curatolo, Paolo; de Vries, Petrus J; Fladrowski, Carla; Hertzberg, Christoph; Jansen, Anna C; Jozwiak, Sergiusz; Nabbout, Rima; Sauter, Matthias; Touraine, Renaud; O'Callaghan, Finbar; Zonnenberg, Bernard; Crippa, Stefania; Comis, Silvia; d'Augères, Guillaume Beaure; Belousova, Elena; Carter, Tom; Cottin, Vincent; Dahlin, Maria; Ferreira, José Carlos; Macaya, Alfons; Benedik, Mirjana Perkovic; Sander, Valentin; Youroukos, Sotirios; Castellana, Ramon; Ulker, Bulent; Feucht, Martha

2014-11-26

Tuberous sclerosis complex (TSC) is a rare, multisystem, genetic disorder with an estimated prevalence between 1/6800 and 1/15000. Although recent years have seen huge progress in understanding the pathophysiology and in the management of TSC, several questions remain unanswered. A disease registry could be an effective tool to gain more insights into TSC and thus help in the development of improved management strategies. TuberOus SClerosis registry to increase disease Awareness (TOSCA) is a multicentre, international disease registry to assess manifestations, interventions, and outcomes in patients with TSC. Patients of any age diagnosed with TSC, having a documented visit for TSC within the preceding 12 months, or newly diagnosed individuals are eligible. Objectives include mapping the course of TSC manifestations and their effects on prognosis, identifying patients with rare symptoms and co-morbidities, recording interventions and their outcomes, contributing to creation of an evidence-base for disease assessment and therapy, informing further research on TSC, and evaluating the quality of life of patients with TSC. The registry includes a 'core' section and subsections or 'petals'. The 'core' section is designed to record general information on patients' background collected at baseline and updated annually. Subsections will be developed over time to record additional data related to specific disease manifestations and will be updated annually. The registry aimed to enrol approximately 2000 patients from about 250 sites in 31 countries. The initial enrolment period was of 24 months. A follow-up observation period of up to 5 years is planned. A pre-planned administrative analysis of 'core' data from the first 100 patients was performed to evaluate the feasibility of the registry. Results showed a high degree of accuracy of the data collection procedure. Annual interim analyses are scheduled. Results of first interim analysis will be presented subsequent to

Methodology of site studies

International Nuclear Information System (INIS)

Caries, J.C.; Hugon, J.; Grauby, A.

1980-01-01

This methodology consists in an essentially dynamic, estimated and follow-up analysis of the impact of discharges on all the environment compartments, whether natural or not, that play a part in the protection of man and his environment. It applies at two levels, to wit: the choice of site, or the detailed study of the site selected. Two examples of its application will be developed, namely: at the choice of site level in the case of marine sites, and of the detailed study level of the chosen site in that of a riverside site [fr

Study of site layout in the Rokkasho site

International Nuclear Information System (INIS)

Sato, Kazuyoshi; Tamura, Kousaku; Yagenji, Akira; Sekiya, Shigeki; Takahashi, Hideo; Neyatani, Yuzuru; Uehara, Masaharu; Motohashi, Keiichi; Hashimoto, Masayoshi; Ogino, Shunji; Nagamatsu, Nobuhide

2006-03-01

The Final Design Report (FDR) of the International Thermonuclear Experimental Reactor (ITER) was published on July 2001 as a summary of the Engineering Design Activity (EDA). After the EDA, site dependent design has been investigated for the invitation of ITER toward Rokkasho Site (Iyasakadai area) in Aomori prefecture. This report describes the results of site layout of major buildings and structures of ITER in the Rokkasho-Site. The data of the ground near the site and the results of site dependent design in Japan were applied to this study. Through this study, the most appropriate site layout has been constructed with satisfaction of following conditions. (1) Bedrock level at the tokamak complex building is relatively high and it can be reduced the cost of excavation and foundation work. (2) Total amount of excavation soil for site preparation is minimized and the flexibility of the layout is ensured with flat ground level. (3) Accessibility of human and equipments, reduction of noise and vibration to the environment can be obtained. Total length of ducts and piping between buildings in site is minimized. (author)

Tc-99m sulfur colloid spleen imaging following splenic artery and vein resection for pancreas organ donation

International Nuclear Information System (INIS)

Kuni, C.C.; Crass, J.R.; Du Cret, R.P.; Boudreau, R.J.; Loken, M.K.

1987-01-01

The authors retrospectively studied the records and Tc-99m sulfur colloid (TSC) splenic artery and vein resection for donation to HLA-compatible relatives. Of 37 patients with postoperative TSC studies, four had no postoperative splenic abnormalities. Nineteen of the abnormal TSC studies were followed with TSC studies 2 weeks to 14 months later; three showed no change, seven showed improvements,and ten became normal. One patient required splenectomy 2 days after pancreatectomy for splenic infarction; her TSC study showed no uptake. These data suggest that the spleen usually survives splenic artery and vein resection. Absent splenic TSC uptake raises the possibility of splenic infarction but usually improves

Elastic-plastic fracture mechanics study of thermal shock cracking

International Nuclear Information System (INIS)

Hirano, K.; Kobayashi, H.; Nakazawa, H.

1980-01-01

This paper describes thermal shock experiments conducted on a nuclear pressure vessel steel (A533 Grade B Class 1), an AISI304 steel and a tool steel (JIS SKD62) using both a new thermal shock test facility and method. Analysis of their quasi-static thermal stress intensity factors is performed on the basis of linear-elastic fracture mechanics; and a thermal shock fracture toughness value, Ksub(tsc) is evaluated. Then elastic-plastic fracture toughness tests are carried out in the same high temperature range of the thermal shock experiment, and a relation between the stretched zone width, SZW, formed as a result of the fatigue precrack tip plastic blunting and the J-integral is clarified. An elastic-plastic thermal shock fracture toughness value, Jsub(tsc), is evaluated from a critical value of the stretched zone width, SZWsub(tsc), at the initiation of the thermal shock cracking by using the relation between SZW and J. The Jsub(tsc) value is compared with an elastic-plastic fracture toughness value, Jsub(Ic), and the difference between these Jsub(tsc) and Jsub(Ic) values is discussed on the basis of fractography. (author)

Zebrafish model of tuberous sclerosis complex reveals cell-autonomous and non-cell-autonomous functions of mutant tuberin

Directory of Open Access Journals (Sweden)

Seok-Hyung Kim

2011-03-01

Tuberous sclerosis complex (TSC is an autosomal dominant disease caused by mutations in either the TSC1 (encodes hamartin or TSC2 (encodes tuberin genes. Patients with TSC have hamartomas in various organs throughout the whole body, most notably in the brain, skin, eye, heart, kidney and lung. To study the development of hamartomas, we generated a zebrafish model of TSC featuring a nonsense mutation (vu242 in the tsc2 gene. This tsc2vu242 allele encodes a truncated Tuberin protein lacking the GAP domain, which is required for inhibition of Rheb and of the TOR kinase within TORC1. We show that tsc2vu242 is a recessive larval-lethal mutation that causes increased cell size in the brain and liver. Greatly elevated TORC1 signaling is observed in tsc2vu242/vu242 homozygous zebrafish, and is moderately increased in tsc2vu242/+ heterozygotes. Forebrain neurons are poorly organized in tsc2vu242/vu242 homozygous mutants, which have extensive gray and white matter disorganization and ectopically positioned cells. Genetic mosaic analyses demonstrate that tsc2 limits TORC1 signaling in a cell-autonomous manner. However, in chimeric animals, tsc2vu242/vu242 mutant cells also mislocalize wild-type host cells in the forebrain in a non-cell-autonomous manner. These results demonstrate a highly conserved role of tsc2 in zebrafish and establish a new animal model for studies of TSC. The finding of a non-cell-autonomous function of mutant cells might help explain the formation of brain hamartomas and cortical malformations in human TSC.

Socioeconomic Site Study Plan: Draft

International Nuclear Information System (INIS)

1987-07-01

Social and economic issues and concerns of the Deak Smith County site area will be evaluated during site characterization. Effects that the area could experience from a repository project include demographic, economic, community service, fiscal, and social impacts. The Socioeconomic Site Study Plan is designed to provide a strategy to assess the potential for those impacts. The Socioeconomic Site Study Plan is structured to provide an overview of the socioeconomic program requirements, objectives, and activities to be conducted during site characterization. This report will describe the study design and its rationale; data collection, management, and reporting; program schedules and milestones; site study organization and management; and quality assurance issues. 43 refs

Site locality identification study: Hanford Site. Volume II. Data cataloging

International Nuclear Information System (INIS)

1980-07-01

Data compilation and cataloging for the candidate site locality identification study were conducted in order to provide a retrievable data cataloging system for the present siting study and future site evaluation and licensng processes. This task occurred concurrently with and also independently of other tasks of the candidate site locality identification study. Work in this task provided the data utilized primarily in the development and application of screening and ranking processes to identify candidate site localities on the Hanford Site. The overall approach included two steps: (1) data acquisition and screening; and (2) data compilation and cataloging. Data acquisition and screening formed the basis for preliminary review of data sources with respect to their probable utilization in the candidate site locality identification study and review with respect to the level of completeness and detail of the data. The important working assumption was that the data to be used in the study be based on existing and available published and unpublished literature. The data compilation and cataloging provided the basic product of the Task; a retrievable data cataloging system in the form of an annotated reference list and key word index and an index of compiled data. The annotated reference list and key word index are cross referenced and can be used to trace and retrieve the data sources utilized in the candidate site locality identification study

Tools to support important technical decisions during accident conditions

International Nuclear Information System (INIS)

Tenschert, J.; Bergiers, C.

2008-01-01

To handle design basis and beyond design basis accidents with intact reactor core, Nuclear Power Plants are using Emergency Operating Procedures (EOP) that they may have developed based on the generic Westinghouse Emergency Response Guidelines. Even though the EOPs are very directive, some questions are left to external support, i.e. to a team of persons constituting the so-called Technical Support Center (TSC). The Pressurized Water Reactor Owner Group (PWROG, previously Westinghouse Owner Group, WOG) has developed a TSC manual to support this group in their decision making process. Because of the specific and particular design of the Beznau NPP (KKB) Safety Systems, development of a plant-specific TSC manual required a lot of additions compared to the generic material. This plant-specific TSC manual is a helpful tool for the Site Emergency Director (SED) of the KKB to better evaluate issues and potential concerns arising while executing the EOPs. The majority of considered issues are relevant for beyond design basis accidents and external events. (orig.)

Impact of in-home behavioral management versus telephone support to reduce depressive symptoms and perceived stress in Chinese caregivers: results of a pilot study.

Science.gov (United States)

Gallagher-Thompson, Dolores; Gray, Heather L; Tang, Paulette C Y; Pu, Chun Yu; Leung, Laurie Y L; Wang, Peng-Chih; Tse, Collins; Hsu, Shannon; Kwo, Elizabeth; Tong, Hui-Qi; Long, James; Thompson, Larry W

2007-05-01

Recent work has shown that Chinese Americans caring for a family member with dementia experience considerable psychological distress. However, few studies evaluate treatments for them. This study evaluated the efficacy of in-home intervention, based on cognitive behavior therapy principles, to relieve stress and depression in female Chinese American caregivers (CGs). Fifty-five CGs who met inclusion criteria were randomly assigned to a telephone support condition (TSC) or to an in-home behavioral management program (IHBMP) for 4 months. In the TSC, biweekly calls were made and relevant material was mailed. In the IHBMP, specific psychological skills were taught to deal with caregiving stress. CGs were assessed before and after treatment. Outcome measures evaluated overall perceived stress, caregiving-specific stress, and depressive symptoms. CGs in IHBMP were less bothered by caregiving-specific stressors and had lower depression levels than CGs in TSC. There was no difference in overall stress. CGs with low baseline level of self-efficacy for obtaining respite benefited from IHBMP, but showed little improvement in the TSC. CGs with higher self-efficacy benefited from both treatments. This intervention is promising and warrants replication in future studies. Additional research is needed to evaluate longer-term effects and to identify individual differences associated with improvement.

In silico modeling and experimental evidence of coagulant protein interaction with precursors for nanoparticle functionalization.

Science.gov (United States)

Okoli, Chuka; Sengottaiyan, Selvaraj; Arul Murugan, N; Pavankumar, Asalapuram R; Agren, Hans; Kuttuva Rajarao, Gunaratna

2013-10-01

The design of novel protein-nanoparticle hybrid systems has applications in many fields of science ranging from biomedicine, catalysis, water treatment, etc. The main barrier in devising such tool is lack of adequate information or poor understanding of protein-ligand chemistry. Here, we establish a new strategy based on computational modeling for protein and precursor linkers that can decorate the nanoparticles. Moringa oleifera (MO2.1) seed protein that has coagulation and antimicrobial properties was used. Superparamagnetic nanoparticles (SPION) with precursor ligands were used for the protein-ligand interaction studies. The molecular docking studies reveal that there are two binding sites, one is located at the core binding site; tetraethoxysilane (TEOS) or 3-aminopropyl trimethoxysilane (APTES) binds to this site while the other one is located at the side chain residues where trisodium citrate (TSC) or Si60 binds to this site. The protein-ligand distance profile analysis explains the differences in functional activity of the decorated SPION. Experimentally, TSC-coated nanoparticles showed higher coagulation activity as compared to TEOS- and APTES-coated SPION. To our knowledge, this is the first report on in vitro experimental data, which endorses the computational modeling studies as a powerful tool to design novel precursors for functionalization of nanomaterials; and develop interface hybrid systems for various applications.

Site locality identification study: Hanford Site. Volume I. Methodology, guidelines, and screening

International Nuclear Information System (INIS)

1980-07-01

Presented in this report are the results of the site locality identification study for the Hanford Site using a screening process. To enable evaluation of the entire Hanford Site, the screening process was applied to a somewhat larger area; i.e., the Pasco Basin. The study consisted of a series of screening steps that progressively focused on smaller areas which are within the Hanford Site and which had a higher potential for containing suitable repository sites for nuclear waste than the areas not included for further study. Five site localities, designated H-1, H-2, H-3, H-4, H-5 (Figure A), varying in size from approximately 10 to 50 square miles, were identified on the Hanford Site. It is anticipated that each site locality may contain one or more candidate sites suitable for a nuclear waste repository. The site locality identification study began with definition of objectives and the development of guidelines for screening. Three objectives were defined: (1) maximize public health and safety; (2) minimize adverse environmental and socioeconomic impacts; and (3) minimize system costs. The screening guidelines have numerical values that provided the basis for the successive reduction of the area under study and to focus on smaller areas that had a higher likelihood of containing suitable sites

Development testing of the chemical analysis automation polychlorinated biphenyl standard analysis method during surface soils sampling at the David Witherspoon 1630 site

International Nuclear Information System (INIS)

Hunt, M.A.; Klatt, L.N.; Thompson, D.H.

1998-02-01

The Chemical Analysis Automation (CAA) project is developing standardized, software-driven, site-deployable robotic laboratory systems with the objective of lowering the per-sample analysis cost, decreasing sample turnaround time, and minimizing human exposure to hazardous and radioactive materials associated with DOE remediation projects. The first integrated system developed by the CAA project is designed to determine polychlorinated biphenyls (PCB) content in soil matrices. A demonstration and development testing of this system was conducted in conjuction with surface soil characterization activities at the David Witherspoon 1630 Site in Knoxville, Tennessee. The PCB system consists of five hardware standard laboratory modules (SLMs), one software SLM, the task sequence controller (TSC), and the human-computer interface (HCI). Four of the hardware SLMs included a four-channel Soxhlet extractor, a high-volume concentrator, a column cleanup, and a gas chromatograph. These SLMs performed the sample preparation and measurement steps within the total analysis protocol. The fifth hardware module was a robot that transports samples between the SLMs and the required consumable supplies to the SLMs. The software SLM is an automated data interpretation module that receives raw data from the gas chromatograph SLM and analyzes the data to yield the analyte information. The TSC is a software system that provides the scheduling, management of system resources, and the coordination of all SLM activities. The HCI is a graphical user interface that presents the automated laboratory to the analyst in terms of the analytical procedures and methods. Human control of the automated laboratory is accomplished via the HCI. Sample information required for processing by the automated laboratory is entered through the HCI. Information related to the sample and the system status is presented to the analyst via graphical icons

Methodology of site protection studies

International Nuclear Information System (INIS)

Farges, L.

1980-01-01

Preliminary studies preceding building of a nuclear facility aim at assessing the choice of a site and establishing operating and control procedures. These studies are of two types. Studies on the impact of environment on the nuclear facility to be constructed form one type and studies on the impact of nuclear facilities on the environment form the second type. A methodology giving a framework to studies of second type is presented. These studies are undertaken to choose suitable sites for nuclear facilities. After a preliminary selection of a site based on the first estimate, a detailed site study is undertaken. The procedure for this consists of five successive phases, namely, (1) an inquiry assessing the initial state of the site, (2) an initial synthesis of accumulated information for assessing the health and safety consequences of releases, (3) laboratory and field studies simulating the movement of waste products for a quantitative assessment of effects, (4) final synthesis for laying down the release limits and radiological control methods, and (5) conclusions based on comparing the data of final synthesis to the limits prescribed by regulations. These five phases are outlined. Role of periodic reassessments after the facility is in operation for same time is explained. (M.G.B.)

Cofortification of ferric pyrophosphate and citric acid/trisodium citrate into extruded rice grains doubles iron bioavailability through in situ generation of soluble ferric pyrophosphate citrate complexes.

Science.gov (United States)

Hackl, Laura; Cercamondi, Colin I; Zeder, Christophe; Wild, Daniela; Adelmann, Horst; Zimmermann, Michael B; Moretti, Diego

2016-05-01

Iron fortification of rice is a promising strategy for improving iron nutrition. However, it is technically challenging because rice is consumed as intact grains, and ferric pyrophosphate (FePP), which is usually used for rice fortification, has low bioavailability. We investigated whether the addition of a citric acid/trisodium citrate (CA/TSC) mixture before extrusion increases iron absorption in humans from FePP-fortified extruded rice grains. We conducted an iron absorption study in iron-sufficient young women (n = 20), in which each participant consumed 4 different meals (4 mg Fe/meal): 1) extruded FePP-fortified rice (No CA/TSC); 2) extruded FePP-fortified rice with CA/TSC added before extrusion (CA/TSC extruded); 3) extruded FePP-fortified rice with CA/TSC solution added after cooking and before consumption (CA/TSC solution); and 4) nonextruded rice fortified with a FeSO4 solution added after cooking and before consumption (reference). Iron absorption was calculated from erythrocyte incorporation of stable iron isotopes 14 d after administration. In in vitro experiments, we assessed the soluble and dialyzable iron from rice meals in which CA/TSC was added at different preparation stages and from meals with different iron:CA:TSC ratios. Fractional iron absorption was significantly higher from CA/TSC-extruded meals (3.2%) than from No CA/TSC (1.7%) and CA/TSC solution (1.7%; all P solubility and dialyzability were higher in CA/TSC-extruded rice than in rice with No CA/TSC and CA/TSC solution, and solubility increased with higher amounts of added CA and TSC in extruded rice. Iron bioavailability nearly doubled when CA/TSC was extruded with FePP into fortified rice, resulting in iron bioavailability comparable to that of FeSO4 We attribute this effect to an in situ generation of soluble FePP citrate moieties during extrusion and/or cooking because of the close physical proximity of FePP and CA/TSC in the extruded rice matrix. This trial was registered at

Cortical dysplasia and autistic trait severity in children with Tuberous Sclerosis Complex : A clinical epidemiological study

NARCIS (Netherlands)

S.E. Mous (Sabine); I.E. Overwater (Iris); R. Vidal Gato (Rita); J. Duvekot (Jorieke); L.W. ten Hoopen (Leontine); M. Leguin (Maarten); M.C.Y. de Wit (Marie Claire); G.C. Dieleman (Gwen)

2017-01-01

textabstractTuberous Sclerosis Complex (TSC) is characterized by a high prevalence of autism spectrum disorders (ASD). Little is known about the relation between cortical dysplasia and ASD severity in TSC. We assessed ASD severity (using the Autism Diagnostic Observation Scale), tuber and radial

Thermally stimulated currents in α-HgI2 polycrystalline films

International Nuclear Information System (INIS)

Shiu, Y.-T.; Huang, T.-J.; Shih, C.-T.; Su, C.-F.; Lan, S.-M.; Chiu, K.-C.

2007-01-01

A study of thermally stimulated currents (TSC) is applied to α-HgI 2 polycrystalline films grown by physical vapour deposition with various thermal boundary conditions. Five TSC peaks are clearly observed and numerically fitted. The activation energy and the density of the trapping centre that corresponds to each TSC peak are then calculated. Finally, the effects of the deposition conditions on the TSC results are discussed

Yes, but are they happy? Effects of trait self-control on affective well-being and life satisfaction.

Science.gov (United States)

Hofmann, Wilhelm; Luhmann, Maike; Fisher, Rachel R; Vohs, Kathleen D; Baumeister, Roy F

2014-08-01

Does trait self-control (TSC) predict affective well-being and life satisfaction--positively, negatively, or not? We conducted three studies (Study 1: N = 414, 64% female, Mage = 35.0 years; Study 2: N = 208, 66% female, Mage = 25.24 years; Study 3: N = 234, 61% female, Mage = 34.53 years). The key predictor was TSC, with affective well-being and life satisfaction ratings as key outcomes. Potential explanatory constructs including goal conflict, goal balancing, and emotional distress also were investigated. TSC is positively related to affective well-being and life satisfaction, and managing goal conflict is a key as to why. All studies, moreover, showed that the effect of TSC on life satisfaction is at least partially mediated by affect. Study 1's correlational study established the effect. Study 2's experience sampling approach demonstrated that compared to those low in TSC, those high in TSC experience higher levels of momentary affect even as they experience desire, an effect partially mediated through experiencing lower conflict and emotional distress. Study 3 found evidence for the proposed mechanism--that TSC may boost well-being by helping people avoid frequent conflict and balance vice-virtue conflicts by favoring virtues. Self-control positively contributes to happiness through avoiding and dealing with motivational conflict. © 2013 Wiley Periodicals, Inc.

Nuclear site selection studies

International Nuclear Information System (INIS)

Gharib, A.; Zohoorian Izadpanah, A.A.; Iranmanesh, H.

2000-01-01

It is of special importance, especially from the nuclear safety viewpoint, to select suitable sites for different nuclear structures with the considered future activities. Site selection sometimes involves high costs not necessarily for merely selecting of site but for some preliminary measures to be taken so as the site may have the necessary characteristics. The more suitable the natural characteristics of the site for the considered project, the more successful and efficient the project, the lower the project costs and the longer the project operation period. If so, the project will cause the growth of public culture and sustainable socioeconomic development. This paper is the result of the conclusion of numerous massive reports of this activity in the preliminary phase based on theories, practices and the related safety principles on this ground as well as the application of data and information of the past and a glance to the future. The conception of need for a site for medium structures and nuclear research projects and how to perform this process are presented step by step here with a scientific approach to its selection during the investigations. In this study, it is practically described how the site is selected, by determining and defining the characteristics of research and nuclear projects with medium structures and also its fitting to the optimum site. The discovered sites typically involve the best advantages in technical and economic aspects and no particular contrast with the concerned structures

Comparison of Color Fundus Photography, Infrared Fundus Photography, and Optical Coherence Tomography in Detecting Retinal Hamartoma in Patients with Tuberous Sclerosis Complex.

Science.gov (United States)

Bai, Da-Yong; Wang, Xu; Zhao, Jun-Yang; Li, Li; Gao, Jun; Wang, Ning-Li

2016-05-20

A sensitive method is required to detect retinal hamartomas in patients with tuberous sclerosis complex (TSC). The aim of the present study was to compare the color fundus photography, infrared imaging (IFG), and optical coherence tomography (OCT) in the detection rate of retinal hamartoma in patients with TSC. This study included 11 patients (22 eyes) with TSC, who underwent color fundus photography, IFG, and spectral-domain OCT to detect retinal hamartomas. TSC1 and TSC2RESULTS: The mean age of the 11 patients was 8.0 ± 2.1 years. The mean spherical equivalent was -0.55 ± 1.42 D by autorefraction with cycloplegia. In 11 patients (22 eyes), OCT, infrared fundus photography, and color fundus photography revealed 26, 18, and 9 hamartomas, respectively. The predominant hamartoma was type I (55.6%). All the hamartomas that detected by color fundus photography or IFG can be detected by OCT. Among the methods of color fundus photography, IFG, and OCT, the OCT has higher detection rate for retinal hamartoma in TSC patients; therefore, OCT might be promising for the clinical diagnosis of TSC.

The cochaperone BAG3 coordinates protein synthesis and autophagy under mechanical strain through spatial regulation of mTORC1.

Science.gov (United States)

Kathage, Barbara; Gehlert, Sebastian; Ulbricht, Anna; Lüdecke, Laura; Tapia, Victor E; Orfanos, Zacharias; Wenzel, Daniela; Bloch, Wilhelm; Volkmer, Rudolf; Fleischmann, Bernd K; Fürst, Dieter O; Höhfeld, Jörg

2017-01-01

The cochaperone BAG3 is a central protein homeostasis factor in mechanically strained mammalian cells. It mediates the degradation of unfolded and damaged forms of the actin-crosslinker filamin through chaperone-assisted selective autophagy (CASA). In addition, BAG3 stimulates filamin transcription in order to compensate autophagic disposal and to maintain the actin cytoskeleton under strain. Here we demonstrate that BAG3 coordinates protein synthesis and autophagy through spatial regulation of the mammalian target of rapamycin complex 1 (mTORC1). The cochaperone utilizes its WW domain to contact a proline-rich motif in the tuberous sclerosis protein TSC1 that functions as an mTORC1 inhibitor in association with TSC2. Interaction with BAG3 results in a recruitment of TSC complexes to actin stress fibers, where the complexes act on a subpopulation of mTOR-positive vesicles associated with the cytoskeleton. Local inhibition of mTORC1 is essential to initiate autophagy at sites of filamin unfolding and damage. At the same time, BAG3-mediated sequestration of TSC1/TSC2 relieves mTORC1 inhibition in the remaining cytoplasm, which stimulates protein translation. In human muscle, an exercise-induced association of TSC1 with the cytoskeleton coincides with mTORC1 activation in the cytoplasm. The spatial regulation of mTORC1 exerted by BAG3 apparently provides the basis for a simultaneous induction of autophagy and protein synthesis to maintain the proteome under mechanical strain. Copyright © 2016 Elsevier B.V. All rights reserved.

ITER site selection studies in Spain

International Nuclear Information System (INIS)

Medrano, M.; Alejaldre, C.; Doncel, J.; Garcia, A.; Ibarra, A.; Jimenez, J.A.; Sanchez de Mora, M.A.; Alcala, F.; Diez, J.E.; Dominguez, M.; Albisu, F.

2003-01-01

The studies carried out to evaluate and select a candidate site for International Thermonuclear Experimental Reactor (ITER) construction in Spain are presented in this paper. The ITER design, completed in July 2001, considered a number of technical requirements that must be fulfilled by the selected site. Several assumptions concerning the ITER site were made in order to carry on the design before final site selection. In the studies undertaken for ITER site selection in Spain, the referred technical requirements and assumptions were applied across the whole of Spain and two areas were identified as being preferential. These areas are on the Mediterranean coast and are situated in the Catalan and Valencian regions. A comparative evaluation based on technical characteristics for the concrete plots, proposed within the preferential areas, has been done. The result of these studies was the selection of a site that was deemed to be the most competitive--Vandellos (Tarragona)--and it was proposed to the European Commission for detailed studies in order to be considered as a possible European site for ITER construction. Another key factor for hosting ITER in Spain, is the licensing process. The present status is summarised in this paper

An Australian tuberous sclerosis cohort: are surveillance guidelines being met?

NARCIS (Netherlands)

Chopra, M.; Lawson, J.A.; Wilson, M.; Kennedy, S.E.; Taylor, P.; Buckley, M.F.; Wargon, O.; Parasivam, G.; Camphausen, C.; Yates, D.; Mowat, D.

2011-01-01

AIM: This study aims to describe the phenotypic and genotypic characteristics of 45 Australian patients with tuberous sclerosis complex (TSC), to assess risk factors for intellectual disability, to compare patients with TSC1 and TSC2 mutations and to assess adherence to surveillance recommendations.

Site study plan for Transportation, Deaf Smith County Site, Texas: Preliminary draft

International Nuclear Information System (INIS)

1987-06-01

This site study plan describes transportation field studies to be conducted during the characterization of the Deaf Smith County, Texas, site for the US Department of Energy's Salt Repository Project. The studies are needed to identify and assess potential project impacts to transportation infrastructure and systems in the project vicinity and along potential transportation routes to the site across the State of Texas. The studies are also needed to locate and design project transportation facilities, and to evaluate and design impact mitigation. After identifying the transportation information requirements needed to comply with Federal, State, and local regulations and repository program requirements, the site study plan describes the study design and rationale, the field data collection procedures and equipment, the data analysis methods and application of results, the data management strategy, the schedule of field activities, the management of the study, and the study's quality assurance program. The field data collection activities are organized into programs for the characterization of site vicinity rail corridors and highway corridors, characterization of alternative statewide transportation routes, monitoring of site characterization effects on transportation, characterization of aircraft overflight patterns and hazardous material transportation patterns, and assessment of emergency response preparedness along alternative statewide transportation routes. 34 refs., 10 figs., 2 tabs

Motivation toward Physical Exercise and Subjective Wellbeing: The Mediating Role of Trait Self-Control

Directory of Open Access Journals (Sweden)

Walid Briki

2016-10-01

Full Text Available Motivation toward physical exercise (MPE and trait self-control (TSC were identified as key predictors of subjective wellbeing (SWB. However, there has not been any research designed to examine the mediating role of TSC in the relationship between MPE and SWB. The present study utilizes self-determination theory, control-process theory of self-regulation, and theory of multiple pathways of TSC in order to examine whether TSC mediates the relationships of autonomous MPE (A-MPE, controlled MPE (C-MPE, and impersonal MPE (NO-MPE with SWB using structural equation modeling (XLSTAT PLS. Three hundred seventeen adult American individuals (Mage = 32.97, SDage = 11.30, who reported to be regular exercisers, voluntarily answered questionnaires assessing MPE, TSC, and SWB. Correlational analyses revealed positive relationships between A-MPE, TSC, and SWB, and negative relationships of C-MPE and NO-MPE with TSC and SWB. Mediation analyses revealed that TSC mediated the relationships of A-MPE (partial mediation and C-MPE (full mediation with SWB, but did not mediate the relationship between NO-MPE and SWB. The estimates of the quality of the hypothesized model were acceptable (outer model GoF = .935; absolute GoF = .330; relative GoF = .942; inner model GoF = 1.008; R2 = 36.947%. Finally, this study supports the view that MPE can influence SWB through TSC, and incites to pursue the examination of the relationships between self-determined motivation, self-regulation mechanisms, and health-related outcomes.

Imaging features of tuberous sclerosis complex with autosomal-dominant polycystic kidney disease: a contiguous gene syndrome

International Nuclear Information System (INIS)

Back, Susan J.; Andronikou, Savvas; Kilborn, Tracy; Kaplan, Bernard S.; Darge, Kassa

2015-01-01

Genes for tuberous sclerosis complex (TSC) type 2 and autosomal-dominant polycystic kidney disease (ADPKD) type 1 are both encoded over a short segment of chromosome 16. When deletions involve both genes, an entity known as the TSC2/ADPKD1 contiguous gene syndrome, variable phenotypes of TSC and ADPKD are exhibited. This syndrome has not been reviewed in the radiology literature. Unlike renal cysts in TSC, cystic disease in TSC2/ADPKD1 contiguous gene syndrome results in hypertension and renal failure. A radiologist might demonstrate polycystic kidney disease before the patient develops other stigmata of TSC. Conversely, in patients with known TSC, enlarged and polycystic kidneys should signal the possibility of the TSC2/ADPKD1 contiguous gene syndrome and not simply TSC. Distinguishing these diagnoses has implications in prognosis, treatment and genetic counseling. To describe the clinical and imaging findings of tuberous sclerosis complex and polycystic kidney disease in seven pediatric patients. We retrospectively reviewed renal and brain imaging of children and young adults with genetically proven or high clinical suspicion for TSC2/ADPKD1 contiguous gene syndrome. We included seven pediatric patients from two referral institutions. Ages ranged from birth to 21 years over the course of imaging. The mean follow-up period was 9 years 8 months (4 years 6 months to 20 years 6 months). No child progressed to end-stage renal disease during this period. Three patients were initially imaged for stigmata of TSC, three for abdominal distension and one for elevated serum creatinine concentration. All patients developed enlarged, polycystic kidneys. The latest available imaging studies demonstrated that in 12 of the 14 kidneys 50% or more of the parenchyma was ultimately replaced by >15 cysts, resulting in significant cortical thinning. The largest cysts in each kidney ranged from 2.4 cm to 9.3 cm. Echogenic lesions were present in 13 of the 14 kidneys, in keeping with

Imaging features of tuberous sclerosis complex with autosomal-dominant polycystic kidney disease: a contiguous gene syndrome

Energy Technology Data Exchange (ETDEWEB)

Back, Susan J. [The Children' s Hospital of Philadelphia, Department of Radiology, Philadelphia, PA (United States); Andronikou, Savvas [University of the Witwatersrand, Radiology Department, Faculty of Health Sciences, Johannesburg (South Africa); Kilborn, Tracy [University of Cape Town, Red Cross War Memorial Children' s Hospital, Cape Town (South Africa); Kaplan, Bernard S. [The Children' s Hospital of Philadelphia, Division of Nephrology, Philadelphia, PA (United States); University of Pennsylvania, Perelman School of Medicine, Philadelphia, PA (United States); Darge, Kassa [The Children' s Hospital of Philadelphia, Department of Radiology, Philadelphia, PA (United States); University of Pennsylvania, Perelman School of Medicine, Philadelphia, PA (United States)

2015-03-01

Genes for tuberous sclerosis complex (TSC) type 2 and autosomal-dominant polycystic kidney disease (ADPKD) type 1 are both encoded over a short segment of chromosome 16. When deletions involve both genes, an entity known as the TSC2/ADPKD1 contiguous gene syndrome, variable phenotypes of TSC and ADPKD are exhibited. This syndrome has not been reviewed in the radiology literature. Unlike renal cysts in TSC, cystic disease in TSC2/ADPKD1 contiguous gene syndrome results in hypertension and renal failure. A radiologist might demonstrate polycystic kidney disease before the patient develops other stigmata of TSC. Conversely, in patients with known TSC, enlarged and polycystic kidneys should signal the possibility of the TSC2/ADPKD1 contiguous gene syndrome and not simply TSC. Distinguishing these diagnoses has implications in prognosis, treatment and genetic counseling. To describe the clinical and imaging findings of tuberous sclerosis complex and polycystic kidney disease in seven pediatric patients. We retrospectively reviewed renal and brain imaging of children and young adults with genetically proven or high clinical suspicion for TSC2/ADPKD1 contiguous gene syndrome. We included seven pediatric patients from two referral institutions. Ages ranged from birth to 21 years over the course of imaging. The mean follow-up period was 9 years 8 months (4 years 6 months to 20 years 6 months). No child progressed to end-stage renal disease during this period. Three patients were initially imaged for stigmata of TSC, three for abdominal distension and one for elevated serum creatinine concentration. All patients developed enlarged, polycystic kidneys. The latest available imaging studies demonstrated that in 12 of the 14 kidneys 50% or more of the parenchyma was ultimately replaced by >15 cysts, resulting in significant cortical thinning. The largest cysts in each kidney ranged from 2.4 cm to 9.3 cm. Echogenic lesions were present in 13 of the 14 kidneys, in keeping with

Comparative study of low-temperature photoluminescence and thermally stimulated current in quinary Tl{sub 4}Ga{sub 3}InSe{sub 6}S{sub 2} layered crystals

Energy Technology Data Exchange (ETDEWEB)

Gasanly, N.M., E-mail: nizami@metu.edu.tr [Physics Department, Middle East Technical University, 06800 Ankara (Turkey); Virtual International Scientific Research Centre, Baku State University, 1148 Baku (Azerbaijan)

2016-04-15

Photoluminescence (PL) and thermally stimulated current (TSC) spectra of Tl{sub 4}Ga{sub 3}InSe{sub 6}S{sub 2} layered crystals grown by Bridgman method have been studied in the wavelength region of 560–690 nm and the temperature range of 15–45 K (PL) and in the temperature region of 10–90 K (TSC). A broad PL band centered at 620 nm (2.00 eV) was observed at T=15 K. Variations of emission band has been studied as a function of excitation laser intensity in the 0.7–36.5 mW cm{sup −2} range. Radiative transitions from donor level located at 150 meV below the bottom of conduction band to shallow acceptor level located at 10 meV above the top of the valence band were suggested to be responsible for the observed PL band. TSC curve of Tl{sub 4}Ga{sub 3}InSe{sub 6}S{sub 2} crystal exhibited one broad peak at about 41 K. The activation energy of trap level was found to be 11 meV. An energy level diagram showing transitions in the band gap of the crystal was plotted taking into account the results of PL and TSC measurements carried out below room temperature.

Role of the exercises in the training of the technical support center members in Croatia

International Nuclear Information System (INIS)

Jurkovic, I.-A.; Grgic, D.; Skanata, D.

1999-01-01

Since the beginning of 1998, when the modernization of the existing Emergency Plan and Program in Croatia started, lots of things were done and changed. New organizational structure was set up and Technical Support Center (TSC) as a lead technical agency was introduced. Role, obligations and responsibilities of the TSC were defined and appropriate procedures were developed. These activities are more or less the same recommended by the International Atomic Energy Agency (IAEA). As exercises and workshops are important part of all emergency planning and preparedness activities, they also play an important role in out today and future plans. Croatian Off-site Emergency Response Plan (OERP) recognizes three national workshops that are going to be organized every year. The first one was already organized at the beginning of 1998, in February. Second workshop was organized at the beginning of November 1998. Main workshop objective was to train the TSC staff.(author)

Report of JLC site study group

CERN Document Server

Hasegawa, T; Yamashita, S

2003-01-01

This study group selected some good sites for construction of JLC (Electron-Positron Linear Collider) on the basis of investigation of data and field survey. The aims, activity, use of underground of private land, conditions of site, selection of site at present and future, summary and proposal are reported. 9 sites (Hidaka, Kitakami, Murayama, Abukuma, Kitaibaraki, Aichi and Gifu, Takamatsu, Hiroshima and Seburi range) are selected for the construction on the basis of firm ground and 4 sites (Okinawa, Harima, Tsukuba and Mutsuogawara) for development and researches. 9 sites area consists of plutonic rock or old strata of Paleozoic era. Many problems in each site are reported. There are three following proposals; 1) the self-governing communities of the sites have to understand JLC and start to construct it by information, 2) a site evaluation committee consists of specialist of civil engineering, building, social and natural environment and disaster prevention and 3) the vibration test should be carried out ...

Lysosomal regulation of cholesterol homeostasis in tuberous sclerosis complex is mediated via NPC1 and LDL-R.

Science.gov (United States)

Filippakis, Harilaos; Alesi, Nicola; Ogorek, Barbara; Nijmeh, Julie; Khabibullin, Damir; Gutierrez, Catherine; Valvezan, Alexander J; Cunningham, James; Priolo, Carmen; Henske, Elizabeth P

2017-06-13

Tuberous sclerosis complex (TSC) is a multisystem disease associated with hyperactive mTORC1. The impact of TSC1/2 deficiency on lysosome-mediated processes is not fully understood. We report here that inhibition of lysosomal function using chloroquine (CQ) upregulates cholesterol homeostasis genes in TSC2-deficient cells. This TSC2-dependent transcriptional signature is associated with increased accumulation and intracellular levels of both total cholesterol and cholesterol esters. Unexpectedly, engaging this CQ-induced cholesterol uptake pathway together with inhibition of de novo cholesterol synthesis allows survival of TSC2-deficient, but not TSC2-expressing cells. The underlying mechanism of TSC2-deficient cell survival is dependent on exogenous cholesterol uptake via LDL-R, and endosomal trafficking mediated by Vps34. Simultaneous inhibition of lysosomal and endosomal trafficking inhibits uptake of esterified cholesterol and cell growth in TSC2-deficient, but not TSC2-expressing cells, highlighting the TSC-dependent lysosome-mediated regulation of cholesterol homeostasis and pointing toward the translational potential of these pathways for the therapy of TSC.

General siting study 95. Siting of a deep repository for spent nuclear fuel

International Nuclear Information System (INIS)

1995-10-01

General Siting Study 95 is a detailed description of the work carried out to put the siting of a deep repository in a national and regional context. The report is based on SKB's siting factors, which have been applied on a national scale. Different factors of importance or of possible importance for the long-term radiological safety, technology, land and environment as well as society are described and evaluated. This report is the overall description on general siting studies which the government considered that SKB should report in connection with the RD and D Programme 95. 121 refs, 40 figs

General siting study 95. Siting of a deep repository for spent nuclear fuel

Energy Technology Data Exchange (ETDEWEB)

NONE

1995-10-01

General Siting Study 95 is a detailed description of the work carried out to put the siting of a deep repository in a national and regional context. The report is based on SKB`s siting factors, which have been applied on a national scale. Different factors of importance or of possible importance for the long-term radiological safety, technology, land and environment as well as society are described and evaluated. This report is the overall description on general siting studies which the government considered that SKB should report in connection with the RD and D Programme 95. 121 refs, 40 figs.

Altered inhibition in Tuberous Sclerosis and Type IIb cortical dysplasia

Science.gov (United States)

Talos, Delia M.; Sun, Hongyu; Kosaras, Bela; Joseph, Annelise; Folkerth, Rebecca D.; Poduri, Annapurna; Madsen, Joseph R.; Black, Peter M.; Jensen, Frances E.

2012-01-01

Objective The most common neurological symptom of tuberous sclerosis complex (TSC) and focal cortical dysplasia (FCD) is early-life refractory epilepsy. As previous studies have shown enhanced excitatory glutamatergic neurotransmission in TSC and FCD brains, we hypothesized that neurons associated with these lesions may also express altered GABAA receptor (GABAAR)-mediated inhibition. Methods Expression of the GABAAR subunitsα1 and α4, the Na+-K+-2Cl− (NKCC1), and the K+−Cl− (KCC2) transporters in human TSC and FCD Type II specimens were analyzed by Western blot and double label immunocytochemistry. GABAAR responses in dysplastic neurons from a single case of TSC were measured by perforated-patch recording and compared to normal-appearing cortical neurons from a non-TSC epilepsy case. Results TSC and FCD Type IIb lesions demonstrated decreased expression of the GABAAR α1, increased NKCC1 and decreased KCC2 levels. In contrast, FCD Type IIa lesions showed decreased α4, and increased expression of both NKCC1 and KCC2 transporters. Patch clamp recordings from dysplastic neurons in acute slices from TSC tubers demonstrated excitatory GABAAR responses that were significantly attenuated by the NKCC1 inhibitor bumetanide, in contrast to hyperpolarizing GABAAR-mediated currents in normal neurons from non-TSC cortical slices. Interpretation Expression and function of GABAARs in TSC and FCD IIb suggests the relative benzodiazepine insensitivity and more excitatory action of GABA compared to FCD IIa. These factors may contribute to resistance of seizure activity to anticonvulsants that increase GABAergic function, and may justify add-on trials of the NKCC1 inhibitor bumetanide for the treatment of TSC and FCD Type IIb related epilepsy. PMID:22447678

International Journal of Humanistic Studies: Site Map

African Journals Online (AJOL)

International Journal of Humanistic Studies: Site Map. Journal Home > About the Journal > International Journal of Humanistic Studies: Site Map. Log in or Register to get access to full text downloads.

Exploring the role and function of trial steering committees: results of an expert panel meeting.

Science.gov (United States)

Harman, Nicola L; Conroy, Elizabeth J; Lewis, Steff C; Murray, Gordon; Norrie, John; Sydes, Matt R; Lane, J Athene; Altman, Douglas G; Baigent, Colin; Bliss, Judith M; Campbell, Marion K; Elbourne, Diana; Evans, Stephen; Sandercock, Peter; Gamble, Carrol

2015-12-30

The independent oversight of clinical trials, which is recommended by the Medical Research Council (MRC) Guidelines for Good Clinical Practice, is typically provided by an independent advisory Data Monitoring Committee (DMC) and an independent executive committee, to whom the DMC makes recommendations. The detailed roles and function of this executive committee, known as the Trial Steering Committee (TSC), have not previously been studied or reviewed since those originally proposed by the MRC in 1998. An expert panel (n = 7) was convened comprising statisticians, clinicians and trial methodologists with prior TSC experience. Twelve questions about the role and responsibilities of the TSC were discussed by the panel at two full-day meetings. Each meeting was transcribed in full and the discussions were summarised. The expert panel reached agreement on the role of the TSC, to which it was accountable, the membership, the definition of independence, and the experience and training needed. The management of ethical issues, difficult/complex situations and issues the TSC should not ask the DMC to make recommendations on were more difficult to discuss without specific examples, but support existed for further work to help share issues and to provide appropriate training for TSC members. Additional topics discussed, which had not been identified by previous work relating to the DMCs but were pertinent to the role of the TSC, included the following: review of data sharing requests, indemnity, lifespan of the TSC, general TSC administration, and the roles of both the Funder and the Sponsor. This paper presents recommendations that will contribute to the revision and update of the MRC TSC terms of reference. Uncertainty remains in some areas due to the absence of real-life examples; future guidance on these issues would benefit from a repository of case studies. Notably, the role of a patient and public involvement (PPI) contributor was not discussed, and further work is

Nuclear power plant site selection: a case study

International Nuclear Information System (INIS)

Lugasi, Y.; Mehrez, A.; Sinuany-Stern, Z.

1985-01-01

Selecting the site for a nuclear power plant involves the evaluation of numerous criteria and the professional judgment of various experts. The Israel Atomic Energy Commission has been concerned with the problem of selecting a site for a nuclear power station. Previous studies have been performed by the commission to identify potential sites. There were initial screenings where potential sites were chosen according to various minimal criteria and international standards. Only sites that met all the criteria were chosen. A study was made to find the most preferred site among the potential sites that met all the criteria. Two mathematical approaches were used: Keeney's multiattribute utility function and Saaty's eigenvalue prioritization technique. Both models ranked the same site as the most desirable; however, the models differed in their ranking of the other sites

One-site versus two-site phacotrabeculectomy: a prospective randomized study

Directory of Open Access Journals (Sweden)

Moschos MM

2015-08-01

Full Text Available Marilita M Moschos,1 Irini P Chatziralli,2 Michael Tsatsos3 1First Department of Ophthalmology, University of Athens, 2Second Department of Ophthalmology, Ophthalmiatrion Athinon, Athens, Greece; 3Department of Ophthalmology, Cambridge University Hospital NHS, Cambridge, UK Purpose: The purpose of this study is to compare the efficacy and safety of one-site and two-site combined phacotrabeculectomy with foldable posterior chamber intraocular lens implantation.Methods: Thirty-four patients (41 eyes with glaucoma and cataract were randomly assigned to undergo either a one-site (22 eyes or a two-site (19 eyes combined procedure. One-site approach consisted of a standard superior phacotrabeculectomy with a limbus-based conjunctival flap, while two-site approach consisted of a clear cornea phacoemulsification and a separate superior trabeculectomy with a limbus-based conjunctival flap.Results: Mean follow-up period was 54 months (standard deviation [SD] 2.3. Mean preoperative intraocular pressure (IOP in the one-site group was 21.3 mmHg (SD 2.8 and in the two-site group was 21.8 mmHg (SD 3.0 (P>0.1. Mean postoperative IOP significantly decreased in both groups compared to the preoperative level and was 15.6 mmHg (SD 3.5 in the one-site group and 14.9 mmHg (SD 2.7 in the two-site group. Three months later, the difference between the two groups was not statistically significant (P=0.058. The one-site group required significantly more medications than the two-site group (P=0.03. Best-corrected visual acuity (BCVA improved similarly in both groups, but there was less postoperative (induced astigmatism in the two-site group in a marginal statistical level (P=0.058. Intra- and postoperative complications were comparable in the two groups.Conclusion: Both techniques yielded similar results concerning final BCVA and IOP reduction. However, the two-site group had less induced astigmatism and a better postoperative IOP control with less required

Site Response Analysis Using DeepSoil: Case Study of Bangka Site, Indonesia

Energy Technology Data Exchange (ETDEWEB)

Iswanto, Eko Rudi; Yee, Eric [KEPCO International Nuclear Graduate School, Ulsan (Korea, Republic of)

2015-10-15

Indonesia government declared through Act No. 17 year 2007 on the National Long-Term Development Plant Year 2005-2025 and Presidential Decree No. 5 year 2006 on the National Energy Policy (Indonesia 2007; Indonesia 2006), that nuclear energy is stated as a part of the national energy system. In order to undertake the above national policy, National Nuclear Energy Agency of Indonesia, as the promotor for the utilization of nuclear energy will conduct site study, which is a part of infrastructure preparation for NPP construction. Thorough preparation and steps are needed to operate an NPP and it takes between 10 to 15 years from the preliminary study (site selection, financial study, etc.) up to project implementation (manufacturing, construction, commissioning). During project implementation, it is necessary to prepare various documents relevant for permit application such as Safety Evaluation Report for site permit, Preliminary Safety Analysis Report and Environment Impact Assessment Report for construction permit. Considering the continuously increasing electricity energy demand, it is necessary to prepare for alternative NPP sites. The safety requirements of NPP's are stringent; amongst the various requirements is the ability to safely shut down in the wake of a possible earthquake. Ground response analysis of a potential site therefore needs to be carried out, parameter that affect the resistance of an NPP to earthquakes such as peak strain profiles is analysed. The objective of this paper is to analyse the ground response of the selected site for a NPP, using The Mw 7.9 in Sikuai Island, West Sumatra on September 12, 2007 as present input motion. This analysis will be carried out using a ground response analysis program, DeepSoil. In addition to this, an attempt was made to define the site specific input motion characteristics of the selected site for use in DeepSoil (DeepSoil 5.0). A site investigation at the WB site was performed primarily on the PS

Development of hypomelanotic macules is associated with constitutive activated mTORC1 in tuberous sclerosis complex

DEFF Research Database (Denmark)

Møller, Lisbeth Birk; Schönewolf-Greulich, Bitten; Rosengren, Thomas

2017-01-01

TSC1 and TSC2 are genes mutated in the syndrome TSC (tuberous sclerosis complex). We describe a 3-generation family with 17 affected members, all presenting classic TSC features except renal manifestations. The disease segregates with a silent substitution in TSC2, c.4149C>T, p.(Ser1838Ser), whic...

Magnetic resonance imaging and diffusion-weighted imaging of normal-appearing white matter in children and young adults with tuberous sclerosis complex

International Nuclear Information System (INIS)

Arulrajah, Sahayini; Ertan, Gulhan; Tekes, Aylin; Huisman, Thierry A.G.M.; Jordan, Lori; Khaykin, Elizabeth; Izbudak, Izlem

2009-01-01

Patients with tuberous sclerosis complex (TSC) frequently present with neurocognitive deficits which may be related to impaired white matter maturation. The purposes of our study were (a) to evaluate the white matter maturation in children and young adults with TSC by comparing the apparent diffusion coefficient (ADC) values of normal-appearing white matter (NAWM) with age-matched healthy controls and (b) to determine the association of NAWM-ADC values with the severity of neurological symptoms in TSC patients. Twenty-three TSC patients who underwent magnetic resonance imaging/diffusion-weighted imaging between January 2000 and January 2009 were studied. ADC values of NAWM were measured in the frontal, parietal, occipital lobes, and in the pons. ADC data were compared with age-matched normative data derived from healthy controls. Patients were neurologically scored by a pediatric neurologist. Two-sample t tests and linear regression were conducted using STATA software. ADC values of NAWM were higher in TSC patients compared with healthy controls; the increase, however, only reached statistical significance in the frontal white matter and pons in the age group between 96 and 144 months and in the right parietal and occipital white matter in the age group above 144 months. There was no significant change in neurological severity score per unit increase in ADC measurement. ADC values of NAWM appear increased in TSC patients. The abnormal ADC values suggest that myelination may be delayed/impaired in TSC patients, which could explain global neurocognitive deficits. Larger prospective studies, including diffusion tensor imaging, are necessary to validate our results. (orig.)

Magnetic resonance imaging and diffusion-weighted imaging of normal-appearing white matter in children and young adults with tuberous sclerosis complex

Energy Technology Data Exchange (ETDEWEB)

Arulrajah, Sahayini; Ertan, Gulhan; Tekes, Aylin; Huisman, Thierry A.G.M. [Johns Hopkins Hospital, Division of Pediatric Radiology, Department of Radiology and Radiological Science, Baltimore, MD (United States); Jordan, Lori [Johns Hopkins School of Public Health, Division of Pediatric Neurology, Baltimore, MD (United States); Khaykin, Elizabeth [Johns Hopkins School of Public Health, Department of Mental Health, Baltimore, MD (United States); Izbudak, Izlem [Johns Hopkins Hospital, Division of Neuroradiology, Department of Radiology and Radiological Science, Baltimore, MD (United States)

2009-11-15

Patients with tuberous sclerosis complex (TSC) frequently present with neurocognitive deficits which may be related to impaired white matter maturation. The purposes of our study were (a) to evaluate the white matter maturation in children and young adults with TSC by comparing the apparent diffusion coefficient (ADC) values of normal-appearing white matter (NAWM) with age-matched healthy controls and (b) to determine the association of NAWM-ADC values with the severity of neurological symptoms in TSC patients. Twenty-three TSC patients who underwent magnetic resonance imaging/diffusion-weighted imaging between January 2000 and January 2009 were studied. ADC values of NAWM were measured in the frontal, parietal, occipital lobes, and in the pons. ADC data were compared with age-matched normative data derived from healthy controls. Patients were neurologically scored by a pediatric neurologist. Two-sample t tests and linear regression were conducted using STATA software. ADC values of NAWM were higher in TSC patients compared with healthy controls; the increase, however, only reached statistical significance in the frontal white matter and pons in the age group between 96 and 144 months and in the right parietal and occipital white matter in the age group above 144 months. There was no significant change in neurological severity score per unit increase in ADC measurement. ADC values of NAWM appear increased in TSC patients. The abnormal ADC values suggest that myelination may be delayed/impaired in TSC patients, which could explain global neurocognitive deficits. Larger prospective studies, including diffusion tensor imaging, are necessary to validate our results. (orig.)

Site characterization studies in the NWTS program

International Nuclear Information System (INIS)

Shipler, D.; Evans, G.

1980-01-01

The US Department of Energy (DOE) has the responsibility to identify sites and construct and operate facilities for the storage or isolation of spent fuel and/or reprocessing radioactive wastes from commercial nuclear power plants. The National Waste Terminal Storage (NWTS) Program has been initiated by the DOE to develop the technology and demonstrate the feasibility of burial and isolation of high level radioactive waste in deep geologic formations. The NTWS Program plan which sets forth the criteria, procedures, and other considerations required to characterize and select a site in a comprehensive stepwise manner is discussed. The plan is not specific to any given geologic medium but serves as a guide for site selection in any geohydrologic system deemed appropriate for consideration for a deep geologic repository. The plan will be used by all NWTS Project Offices in the conduct of their site characterization program. The plan will be updated, as warranted, to reflect technology development, National policies, rulemakings by regulatory agencies, and other changing political, social, and institutional considerations. Site characterization begins with the identification of regions believed to have suitable geologic, hydrologic, and environmental characteristics for repository siting. This is followed by an iterative process of data collection and analysis to identify areas and locations which appear most suitable for further investigations. In addition, screening studies of the DOE's nuclear complexes has led to the selection of the Nevada Test Site and the Hanford Site for further characterization studies. The site characterization process results in a number of candidate sites from which a site will be selected and proposed to the NRC for licensing

Renal angiomyolipoma bleeding in a patient with TSC2/PKD1 contiguous gene syndrome after 17 years of renal replacement therapy.

Science.gov (United States)

Furlano, Mónica; Barreiro, Yaima; Martí, Teresa; Facundo, Carme; Ruiz-García, César; DaSilva, Iara; Ayasreh, Nadia; Cabrera-López, Cristina; Ballarín, José; Ars, Elisabet; Torra, Roser

We report the case of a 32-year-old male diagnosed with TSC2/PKD1 contiguous gene syndrome, presenting with tuberous sclerosis (TS) and autosomal dominant polycystic kidney disease simultaneously. He progressed to end-stage renal disease and received a kidney transplant at the age of 12. The native kidneys presented angiomyolipomas (AML), which are common benign tumours in patients with TS. Seventeen years after transplantation, he presented with abdominal pain, anaemia and a retroperitoneal haematoma, the latter caused by renal AML bleeding. Selective embolisation was performed. Our patient could have benefited from the administration of mTOR inhibitors at transplant. This therapy is immunosuppressive and reduces the size of benign tumours in TS as well as the risk of rupture and bleeding. This patient did not receive mTOR inhibitors at the time of the transplant because the relationship between mTOR inhibitors and TS was unknown at that time. This case confirms the persistent risk of renal AML bleeding for both transplanted patients and patients on dialysis. As a result, we would recommend routine check-ups of native kidneys and nephrectomy assessment. Copyright © 2017 Sociedad Española de Nefrología. Published by Elsevier España, S.L.U. All rights reserved.

Ecological and radioecological studies of nuclear installation sites

International Nuclear Information System (INIS)

Caries, J. C.; Hugon, J.; Grauby, A.

1988-01-01

The site study method consists of a dynamic and estimated analysis and of following up the release impact on all natural or non natural media compartments that take a part in the protection of man and his environment. The stages of knowing a nuclear site include the site preliminary radioecological evaluation, diffusion parameters evaluation, the quantification of factors of radioelements transfer to man, the ecological baseline carrying out, the radioactive baseline establishment, the radioecological synthesis of the results, the site radioactive and ecological control. This method applys to selection and detailed study of site. 1 tab., 7 refs. (F.M.)

Sternoe study site. Scope of activities and main results

International Nuclear Information System (INIS)

Ahlbom, K.; Andersson, J.E.; Nordqvist, R.; Tiren, S.; Ljunggren, C.; Voss, C.

1992-01-01

During the period from 1977-1986 SKB (Swedish Nuclear Fuel and Waste Management Co) performed surface and borehole investigations of 14 study sites for the purpose of assessing their suitability for a repository of spent nuclear fuel. The next phase in the SKB site selection programme will be to perform detailed characterization, including characterization from shafts and/or tunnels, of two or three sites. The detailed investigations will continue over several years to provide all the data needed for a licensing application to build a repository. Such an application is foreseen to be given to the authorities around the year 2003. It is presently not clear if anyone of the previously investigated study sites will be selected as a site for detailed characterization. Other sites with geological and/or socio-economical characteristics judged more favourable may very well be the ones selected. However, as a part of the background documentation needed for the site selection studies to come, summary reports will be prepared for most study sites. These reports will include scope of activities, main results, uncertainties and need of complementary investigations. This report concerns the Sternoe study site. This site was one of the first sites to be investigated by SKB . The studies at Sternoe were made under severe time-constraints and with prototype borehole instrumentations. These limitations should be kept in mind when reading the report. (41 refs., 16 figs., 12 tabs.) (au)

High gamma-aminobutyric acid level in cortical tubers in epileptic infants with tuberous sclerosis complex measured with the MEGA-editing J-difference method and a three-Tesla clinical MRI Instrument.

Science.gov (United States)

Taki, Masako Minato; Harada, Masafumi; Mori, Kenji; Kubo, Hitoshi; Nose, Ayumi; Matsuda, Tsuyoshi; Nishitani, Hiromu

2009-10-01

The purpose of this study was to estimate the gamma-aminobutyric acid (GABA) and glutamate plus glutamine (Glx) concentrations in the cortical tubers of patients with tuberous sclerosis complex (TSC) using the MEGA-editing J-difference method and a stimulated echo-acquisition mode with a short echo time, and to determine which abnormality was more dominant between GABA and Glx in patients with TSC with epilepsy. This study included six patients with TSC (mean age, 4.3 years) and seven control subjects (mean age, 4.8 years). Measurements were obtained with a three-Tesla apparatus and postprocessing was conducted with an LCModel. The GABA level in the cortical gray matter (cgGABA) was calculated as a result of segmentation in voxels and from the literature values for gray and white matter ratios for GABA. Increased GABA and myo-inositol (mI) concentrations and a decreased N-acetyl aspartate (NAA) concentration were observed in the cortical tubers. The cgGABA level, and cgGABA/NAA and cgGABA/Glx ratios were also higher in patients with TSC than in control subjects. No significant difference was found in Glx concentration between patients with TSC and control subjects. Although the number of patients with TSC in this study was small, the increase in GABA and no significant change in Glx were consistent with previous neurochemical studies and support the hypothesis that brain GABA plays a key role in the pathophysiology of epilepsy during the process of neuronal development.

Gideaa study site. Scope of activities and main results

International Nuclear Information System (INIS)

Ahlbom, K.; Andersson, J.E.; Nordqvist, R.; Ljunggren, C.; Tiren, S.; Voss, C.

1991-10-01

During the period from 1977-1986 SKB (Swedish Nuclear Fuel and Waste Management Co) performed surface and borehole investigations of 14 study sites for the purpose of assessing their suitability for a repository of spent nuclear fuel. The next phase in the SKB site selection programme will be to perform detailed characterization, including characterization from shafts and/or tunnels, of two or three sites. The detailed investigations will continue over several years to provide all the data needed for a licensing application to build a repository. Such an application is foreseen to be given to the authorities around the year 2003. It is presently not clear if anyone of the study sites will be selected as a site for detailed characterization. Other site with geological and/or socio-economical characteristics judged more favourable may very well be the ones selected. However, as a part of the background documentation needed for the site selection studies to come, summary reports will be prepared for most study sites. These reports will include scope of activities, main results, uncertainties and need of complementary investigations. This report concerns the Gideaa study site. (au)

Kamlunge study site. Scope of activities and main results

International Nuclear Information System (INIS)

Ahlbom, K.; Andersson, J.E.; Andersson, P.; Ittner, T.; Tiren, S.; Ljunggren, C.

1992-05-01

During the period from 1977-1986 SKB (Swedish nuclear Fuel and Waste Management Co.) performed surface and borehole investigations of 14 study sites for the purpose of assessing their suitability for a repository of spent nuclear fuel. The next phase in the SKB site selection programme will be to perform detailed characterization, including characterization from shafts and/or tunnels, of two or three sites. The detailed investigations will continue over several years to provide all the data needed for a licensing application to build a repository. Such an application is foreseen to be given to the authorities around the year 2003. It is presently not clear if anyone of the study sites will be selected as a site for detailed characterization. Other sites with geological and/or socio-economical characteristics judged more favourable may very well be selected. However, as a part of the background documentation needed for the site selection studies to come, summary reports will be prepared for most study sites. These reports will include scope of activities, main results, uncertainties and need of complementary investigations. This report concerns the Kamlunge study site. (79 refs.) (au)

Fjaellveden study site. Scope of activities and main results

International Nuclear Information System (INIS)

Ahlbom, K.; Andersson, J.E.; Nordqvist, R.; Ljunggren, C.; Tiren, S.; Voss, C.

1991-10-01

During the period from 1977-1986 SKB (Swedish Nuclear Fuel and Waste Management CO) performed surface and borehole investigations of 14 study sites for the purpose of assessing their suitability for a repository of spent nuclear fuel. The next phase in the SKB site selection programme will be to perform detailed characterization, including characterization from shafts and/or tunnels, of two or three sites. The detailed investigations will continue over several years to provide all the data needed for a licensing application to build repository. Such an application is foreseen to be given to the authorities around the year 2003. It is presently not clear if anyone of the study sites will be selected as a site for detailed characterization. Other sites with geological and/or socio-economical characteristics judged more favourable may very well be the ones selected. However, as a part of the background documentation needed for the site selection studies to come, summary reports will be prepared for most study sites. These reports will include scope of activities, main results, uncertainties and need for complementary investigations. This report concerns the Fjaellveden study site. (au)

Screening and identification of host factors interacting with UL14 of herpes simplex virus 1.

Science.gov (United States)

Wu, Fuqing; Xing, Junji; Wang, Shuai; Li, Meili; Zheng, Chunfu

2011-08-01

The UL14 protein of herpes simplex virus type 1 (HSV-1) is highly conserved in herpesvirus family. However, its exact function during the HSV-1 replication cycle is little known. In the present study, a high throughput yeast two-hybrid system was employed to screen the cellular factors interacting with UL14, and five target candidates were yielded: (1) TSC22 domain family protein 3 (TSC22D3); (2) Mediator of RNA polymerase II transcription subunit 8 isoform 1(MED8); (3) Runt-related transcription factor 3 (RUNX3); (4) Arrestin beta-2 (ARRB2); (5) Cereblon (CRBN). Indirect immunofluorescent assay showed that both TSC22D3 and MED8 co-localized with UL14. Co-immunoprecipitation assay demonstrated that UL14 could be immunoprecipitated by TSC22D3, suggesting that UL14 interacted with TSC22D3 under physiological condition. In summary, this study opened up new avenues toward delineating the function and physiological significance of UL14 during the HSV-1 replication cycle.

[Clinical analysis of 15 pediatric patients with tuberous sclerosis complex complicated by cardiac rhabdomyomas].

Science.gov (United States)

Huang, Guo-Qiang; Zhai, Qiong-Xiang; Yu, Jun-Hao; Wang, Chun; Zhuo, Mu-Qing; Wang, Lin-Gan

2015-05-01

To investigate the clinical features in children with tuberous sclerosis complex (TSC)-associated cardiac rhabdomyomas (CRM). The clinical data of 15 children with TSC complicated by CRM were collected. The clinical features of the patients were analyzed, and TSC gene mutations were detected. Eleven cases (73%) developed multiple CRM. The majority of the tumors were located in the left and right ventricles. Most tumors presented as a round-like hyperechogenic mass with a clear margin on echocardiography. Arrhythmias occurred in 3 patients and 2 patients experienced heart failure. Gene mutation tests were performed in 2 patients, and pathogenic mutations were detected in both patients, which were TSC1 mutation and TSC2 mutation, respectively. Three patients were followed up for 6 to 38 months, and their CRM shrank or regressed spontaneously. TSC-associated CRM is generally multiple. Heart failure and arrhythmias may occur in some patients. Echocardiography is important for diagnosis of CRM. TSC-associated CRM has an inclination to spontaneous regression. TSC can be diagnosed at a molecular genetic level by TSC gene mutation detection.

[Tuberous sclerosis: clinical characteristics and their relationship to genotype/phenotype].

Science.gov (United States)

Monteiro, T; Garrido, C; Pina, S; Chorão, R; Carrilho, I; Figueiroa, S; Santos, M; Temudo, T

2014-11-01

Tuberous sclerosis (TS) is an inherited disorder with multisystemic involvement and a high phenotypic variability. There are two genes that cause this condition: TSC1 and TSC2. Our goal was to clinically characterize patients with TS followed up in the Pediatric Neurology Clinic of a tertiary hospital during the last 10 years, and correlate the genotype with the severity of neurological manifestations and imaging studies. Retrospective analysis of patients with TS, including review of medical records and available MRI imaging. We studied 35 cases with a median age at diagnosis of ten months. Seizures were the first manifestation in 91.4% of cases, with a predominance of epileptic spasms. Over 50% had cognitive impairment and 49% behavioral disorders. A genetic study was performed on 24 children, and TSC2 mutations identified in 58.3% of them. Of the 11 cases of refractory epilepsy, six had the TSC2 gene mutation. In the group of eight patients with moderate/severe cognitive deficits, five had TSC2 mutations. We reviewed 26 MRI scans, in which it was observed that 76.9% had diffuse involvement of cerebral lobes, which reflects a greater burden of injury. Of the patients who had an MRI scan performed and had TSC2 mutations, all had a high tuber load, and5 of them had refractory epilepsy. In our sample we observe a high percentage of mutations in the TSC2 gene. This mutation carries a worse neurological prognosis, with drug-resistant epilepsy and a more severe cognitive impairment. Copyright © 2013 Asociación Española de Pediatría. Published by Elsevier Espana. All rights reserved.

Research trend in thermally stimulated current method for development of materials and devices in Japan

Science.gov (United States)

Iwamoto, Mitsumasa; Taguchi, Dai

2018-03-01

Thermally stimulated current (TSC) measurement is widely used in a variety of research fields, i.e., physics, electronics, electrical engineering, chemistry, ceramics, and biology. TSC is short-circuit current that flows owing to the displacement of charges in samples during heating. TSC measurement is very simple, but TSC curves give very important information on charge behaviors. In the 1970s, TSC measurement contributed greatly to the development of electrical insulation engineering, semiconductor device technology, and so forth. Accordingly, the TSC experimental technique and its analytical method advanced. Over the past decades, many new molecules and advanced functional materials have been discovered and developed. Along with this, TSC measurement has attracted much attention in industries and academic laboratories as a way of characterizing newly discovered materials and devices. In this review, we report the latest research trend in the TSC method for the development of materials and devices in Japan.

Finnsjoen study site. Scope of activities and main results

International Nuclear Information System (INIS)

Ahlbom, K.; Andersson, J.E.; Andersson, Peter; Ittner, T.; Tiren, S.; Ljunggren, C.

1992-12-01

The Finnsjoen study site was selected in 1977 to provide input to the KBS-1 and KBS-2 performance assessments. The site was later used as a test site for testing new instruments and new site characterization methods, as well as a research site for studying mainly groundwater flow and groundwater transport. All together, the Finnsjoen studies have involved 11 cored boreholes, down to max 700 m depth, and extensive borehole geophysical, geochemical and geohydraulic measurements, as well as rock stress measurements and tracer tests. This report presents the scope of the Finnsjoen studies together with main results. Conceptual uncertainties in assumptions and models are discussed with emphasis on the models used for the performance assessment SKB91. Of special interest for the Finnsjoen study site is the strong influence caused by a subhorizontal fracture zone on groundwater flow, transport and chemistry

Klipperaas study site. Scope of activities and main results

International Nuclear Information System (INIS)

Ahlbom, K.; Andersson, J.E.; Andersson, Peter; Ittner, T.; Tiren, S.; Ljunggren, C.

1992-09-01

During the period from 1977 - 1986 SKB (Swedish Nuclear Fuel and Waste Management Co.) performed surface and borehole investigations of 14 study sites for the purpose of assessing their suitability for a repository of spent nuclear fuel. The next phase in the SKB site selection rpogramme will be to perform detailed characterisation, including characterization from shafts and/or tunnels, of two or three sites. The detailed investigations will continue over several years to provide all the data needed for a licensing application to build a repository. Such an application is foreseen to be given to the authorities around the year 2003. It is presently not clear if any of the study sites will be selected as a site for detailed characterization. Other sites with geological and/or socio-economical characteristics judged more favorable may very well be the ones selected. However, as a part of the background documentation needed for the site selection studies to come, summary reports will be prepared for most study sites. These reports will include scope of activities, main results, uncertainties and need of complementary investigations. This report concern the Klipperaas study site. The main topics are the scope of activities, geologic model, geohydrological model, groundwater chemistry, assessment of solute transport, and rock mechanics

Considerations applicable to the transportability of a transportable storage cask at the end of the storage period

International Nuclear Information System (INIS)

Sanders, T.L.; Ottinger, C.A.; Brimhall, J.L.; Creer, J.M.; Gilbert, E.R.; Jones, R.H.; McConnell, P.E.

1991-11-01

Additional spent fuel storage capacity is needed at many nuclear power plant sites where spent fuel storage pools have either reached or are expected to reach maximum capacities before spent fuel can be removed. This analysis examines certain aspects of Transportable Storage Casks (TSC) to assist in the determination of their feasibility as an option for at-reactor dry storage. Factors that can affect in-transport reliability include: the quality of design, development, and fabrication activities; the possibilities of damage or error during loading and closure; in-storage deterioration or unanticipated storage conditions; and the potential for loss of storage period monitoring/measurement data necessary for verifying the TSC fitness-for-transport. The reported effort utilizes a relative reliability comparison of TSCs to Transport-Only Casks (TOC) to identify and prioritize those issues and activities that are unique to TSCs. TSC system recommendations combine certain design and operational features, such as in-service monitoring, pretransport assessments, and conservation design assumptions, which when implemented and verified, should sufficiently ensure that the system will perform as intended in a later transport environment

Tuberous Sclerosis Complex in 29 Children: Clinical and Genetic Analysis and Facial Angiofibroma Responses to Topical Sirolimus.

Science.gov (United States)

Wang, Senfen; Liu, Yuanxiang; Wei, Jinghai; Zhang, Jian; Wang, Zhaoyang; Xu, Zigang

2017-09-01

Tuberous sclerosis complex (TSC) is a genetic disorder and facial angiofibromas are disfiguring facial lesions. The aim of this study was to analyze the clinical and genetic features of TSC and to assess the treatment of facial angiofibromas using topical sirolimus in Chinese children. Information was collected on 29 patients with TSC. Genetic analyses were performed in 12 children and their parents. Children were treated with 0.1% sirolimus ointment for 36 weeks. Clinical efficacy and plasma sirolimus concentrations were evaluated at baseline and 12, 24, and 36 weeks. Twenty-seven (93%) of the 29 patients had hypomelanotic macules and 15 (52%) had shagreen patch; 11 of the 12 (92%) who underwent genetic analysis had gene mutations in the TSC1 or TSC2 gene. Twenty-four children completed 36 weeks of treatment with topical sirolimus; facial angiofibromas were clinically undetectable in four (17%). The mean decrease in the Facial Angiofibroma Severity Index (FASI) score at 36 weeks was 47.6 ± 30.4%. There was no significant difference in the FASI score between weeks 24 and 36 (F = 1.00, p = 0.33). There was no detectable systemic absorption of sirolimus. Hypomelanotic macules are often the first sign of TSC. Genetic testing has a high detection rate in patients with a clinical diagnosis of TSC. Topical sirolimus appears to be both effective and well-tolerated as a treatment of facial angiofibromas in children with TSC. The response typically plateaus after 12 to 24 weeks of treatment. © 2017 Wiley Periodicals, Inc.

Clinical management of tuberous sclerosis complex over the lifetime of a patient

Directory of Open Access Journals (Sweden)

Frost M

2015-08-01

Full Text Available Michael Frost,1 John Hulbert2 1Minnesota Epilepsy Group, PA, St Paul, MN, USA; 2Urologic Physicians, PA, Edina, MN, USA Abstract: Tuberous sclerosis complex (TSC is a genetic disorder characterized by nonmalignant tumors (hamartomas that can occur in various organ systems, including the brain, kidneys, lungs, skin, eyes, and heart. Clinical manifestations of TSC can occur at any age, thereby making the diagnosis difficult. No typical disease presentation is known, and the clinical presentation usually differs between pediatric and adult patients. Furthermore, variable penetrance of the genetic mutation causes a range of disease severity from very mild to severe, and affected individuals can go undetected for years because many of the clinical manifestations of TSC lack specificity. Once a diagnosis is made, TSC management strategies should be tailored to address the symptoms and risks most relevant to the age of the patient. Improved understanding of the genetic basis of TSC and of the central issue of mTOR overactivation has led to use of pharmacotherapies such as the mTOR inhibitors everolimus and sirolimus in the treatment of TSC disease. In Phase II and III studies, everolimus has demonstrated efficacy and safety in the treatment of both brain (subependymal giant cell astrocytoma and renal (angiomyolipoma manifestations associated with TSC. It is important to bear in mind that TSC is a lifelong condition, and for those diagnosed as children, a continuum of care will be needed as they transition from pediatric to adult health services. Clearly identifying the likely differences among diagnosis, monitoring, and management of pediatric and adult patients with TSC is an important step in enabling efficiencies to be maximized without compromising the care provided to patients. Keywords: mammalian target of rapamycin inhibitors, mTOR treatment, treatment strategies

Nuclear Energy Center study. Phase II. Site suitability analysis. Final report

International Nuclear Information System (INIS)

Fellows, W.S.; Sharp, J.M.; Benator, B.I.

1978-06-01

A site screening study was conducted to identify a site or sites for detailed, site-specific study as a nuclear energy center. Using technical criteria of water requirements, geotechnical constraints, and projected load center and transmission considerations as well as environmental and institutional considerations, five potential study sites in the State of South Carolina were identified, evaluated against established criteria, and ranked according to their acceptability as potential nuclear energy center study sites. Consideration of what is ''representative'' of a site as well as the ranking score was factored into site recommendations, since the site deemed easiest to license and permit may not be the most desirable site for future study of the technical and institutional feasibility and practicality of a specific site. The sites near Lake Hartwell and the Savannah River Plant (SRP) of the Department of Energy were selected as potential study sites after consideration of the above criteria. Because the Lake Hartwell site offers the opportunity to consider institutional issues which may be more representative of other possible NEC sites, it is recommended that the Lake Hartwell site be studied to establish the feasibility and practicality of the nuclear energy concept on a site-specific basis

Nuclear Energy Center study. Phase II. Site suitability analysis. Final report

Energy Technology Data Exchange (ETDEWEB)

Fellows, W.S.; Sharp, J.M.; Benator, B.I.

1978-06-01

A site screening study was conducted to identify a site or sites for detailed, site-specific study as a nuclear energy center. Using technical criteria of water requirements, geotechnical constraints, and projected load center and transmission considerations as well as environmental and institutional considerations, five potential study sites in the State of South Carolina were identified, evaluated against established criteria, and ranked according to their acceptability as potential nuclear energy center study sites. Consideration of what is ''representative'' of a site as well as the ranking score was factored into site recommendations, since the site deemed easiest to license and permit may not be the most desirable site for future study of the technical and institutional feasibility and practicality of a specific site. The sites near Lake Hartwell and the Savannah River Plant (SRP) of the Department of Energy were selected as potential study sites after consideration of the above criteria. Because the Lake Hartwell site offers the opportunity to consider institutional issues which may be more representative of other possible NEC sites, it is recommended that the Lake Hartwell site be studied to establish the feasibility and practicality of the nuclear energy concept on a site-specific basis.

Universal Access in Heritage Sites: A Case Study on Historic Sites in Jaipur, India.

Science.gov (United States)

Vardia, Shweta; Khare, Rachna; Khare, Ajay

2016-01-01

A nation is recognized by a range of its significant historical, cultural and natural properties. These properties are generally preserved and maintained either by national administration or by private owners and charitable trusts due to higher value of their cultural inheritance and termed globally as heritage or historic sites. Heritage sites are a significant asset, a unique and irreplaceable resource which reflects a rich and diverse expression of past societies and forms an integral part of local, regional and national cultural identity. Today, heritage sites also play an important role in communication and knowledge exchange. Thus the rapidly increasing heritage tourism industry faces several challenges too. One of the challenges is that there is a segment of society who is not yet able to equally enjoy the visit to historic structures/sites and attractions, facilities and services. This paper aims to study the experience and develop understanding regarding the heritage structures/sites approached and interacted by diverse users. This study is an outcome of a hands on workshop conducted with diverse users at various historic sites in the city of Jaipur viz. at The City Palace Complex, Jaipur, Jaigarh Fort and the Haveli at Kanota near to Jaipur India.

Site study plan for cultural resources, Deaf Smith County site, Texas: Environmental Field Program: Preliminary draft

International Nuclear Information System (INIS)

1987-06-01

The Cultural Resources Site Study Plan describes a field program to identify and evaluate the archaeological, historical, and Native American Indian resources of the site on local and regional perspectives; monitor and manage discovered cultural resources; and establish a worker education program. The archaeological field program consists of three pedestrian surveys: Survey 1 includes two EDBH seismic survey lines and the area within the exploratory shaft facility (ESF); Survey 2 includes the remainder of the site plus a 1/4 to 3/4-mi border area; and Survey 3 includes an assortment of offsite areas. The historical studies will identify and evaluate known and discovered historical sites and structures and the Native American Indian will identify and evaluate cultural and religious concerns expressed by Indian tribal groups. Prehistoric and historic sites will be evaluated to determine if they meet eligibility criteria for listing on the National Register of Historic Places. This site study plan describes the need for each study; its design and design rationale; analysis, management, and use of data; schedule of field activities; organization of field personnel and sample management; and quality assurance requirements. The cultural resource studies will provide data for satisfying the Programmatic Agreement, engineering design needs, and SRP requirements for permits and approvals, and for minimizing effects to any cultural properties discovered during site characterization. 75 refs., 10 figs., 2 tabs

Thermosyphon Cooler Hybrid System for Water Savings in an Energy-Efficient HPC Data Center: Modeling and Installation: Preprint

Energy Technology Data Exchange (ETDEWEB)

Carter, Thomas; Liu, Zan; Sickinger, David; Regimbal, Kevin; Martinez, David

2017-02-01

water on Sandia's site. In addition to describing the installation of the TSC and its integration into the ESIF, this paper focuses on the full heat rejection system simulation program used for hourly analysis of the energy and water consumption of the complete system under varying operating scenarios. A follow-up paper will detail the test results. The evaluation of the TSC's performance at NREL will also determine a path forward at Sandia for possible deployment in a large-scale system not only for data center use but also possibly site wide.

Site study plan for routine laboratory rock mechanics, Deaf Smith County Site, Texas: Revision 1

International Nuclear Information System (INIS)

1987-12-01

This Site Study Plan for Routine Laboratory Rock Mechanics describes routine laboratory testing to be conducted on rock samples collected as part of the characterization of the Deaf Smith County site, Texas. This study plan describes the early laboratory testing. Additional testing may be required and the type and scope of testing will be dependent upon the results of the early testing. This study provides for measurements of index, hydrological, mechanical, and chemical properties with tests which are standardized and used widely in geotechnical investigations. Another Site Study Plan for Nonroutine Laboratory Rock Mechanics describes laboratory testing of samples from the site to determine mechanical, thermomechanical, and thermal properties by less widely used methods, many of which have been developed specifically for characterization of the site. Data from laboratory tests will be used for characterization of rock strata, design of shafts and underground facilities, and modeling of repository behavior in support of resolution of both preclosure and postclosure issues. A tentative testing schedule and milestone log are given. A quality assurance program will be utilized to assure that activities affecting quality are performed correctly and that appropriate documentation is maintained. 18 refs., 8 figs., 3 tabs

Comprehensive analysis of gene mutation and phenotype of tuberous sclerosis complex in China

Directory of Open Access Journals (Sweden)

Guo-qiang HUANG

2015-04-01

Full Text Available Objective To summarize the clinical features of tuberous sclerosis complex (TSC, the distribution and description of TSC gene, and to probe into the correlation of genotype with phenotype.  Methods According to the 1998 International Tuberous Sclerosis Complex Diagnostic Criteria, a total of 163 TSC patients with pathogenic mutation in TSC gene (3 cases were detected in our hospital, and the other 160 cases were collected from other institutions in China were enrolled, and their gene detection results and clinical data were analyzed.  Results Among 163 cases, TSC1 mutation (31 cases accounted for 19.02% [32.26% (10/31 in exon 15, 16.13% (5/31 in exon 21, 12.90% (4/31 in exon 18], and TSC2 mutation (132 cases accounted for 80.98% [9.85% (13/132 in exon 37, 7.58% (10/132 in exon 40, 6.82%(9/132 in exon 33]. The proportion of base replacement in TSC1 was 41.94% (13/31, and 52.27% (69/132 in TSC2. Male patients exhibited significantly more subependymal nodules or calcifications than thefemale patients (χ2 = 8.016, P = 0.005. Sporadic patients exhibited significantly more cortical tubers than familial patients (χ2 = 6.273, P = 0.012. Patients with TSC2 mutations had significantly higher frequencies of hypomelanotic macules than patients with TSC1 mutations (χ2 = 6.756, P = 0.009. Patients with missense mutations were more likely to have facial angiofibromas compared with patients with other mutations (χ2 = 4.438, P = 0.035.  Conclusions Exon 15, 21 and 18 of TSC1 and exon 37, 40 and 33 of TSC2 accounted for higher percentage of mutations. Correlating genotypes with phenotypes should facilitate the individualized treatment and prognostic assessment of tuberous sclerosis complex. DOI: 10.3969/j.issn.1672-6731.2015.04.013

Siting studies for new nuclear power plants in Argentina

International Nuclear Information System (INIS)

Barbaran, Gustavo A.

2008-01-01

This paper is a summary of the thesis prepared by the Group of Prospective and Energy Planning of the National Atomic Energy Commission for the 'Specialization on Applications of Nuclear Technology Course' of the Instituto Balseiro in 2007. It describes the evolution of siting studies through time and the main focus worldwide in this type of studies. Then, it makes a brief review of previous siting studies of nuclear power plants conducted in Argentina. It carries out a description of the methodology to conduct a site evaluation for nuclear power plants according to actual international criteria. Finally, it describes the licensing process that follows every site study. (author) [es

Analysis of laparoscopic port site complications: A descriptive study.

Science.gov (United States)

Karthik, Somu; Augustine, Alfred Joseph; Shibumon, Mundunadackal Madhavan; Pai, Manohar Varadaraya

2013-04-01

The rate of port site complications following conventional laparoscopic surgery is about 21 per 100,000 cases. It has shown a proportional rise with increase in the size of the port site incision and trocar. Although rare, complications that occur at the port site include infection, bleeding, and port site hernia. To determine the morbidity associated with ports at the site of their insertion in laparoscopic surgery and to identify risk factors for complications. Prospective descriptive study. In the present descriptive study, a total of 570 patients who underwent laparoscopic surgeries for various ailments between August 2009 and July 2011 at our institute were observed for port site complications prospectively and the complications were reviewed. Descriptive statistical analysis was carried out in the present study. The statistical software, namely, SPSS 15.0 was used for the analysis of the data. Of the 570 patients undergoing laparoscopic surgery, 17 (3%) had developed complications specifically related to the port site during a minimum follow-up of three months; port site infection (PSI) was the most frequent (n = 10, 1.8%), followed by port site bleeding (n = 4, 0.7%), omentum-related complications (n = 2; 0.35%), and port site metastasis (n = 1, 0.175%). Laparoscopic surgeries are associated with minimal port site complications. Complications are related to the increased number of ports. Umbilical port involvement is the commonest. Most complications are manageable with minimal morbidity, and can be further minimized with meticulous surgical technique during entry and exit.

Analysis of laparoscopic port site complications: A descriptive study

Directory of Open Access Journals (Sweden)

Somu Karthik

2013-01-01

Full Text Available Context: The rate of port site complications following conventional laparoscopic surgery is about 21 per 100,000 cases. It has shown a proportional rise with increase in the size of the port site incision and trocar. Although rare, complications that occur at the port site include infection, bleeding, and port site hernia. Aims: To determine the morbidity associated with ports at the site of their insertion in laparoscopic surgery and to identify risk factors for complications. Settings and Design: Prospective descriptive study. Materials and Methods: In the present descriptive study, a total of 570 patients who underwent laparoscopic surgeries for various ailments between August 2009 and July 2011 at our institute were observed for port site complications prospectively and the complications were reviewed. Statistical Analysis Used: Descriptive statistical analysis was carried out in the present study. The statistical software, namely, SPSS 15.0 was used for the analysis of the data. Results: Of the 570 patients undergoing laparoscopic surgery, 17 (3% had developed complications specifically related to the port site during a minimum follow-up of three months; port site infection (PSI was the most frequent (n = 10, 1.8%, followed by port site bleeding (n = 4, 0.7%, omentum-related complications (n = 2; 0.35%, and port site metastasis (n = 1, 0.175%. Conclusions: Laparoscopic surgeries are associated with minimal port site complications. Complications are related to the increased number of ports. Umbilical port involvement is the commonest. Most complications are manageable with minimal morbidity, and can be further minimized with meticulous surgical technique during entry and exit.

Analysis of laparoscopic port site complications: A descriptive study

Science.gov (United States)

Karthik, Somu; Augustine, Alfred Joseph; Shibumon, Mundunadackal Madhavan; Pai, Manohar Varadaraya

2013-01-01

CONTEXT: The rate of port site complications following conventional laparoscopic surgery is about 21 per 100,000 cases. It has shown a proportional rise with increase in the size of the port site incision and trocar. Although rare, complications that occur at the port site include infection, bleeding, and port site hernia. AIMS: To determine the morbidity associated with ports at the site of their insertion in laparoscopic surgery and to identify risk factors for complications. SETTINGS AND DESIGN: Prospective descriptive study. MATERIALS AND METHODS: In the present descriptive study, a total of 570 patients who underwent laparoscopic surgeries for various ailments between August 2009 and July 2011 at our institute were observed for port site complications prospectively and the complications were reviewed. STATISTICAL ANALYSIS USED: Descriptive statistical analysis was carried out in the present study. The statistical software, namely, SPSS 15.0 was used for the analysis of the data. RESULTS: Of the 570 patients undergoing laparoscopic surgery, 17 (3%) had developed complications specifically related to the port site during a minimum follow-up of three months; port site infection (PSI) was the most frequent (n = 10, 1.8%), followed by port site bleeding (n = 4, 0.7%), omentum-related complications (n = 2; 0.35%), and port site metastasis (n = 1, 0.175%). CONCLUSIONS: Laparoscopic surgeries are associated with minimal port site complications. Complications are related to the increased number of ports. Umbilical port involvement is the commonest. Most complications are manageable with minimal morbidity, and can be further minimized with meticulous surgical technique during entry and exit. PMID:23741110

Electrochemical behaviour of alkaline copper complexes

Indian Academy of Sciences (India)

Abstract. A search for non-cyanide plating baths for copper resulted in the development of alkaline copper complex baths containing trisodium citrate [TSC] and triethanolamine [TEA]. Voltammetric studies were carried out on platinum to understand the electrochemical behaviour of these complexes. In TSC solutions, the.

Site study plan for borehole search and characterization, Deaf Smith County Site, Texas: Revision 1

International Nuclear Information System (INIS)

1987-01-01

This site study plan describes the Borehole Search and Characterization field activities to be conducted during the early stages of Site Characterization at the Deaf Smith County site, Texas. The field program has been designed to provide data useful in addressing information/data needs resulting from Federal/State/Local regulatory requirements and repository program requirements. Air and ground surveys, an extensive literature search, and landowner interviews will be conducted to locate wells within and adjacent to the proposed nuclear waste repository site in Deaf Smith County. Initially, the study will center around the planned Exploratory Shaft Facilities location and will expand outward from that location. Findings from this study may lead to preparation of a new site study plan to search suspected borehole locations, and excavate or reenter known boreholes for additional characterization or remedial action. The Salt Repository Project (SRP) Networks specify the schedule under which the program will operate. The Technical Field Services Contractor (TFSC) is responsible for conducting the field program. Data will be handled and reported in accordance with established SRP procedures. A quality assurance program will be utilized to assure that activities affecting quality are performed correctly and that appropriate documentation is maintained. 13 refs., 6 figs., 3 tabs

Case study of siting technology for underground nuclear power plant

International Nuclear Information System (INIS)

Hibino, Satoshi; Komada, Hiroya; Honsho, Shizumitsu; Fujiwara, Yoshikazu; Motojima, Mutsumi; Nakagawa, Kameichiro; Nosaki, Takashi

1991-01-01

Underground siting method is one of new feasible siting methods for nuclear power plants. This report presents the results on case studies on underground siting. Two sites of a steeply inclined and plateau like configurations were selected. 'Tunnel type cavern; all underground siting' method was applied for the steeply inclined configuration, and 'shaft type semi-cavern; partial underground siting' method was applied for the plateau like configuration. The following designs were carried out for these two sites as case studies; (1) conceptual designs, (2) geological surveys and rock mechanics tests, (3) stability analysis during cavern excavations, (4) seismic stability analysis of caverns during earthquake, (5) reinforcement designs for caverns, (6) drainage designs. The case studies showed that these two cases were fully feasible, and comparison between two cases revealed that the 'shaft type semi-cavern; partial underground siting' method was more suitable for Japanese islands. As a first step of underground siting, therefore, the authors recommend to construct a nuclear power plant by this method. (author)

Conduction and TSC properties of LB films adsorbing cyanine dyes; Cyanine shikiso kyuchaku LB maku ni okeru denki dendo oyobi netsushigeki denryu tokusei

Energy Technology Data Exchange (ETDEWEB)

Shinbo, K.; Kato, K.; Kaneko, F.; Kobayashi, S. [Niigata University, Niigata (Japan)

1995-05-20

The electric properties were investigated for phosphatidic acid (PA) LB films adsorbing cyanide dyes (NK-3) with sandwich electrodes. PA LB adsorbing the dyes and the monolayers of Cd salt without the dyes are deposited using subphases, containing various concentrations of the dyes and containing CdCl2 instead of the dyes, respectively. Conduction currents are measured at room temperature for the PA LB film devices. Increases in the conduction currents are observed in the LB films containing the dyes. Furthermore, the conduction currents remarkably increase using the Au upper electrode. The current(I) vs. voltage (V) properties nearly follow the Poole conduction, log(I)-V. Therefore, the conduction currents are very complicated and are thought to be due to composite mechanisms in the film and at the interface. TSC measurements are used to examine the dipolar polarizations in the PA LB films, in the temperature region between 150 K and 300 K. Remarkable increase of TSCs are observed above 260 K for the devices containing the dyes. The TSCs are caused by the dipolar polarizations, and increase with the quantity of the dyes contained in the LB devices and/or aggregations of the dyes. The results are useful for applications of ultrathin film devices in the future. 16 refs., 11 figs., 3 tabs.

Interactive anatomical and surgical live stream lectures improve students' academic performance in applied clinical anatomy.

Science.gov (United States)

Shiozawa, Thomas; Butz, Benjamin; Herlan, Stephan; Kramer, Andreas; Hirt, Bernhard

2017-01-01

Tuebingen's Sectio Chirurgica (TSC) is an innovative, interactive, multimedia, and transdisciplinary teaching method designed to complement dissection courses. The Tuebingen's Sectio Chirurgica (TSC) allows clinical anatomy to be taught via interactive live stream surgeries moderated by an anatomist. This method aims to provide an application-oriented approach to teaching anatomy that offers students a deeper learning experience. A cohort study was devised to determine whether students who participated in the TSC were better able to solve clinical application questions than students who did not participate. A total of 365 students participated in the dissection course during the winter term of the 2012/2013 academic year. The final examination contained 40 standard multiple-choice (S-MC) and 20 clinically-applied multiple-choice (CA-MC) items. The CA-MC items referred to clinical cases but could be answered solely using anatomical knowledge. Students who regularly participated in the TSC answered the CA-MC questions significantly better than the control group (75% and 65%, respectively; P  0.05). The CA-MC questions had a slightly higher level of difficulty than the S-MC questions (0.725 and 0.801, respectively; P = 0.083). The discriminatory power of the items was comparable (S-MC median Pearson correlations: 0.321; CA-MC: 0.283). The TSC successfully teaches the clinical application of anatomical knowledge. Students who attended the TSC in addition to the dissection course were able to answer CA-MC questions significantly better than students who did not attend the TSC. Thus, attending the TSC in addition to the dissection course supported students' clinical learning goals. Anat Sci Educ 10: 46-52. © 2016 American Association of Anatomists. © 2016 American Association of Anatomists.

Site Study Plan for land use, Deaf Smith County site, Texas: Environmental Field Program: Preliminary draft

International Nuclear Information System (INIS)

1987-06-01

The Land Use Site Study Plan describes a field program consisting of characterization of the site; seismic survey rights-of-way and transportation and utility corridors, the vicinity, the region, future land use, and monitoring land-use change. Aerial photography will be used to characterize the site, seismic rows and transportation and utility corridors, and the vicinity. The resulting land-use maps and overlays will then be verified in the field. Interviews with farm managers and local experts will provide additional information. A Geographic Information System (GIS) and satellite imagery will be used to characterize the region, monitor land-use change, and provide information to assist with the future land use study. The site study plan describes the study design and rationale, the filed data collection procedures and equipment, the data analysis methods and application of results, the data management strategy, the schedule of field activities, the personnel requirements and management of the study, and the study's quality assurance program. The directives and requirements that drive these studies are derived from the Salt Repository Project Requirements Document. 51 refs; 6 figs; 3 tabs

White Matter Loss in a Mouse Model of Periventricular Leukomalacia Is Rescued by Trophic Factors

Directory of Open Access Journals (Sweden)

Pierre Gressens

2013-11-01

Full Text Available Periventricular leukomalacia (PVL is the most frequent cause of cerebral palsy and other intellectual disabilities, and currently there is no treatment. In PVL, glutamate excitotoxicity (GME leads to abnormal oligodendrocytes (OLs, myelin deficiency, and ventriculomegaly. We have previously identified that the combination of transferrin and insulin growth factors (TSC1 promotes endogenous OL regeneration and remyelination in the postnatal and adult rodent brain. Here, we produced a periventricular white matter lesion with a single intracerebral injection of N-methyl-d-aspartate (NMDA. Comparing lesions produced by NMDA alone and those produced by NMDA + TSC1 we found that: NMDA affected survival and reduced migration of OL progenitors (OLPs. In contrast, mice injected with NMDA + TSC1 proliferated twice as much indicating that TSC1 supported regeneration of the OLP population after the insult. Olig2-mRNA expression showed 52% OLP survival in mice receiving a NMDA injection and increased to 78% when TSC1 + NMDA were injected simultaneously and ventricular size was reduced by TSC1. Furthermore, in striatal slices TSC1 reduced the inward currents induced by NMDA in medium-sized spiny neurons, demonstrating neuroprotection. Thus, white matter loss after excitotoxicity can be partially rescued as TSC1 conferred neuroprotection to preexisting OLP and regeneration via OLP proliferation. Furthermore, we showed that early TSC1 administration maximizes neuroprotection.

Studies for site preparation for ITER construction

International Nuclear Information System (INIS)

Fardeau, A.; Blanc, F.; Cardettini, J.D.; Mandine, J.R.; Guerin, R.; Patisson, L.; Bergegere, P.; Santagiustina, A.; Garin, P.

2004-01-01

The implantation of a nuclear facility such as ITER (about 20 buildings on 40 ha) requires many preparatory studies, particularly with respect to: Underground characteristics; Topography, layout; Deforestation, excavations; Networks, fences and roads; Impact of seismic hazard on design. This paper presents the main results of these studies, carried out within the European ITER Site Studies framework. A dedicated paper in the conference deals with the transport studies. To choose the site for ITER implantation, detailed geological, hydrogeological and geophysical investigations have been carried out. Taking into account the meteorological data (particularly the main wind direction), topography, access, electrical supply, fluids needs and constraints (gravity systems), buildings and roads have been implemented. (authors)

Physical mapping of QTL for tuber yield, starch content and starch yield in tetraploid potato (Solanum tuberosum L.) by means of genome wide genotyping by sequencing and the 8.3 K SolCAP SNP array.

Science.gov (United States)

Schönhals, Elske Maria; Ding, Jia; Ritter, Enrique; Paulo, Maria João; Cara, Nicolás; Tacke, Ekhard; Hofferbert, Hans-Reinhard; Lübeck, Jens; Strahwald, Josef; Gebhardt, Christiane

2017-08-22

Tuber yield and starch content of the cultivated potato are complex traits of decisive importance for breeding improved varieties. Natural variation of tuber yield and starch content depends on the environment and on multiple, mostly unknown genetic factors. Dissection and molecular identification of the genes and their natural allelic variants controlling these complex traits will lead to the development of diagnostic DNA-based markers, by which precision and efficiency of selection can be increased (precision breeding). Three case-control populations were assembled from tetraploid potato cultivars based on maximizing the differences between high and low tuber yield (TY), starch content (TSC) and starch yield (TSY, arithmetic product of TY and TSC). The case-control populations were genotyped by restriction-site associated DNA sequencing (RADseq) and the 8.3 k SolCAP SNP genotyping array. The allele frequencies of single nucleotide polymorphisms (SNPs) were compared between cases and controls. RADseq identified, depending on data filtering criteria, between 6664 and 450 genes with one or more differential SNPs for one, two or all three traits. Differential SNPs in 275 genes were detected using the SolCAP array. A genome wide association study using the SolCAP array on an independent, unselected population identified SNPs associated with tuber starch content in 117 genes. Physical mapping of the genes containing differential or associated SNPs, and comparisons between the two genome wide genotyping methods and two different populations identified genome segments on all twelve potato chromosomes harboring one or more quantitative trait loci (QTL) for TY, TSC and TSY. Several hundred genes control tuber yield and starch content in potato. They are unequally distributed on all potato chromosomes, forming clusters between 0.5-4 Mbp width. The largest fraction of these genes had unknown function, followed by genes with putative signalling and regulatory functions. The

Site study plan for regional hydrologic sampling and monitoring: Preliminary draft

International Nuclear Information System (INIS)

Dutton, A.R.

1987-01-01

The purpose of the Regional Hydrologic Studies Plan is to describe those field activities required for completion of the objectives of hydrologic activities. Many of these activities are regional in scope and are designed to provide a framework for understanding the hydrologic setting of the site and the hydrologic processes that influence site characteristics. Site Study Plans (SSPs) define activates at and in the immediate vicinity of the site. The activities specified in the Regional Hydrologic Studies Plan are performed beyond the confines of the site because the hydrologic systems extend beyond the site boundaries, because pertinent data that bear on site suitability are available outside of the site, and because natural analogues exist outside of the site that allow analysis of processes that are expected to operate within the site. 15 refs., 5 figs., 1 tab

Quantifying siting difficulty: a case study of US transmission line siting

Energy Technology Data Exchange (ETDEWEB)

Vajjhala, S.P. [Resources for the Future, Washington DC (United States); Fischbeck, P.S. [Carnegie Mellon University, Pittsburgh, PA (United States). Departments of Social and Decision Sciences and Engineering and Public Policy

2007-01-15

The worldwide demand for new energy infrastructures has been paralleled in recent years by the increasing difficulty of siting major facilities. Siting difficulty is the subject of widespread discussion, but because of the complexity of the problem, potential solutions are not obvious or well understood. This paper presents a two-step policy-level framework that first develops an empirical measure of siting difficulty and then qualitatively assesses its major causes. The approach is based on the creation and aggregation of four siting indicators that are independent of the common causes and localized effects of siting problems. The proposed framework is demonstrated for the case of US transmission line siting. Results of the analyses reveal significant variations in state siting difficulty and industry experts' perceptions of its dominant causes, with implications for the long-term success of Regional Transmission Organizations (RTOs) and knowledge transfer among siting professionals in the deregulated industry. (author)

Hanford tank initiative test facility site selection study

International Nuclear Information System (INIS)

Staehr, T.W.

1997-01-01

The Hanford Tanks Initiative (HTI) project is developing equipment for the removal of hard heel waste from the Hanford Site underground single-shell waste storage tanks. The HTI equipment will initially be installed in the 241-C-106 tank where its operation will be demonstrated. This study evaluates existing Hanford Site facilities and other sites for functional testing of the HTI equipment before it is installed into the 241-C-106 tank

Reliability, factor structure, and validity of the German version of the Trauma Symptom Checklist for Children in a sample of adolescents

Science.gov (United States)

Matulis, Simone; Loos, Laura; Langguth, Nadine; Schreiber, Franziska; Gutermann, Jana; Gawrilow, Caterina; Steil, Regina

2015-01-01

Background The Trauma Symptom Checklist for Children (TSC-C) is the most widely used self-report scale to assess trauma-related symptoms in children and adolescents on six clinical scales. The purpose of the present study was to develop a German version of the TSC-C and to investigate its psychometric properties, such as factor structure, reliability, and validity, in a sample of German adolescents. Method A normative sample of N=583 and a clinical sample of N=41 adolescents with a history of physical or sexual abuse aged between 13 and 21 years participated in the study. Results The Confirmatory Factor Analysis on the six-factor model (anger, anxiety, depression, dissociation, posttraumatic stress, and sexual concerns with the subdimensions preoccupation and distress) revealed acceptable to good fit statistics in the normative sample. One item had to be excluded from the German version of the TSC-C because the factor loading was too low. All clinical scales presented acceptable to good reliability, with Cronbach's α's ranging from .80 to .86 in the normative sample and from .72 to .87 in the clinical sample. Concurrent validity was also demonstrated by the high correlations between the TSC-C scales and instruments measuring similar psychopathology. TSC-C scores reliably differentiated between adolescents with trauma history and those without trauma history, indicating discriminative validity. Conclusions In conclusion, the German version of the TSC-C is a reliable and valid instrument for assessing trauma-related symptoms on six different scales in adolescents aged between 13 and 21 years. PMID:26498182

A Novel Multi-Digital Camera System Based on Tilt-Shift Photography Technology

Science.gov (United States)

Sun, Tao; Fang, Jun-yong; Zhao, Dong; Liu, Xue; Tong, Qing-xi

2015-01-01

Multi-digital camera systems (MDCS) are constantly being improved to meet the increasing requirement of high-resolution spatial data. This study identifies the insufficiencies of traditional MDCSs and proposes a new category MDCS based on tilt-shift photography to improve ability of the MDCS to acquire high-accuracy spatial data. A prototype system, including two or four tilt-shift cameras (TSC, camera model: Nikon D90), is developed to validate the feasibility and correctness of proposed MDCS. Similar to the cameras of traditional MDCSs, calibration is also essential for TSC of new MDCS. The study constructs indoor control fields and proposes appropriate calibration methods for TSC, including digital distortion model (DDM) approach and two-step calibrated strategy. The characteristics of TSC are analyzed in detail via a calibration experiment; for example, the edge distortion of TSC. Finally, the ability of the new MDCS to acquire high-accuracy spatial data is verified through flight experiments. The results of flight experiments illustrate that geo-position accuracy of prototype system achieves 0.3 m at a flight height of 800 m, and spatial resolution of 0.15 m. In addition, results of the comparison between the traditional (MADC II) and proposed MDCS demonstrate that the latter (0.3 m) provides spatial data with higher accuracy than the former (only 0.6 m) under the same conditions. We also take the attitude that using higher accuracy TSC in the new MDCS should further improve the accuracy of the photogrammetry senior product. PMID:25835187

Luminescence and conductivity studies on CVD diamond exposed to UV light

Science.gov (United States)

Bizzarri, A.; Bogani, F.; Bruzzi, M.; Sciortino, S.

1999-04-01

The photoluminescence (PL), thermoluminescence (TL) and thermally stimulated currents (TSC) of four high-quality CVD diamond films have been investigated in the range of temperatures between 300 and 700 K. The sample excitation has been carried out by means of an UV xenon lamp and UV laser lines. The features of the signals have been found equal to those obtained from particle excitation. The TL analysis shows the existence of several deep traps with activation energies between 0.6 and 1.0 eV. The contribution to the TL signal from different traps has been singled out by means of successive annealing processes. The TL results are in good agreement with those obtained from TSC measurements. The combined use of the two techniques allows a precise determination of the trap parameters. The spectral content of the TL response has also been compared with the PL signal in order to investigate the recombination process. This analysis shows that, in this temperature range, the TL signal is likely due to recombination from bound states rather than due to radiative free to bound transitions, as generally assumed in TL theory. The TSC signal is likely to arise from impurity band rather than from free carriers conduction.

Luminescence and conductivity studies on CVD diamond exposed to UV light

International Nuclear Information System (INIS)

Bizzarri, A.; Bogani, F.; Bruzzi, M.; Sciortino, S.

1999-01-01

The photoluminescence (PL), thermoluminescence (TL) and thermally stimulated currents (TSC) of four high-quality CVD diamond films have been investigated in the range of temperatures between 300 and 700 K. The sample excitation has been carried out by means of an UV xenon lamp and UV laser lines. The features of the signals have been found equal to those obtained from particle excitation. The TL analysis shows the existence of several deep traps with activation energies between 0.6 and 1.0 eV. The contribution to the TL signal from different traps has been singled out by means of successive annealing processes. The TL results are in good agreement with those obtained from TSC measurements. The combined use of the two techniques allows a precise determination of the trap parameters. The spectral content of the TL response has also been compared with the PL signal in order to investigate the recombination process. This analysis shows that, in this temperature range, the TL signal is likely due to recombination from bound states rather than due to radiative free to bound transitions, as generally assumed in TL theory. The TSC signal is likely to arise from impurity band rather than from free carriers conduction

Luminescence and conductivity studies on CVD diamond exposed to UV light

Energy Technology Data Exchange (ETDEWEB)

Bizzarri, A.; Bogani, F.; Bruzzi, M.; Sciortino, S

1999-04-21

The photoluminescence (PL), thermoluminescence (TL) and thermally stimulated currents (TSC) of four high-quality CVD diamond films have been investigated in the range of temperatures between 300 and 700 K. The sample excitation has been carried out by means of an UV xenon lamp and UV laser lines. The features of the signals have been found equal to those obtained from particle excitation. The TL analysis shows the existence of several deep traps with activation energies between 0.6 and 1.0 eV. The contribution to the TL signal from different traps has been singled out by means of successive annealing processes. The TL results are in good agreement with those obtained from TSC measurements. The combined use of the two techniques allows a precise determination of the trap parameters. The spectral content of the TL response has also been compared with the PL signal in order to investigate the recombination process. This analysis shows that, in this temperature range, the TL signal is likely due to recombination from bound states rather than due to radiative free to bound transitions, as generally assumed in TL theory. The TSC signal is likely to arise from impurity band rather than from free carriers conduction.

The use of everolimus in the treatment of neurocognitive problems in tuberous sclerosis (TRON): study protocol for a randomised controlled trial.

Science.gov (United States)

Randell, Elizabeth; McNamara, Rachel; Davies, D Mark; Owen-Jones, Eleri; Kirby, Nigel; Angel, Lianna; Drew, Cheney; Cannings-John, Rebecca; Smalley, Michelle; Saxena, Anurag; McDermott, Emer; Stockwell, Laura; de Vries, Petrus J; Hood, Kerry; Sampson, Julian R

2016-08-11

Tuberous sclerosis complex (TSC) is a genetic disorder affecting about 1 in 6000 people and is characterised by the development of tumours in many organs, including the skin and kidneys, and by a range of neurological and neuropsychiatric manifestations. TSC-associated neuropsychiatric disorders (TAND) occur in the majority of those with TSC, and they have a significant impact on patients and their families, given the everyday impact of TAND on education, employment, family and social life. The potential benefits of better treatment for TAND therefore include reduction in health care demands and wider benefits for patients and their carers. We have planned a single-centre, two-arm, individually randomised, phase II, double-blind, placebo-controlled trial of everolimus versus placebo in the treatment of neurocognitive problems in patients with tuberous sclerosis. Everolimus is a licensed medicine in this patient group, but for a different target of effect. The present trial is a proof-of-principle study developed to provide effect size estimates which may be used to inform the design of subsequent trials. Forty-eight patients aged 16-60 years with tuberous sclerosis who have an IQ >60 and a significant deficit (at least -2 SD) in one or more primary outcome measures will be randomly allocated in a ratio of 2:1 to receive everolimus or placebo, respectively. Participants will be assessed for eligibility and then be started on study medication 4 weeks later. They will then be randomised and receive placebo or everolimus for 24 weeks. Neurocognitive and safety assessments will be carried out at baseline and weeks 4, 12, 24 and 36. This study is designed to determine the effect sizes of treatment with everolimus or placebo for 6 months on specific neurocognitive functions-recall memory (verbal and non-verbal) and executive function-in people affected by TSC who have significant deficits in these functions. These data will provide new evidence to determine whether

A Case Study in the Mars Landing Site Selection for Science Objects

Directory of Open Access Journals (Sweden)

Haingja Seo

2012-12-01

Full Text Available It is a crucial matter to select a landing site for landers or rovers in planning the Mars exploration. The landing site must have not only a scientific value as a landing site, but also geographical features to lead a safe landing for Mars probes. In this regard, this study analyzed landing site of Mars probes and rovers in previous studies and discussed the adequacy of the landing site to scientific missions. Moreover, this study also examined domestic studies on the Mars. The frameworks of these studies will guide the selection of exploration sites and a landing site when sending Mars probe to the Mars through our own efforts. Additionally, this paper will be used as the preliminary data for selection of exploration site and a landing site.

Web vulnerability study of online pharmacy sites.

Science.gov (United States)

Kuzma, Joanne

2011-01-01

Consumers are increasingly using online pharmacies, but these sites may not provide an adequate level of security with the consumers' personal data. There is a gap in this research addressing the problems of security vulnerabilities in this industry. The objective is to identify the level of web application security vulnerabilities in online pharmacies and the common types of flaws, thus expanding on prior studies. Technical, managerial and legal recommendations on how to mitigate security issues are presented. The proposed four-step method first consists of choosing an online testing tool. The next steps involve choosing a list of 60 online pharmacy sites to test, and then running the software analysis to compile a list of flaws. Finally, an in-depth analysis is performed on the types of web application vulnerabilities. The majority of sites had serious vulnerabilities, with the majority of flaws being cross-site scripting or old versions of software that have not been updated. A method is proposed for the securing of web pharmacy sites, using a multi-phased approach of technical and managerial techniques together with a thorough understanding of national legal requirements for securing systems.

Tokamak simulation code manual

International Nuclear Information System (INIS)

Chung, Moon Kyoo; Oh, Byung Hoon; Hong, Bong Keun; Lee, Kwang Won

1995-01-01

The method to use TSC (Tokamak Simulation Code) developed by Princeton plasma physics laboratory is illustrated. In KT-2 tokamak, time dependent simulation of axisymmetric toroidal plasma and vertical stability have to be taken into account in design phase using TSC. In this report physical modelling of TSC are described and examples of application in JAERI and SERI are illustrated, which will be useful when TSC is installed KAERI computer system. (Author) 15 refs., 6 figs., 3 tabs

3He/4He production ratios by tetrahedral symmetric condensation

International Nuclear Information System (INIS)

Takahashi, Akito

2006-01-01

The present paper treats application of the Electronic Quasi-Particle Expansion Theory (EQPET) model for Tetrahedral Symmetric Condensate (TSC) of H/D mixed systems for Pd host metal. Production ratios of 3 He/ 4 He for multi-body fusion reactions in H/D mixed TSC systems are calculated as a function of H/D mixing ratio. The model is further extended to treat direct nuclear interactions between host-metal nucleus and TSC of pure four protons (or four deuterons), since TSC can become very small (far less than 1 pm radius) charge-neutral pseudo-particle. Results for the case of Ni + 4p/TSC are discussed with Ni + p capture reactions and Ni + 4p fission reactions. (authors)

Integration of Parent and Nurse Perspectives of Communication to Plan Care for Technology Dependent Children: The Theory of Shared Communication.

Science.gov (United States)

Giambra, Barbara K; Broome, Marion E; Sabourin, Teresa; Buelow, Janice; Stiffler, Deborah

The purpose of this qualitative research study was to expand our understanding of the process of communication between parents of hospitalized technology dependent children and their nurses originally detailed in the Theory of Shared Communication (TSC). This grounded theory study was conducted with five parents of technology dependent children hospitalized in a large Midwestern children's hospital and nine nurses who care for technology dependent children admitted to the same hospital during July and August 2013. Semi-structured interviews and journals (parents only), field notes and a demographic survey were used to collect data which was analyzed using constant comparative analysis. Parents verified the concepts of the TSC and relationships among them. Nurses' perceptions of communication with parents reflected the same parent identified and verified concepts upon which the TSC was originally grounded including respect for own and other's expertise, asking, listening, explaining, advocating, verifying understanding and negotiating roles to achieve mutual understanding of the child's plan of care. The nurses' perceptions differed stylistically but not categorically from those of the parents. The addition of the nurse's perspectives to the verified TSC expands our understanding of this process of communication. With the integration of nurse and parent perspectives, the TSC can be used to enhance communication and care for hospitalized technology dependent children and their families. Copyright © 2017 Elsevier Inc. All rights reserved.

Novel Histopathological Patterns in Cortical Tubers of Epilepsy Surgery Patients with Tuberous Sclerosis Complex.

Directory of Open Access Journals (Sweden)

Angelika Mühlebner

Full Text Available Tuberous Sclerosis Complex (TSC is a genetic hamartoma syndrome frequently associated with severe intractable epilepsy. In some TSC patients epilepsy surgery is a promising treatment option provided that the epileptogenic zone can be precisely delineated. TSC brain lesions (cortical tubers contain dysmorphic neurons, brightly eosinophilic giant cells and white matter alterations in various proportions. However, a histological classification system has not been established for tubers. Therefore, the aim of this study was to define distinct histological patterns within tubers based on semi-automated histological quantification and to find clinically significant correlations. In total, we studied 28 cortical tubers and seven samples of perituberal cortex from 28 TSC patients who had undergone epilepsy surgery. We assessed mammalian target of rapamycin complex 1 (mTORC1 activation, the numbers of giant cells, dysmorphic neurons, neurons, and oligodendrocytes, and calcification, gliosis, angiogenesis, inflammation, and myelin content. Three distinct histological profiles emerged based on the proportion of calcifications, dysmorphic neurons and giant cells designated types A, B, and C. In the latter two types we were able to subsequently associate them with specific features on presurgical MRI. Therefore, these histopathological patterns provide consistent criteria for improved definition of the clinico-pathological features of cortical tubers identified by MRI and provide a basis for further exploration of the functional and molecular features of cortical tubers in TSC.

Novel Histopathological Patterns in Cortical Tubers of Epilepsy Surgery Patients with Tuberous Sclerosis Complex.

Science.gov (United States)

Mühlebner, Angelika; van Scheppingen, Jackelien; Hulshof, Hanna M; Scholl, Theresa; Iyer, Anand M; Anink, Jasper J; van den Ouweland, Ans M W; Nellist, Mark D; Jansen, Floor E; Spliet, Wim G M; Krsek, Pavel; Benova, Barbora; Zamecnik, Josef; Crino, Peter B; Prayer, Daniela; Czech, Thomas; Wöhrer, Adelheid; Rahimi, Jasmin; Höftberger, Romana; Hainfellner, Johannes A; Feucht, Martha; Aronica, Eleonora

2016-01-01

Tuberous Sclerosis Complex (TSC) is a genetic hamartoma syndrome frequently associated with severe intractable epilepsy. In some TSC patients epilepsy surgery is a promising treatment option provided that the epileptogenic zone can be precisely delineated. TSC brain lesions (cortical tubers) contain dysmorphic neurons, brightly eosinophilic giant cells and white matter alterations in various proportions. However, a histological classification system has not been established for tubers. Therefore, the aim of this study was to define distinct histological patterns within tubers based on semi-automated histological quantification and to find clinically significant correlations. In total, we studied 28 cortical tubers and seven samples of perituberal cortex from 28 TSC patients who had undergone epilepsy surgery. We assessed mammalian target of rapamycin complex 1 (mTORC1) activation, the numbers of giant cells, dysmorphic neurons, neurons, and oligodendrocytes, and calcification, gliosis, angiogenesis, inflammation, and myelin content. Three distinct histological profiles emerged based on the proportion of calcifications, dysmorphic neurons and giant cells designated types A, B, and C. In the latter two types we were able to subsequently associate them with specific features on presurgical MRI. Therefore, these histopathological patterns provide consistent criteria for improved definition of the clinico-pathological features of cortical tubers identified by MRI and provide a basis for further exploration of the functional and molecular features of cortical tubers in TSC.

Site Study Plan for laboratory soil mechanics, Deaf Smith County site, Texas: Revision 1

International Nuclear Information System (INIS)

1987-12-01

This Site Study Plan for laboratory soil mechanics describes the laboratory testing to be conducted on soil samples collected as part of the characterization of the Deaf Smith County site, Texas. This study provides for measurements of index, mechanical, thermal, hydrologic, chemical, and mineral properties of soils from boring throughout the site. Samples will be taken from Playa Borings/Trenching, Transportation/Utilities Foundation Borings, Repository Surface Facilities Design Foundation Borings, and Exploratory Shaft Facilities Design Foundation Borings. Data from the laboratory tests will be used for soil strata characterization, design of foundations for surface structures, design of transportation facilities and utility structures, design of impoundments, design of shaft lining, design of the shaft freeze wall, shaft permitting, performance assessment calculations, and other program requirements. A tentative testing schedule and milestone log are given. A quality assurance program will be utilized to assure that activities affecting quality are performed correctly and that appropriate documentation is maintained. 18 refs., 6 figs., 3 tabs

From mTOR to Cognition: Molecular and Cellular Mechanisms of Cognitive Impairments in Tuberous Sclerosis

Science.gov (United States)

Ehninger, D.; de Vries, P. J.; Silva, A. J.

2009-01-01

Background: Tuberous sclerosis (TSC) is a multi-system disorder caused by heterozygous mutations in the "TSC1" or "TSC2" gene and is often associated with neuropsychiatric symptoms, including intellectual disability, specific neuropsychological deficits, autism, other behavioural disorders and epilepsy. Method: Here, we review evidence from animal…

A 28-Year-Old Male Patient with Nail Tumors, Skin Lesions, and Epilepsy.

Science.gov (United States)

Balak, Deepak M W; Zonnenberg, Bernard A; Spitzer-Naaijkens, Juliette M J; Hulshof, Mieke M

2017-01-01

Tuberous sclerosis complex (TSC) is an autosomal dominant disorder characterized by the development of benign hamartomas in multiple organs. Most patients with TSC exhibit cutaneous manifestations. We report a 28-year-old patient with multiple pink papules at the proximal nail fold of several toes. Histopathological analysis of a biopsy of a papule was consistent with an ungual fibroma. Histopathological analysis of a biopsy of an elevated skin-colored plaque at the lower back was diagnostic for a Shagreen patch. These findings were consistent with a clinical diagnosis of TSC. This patient was subsequently referred to a multidisciplinary TSC clinic for further screening, which revealed a giant cell astrocytoma and multiple subependymal tubers. Annual monitoring was recommended. The skin lesions were treated with topical rapamycin ointment. Recognizing dermatological manifestations of TSC is of importance to allow early diagnosis. TSC should be considered as a differential diagnosis in the case of ungual fibromas, even in older patients.

A 28-Year-Old Male Patient with Nail Tumors, Skin Lesions, and Epilepsy

Directory of Open Access Journals (Sweden)

Deepak M.W. Balak

2017-01-01

Full Text Available Background: Tuberous sclerosis complex (TSC is an autosomal dominant disorder characterized by the development of benign hamartomas in multiple organs. Most patients with TSC exhibit cutaneous manifestations. Methods: We report a 28-year-old patient with multiple pink papules at the proximal nail fold of several toes. Results: Histopathological analysis of a biopsy of a papule was consistent with an ungual fibroma. Histopathological analysis of a biopsy of an elevated skin-colored plaque at the lower back was diagnostic for a Shagreen patch. These findings were consistent with a clinical diagnosis of TSC. This patient was subsequently referred to a multidisciplinary TSC clinic for further screening, which revealed a giant cell astrocytoma and multiple subependymal tubers. Annual monitoring was recommended. The skin lesions were treated with topical rapamycin ointment. Conclusions: Recognizing dermatological manifestations of TSC is of importance to allow early diagnosis. TSC should be considered as a differential diagnosis in the case of ungual fibromas, even in older patients.

Coexistence of Autism Spectrum Disorders Among Three Children with Tuberous Sclerosis Complex; Case reports and review of literature

Directory of Open Access Journals (Sweden)

Amna Al-Futaisi

2016-11-01

Full Text Available Tuberous sclerosis complex (TSC is a multisystem neurocutaneous disorder inherited in an autosomal dominant manner and characterised by benign tumours in the brain and other vital organs such as the heart, eyes, kidneys, skin and lungs. Links between autism spectrum disorder (ASD and TSC have been postulated for many decades, with TSC considered to be one of the main syndromic causes of ASD; however, precise confirmation of a relationship between these two disorders required validated diagnostic tools. Fortunately, accurate evaluation of this relationship is now possible with standardised criteria for ASD diagnosis. We report three children who presented to the Sultan Qaboos University Hospital, Muscat, Oman, between 2014 and 2015 with ASD and TSC. These cases demonstrate the spectrum of neuropsychiatric involvement in TSC and highlight the importance of screening children with TSC for ASD features in order to encourage the early enrolment of these children in educational and rehabilitation programmes.

Characterization of an acute molecular marker of nongenotoxic rodent hepatocarcinogenesis by gene expression profiling in a long term clofibric acid study.

Science.gov (United States)

Michel, Cécile; Roberts, Ruth A; Desdouets, Chantal; Isaacs, Kevin R; Boitier, Eric

2005-04-01

Evaluation of the nongenotoxic potential early during the development of a drug presents a major challenge. Recently, two genes were identified as potential molecular markers of rodent hepatic carcinogenesis: transforming growth factor-beta stimulated clone 22 (TSC-22) and NAD(P)H cytochrome P450 oxidoreductase (CYP-R) (1). They were identified after comparing the gene expression profiles obtained from the livers of Sprague-Dawley rats treated with different genotoxic and nongenotoxic compounds in a 5 day repeat dose in vivo study. To assess the potential of these two genes as acute markers of carcinogenesis, we investigated their modulation during a long-term nongenotoxic study in the rat using a classic initiation-promotion regime. Clofibric acid (CLO), which belongs to the broad class of chemicals known as peroxisome proliferators, was used as a nongenotoxic hepatocarcinogen. Male F344 rats were given a single nonnecrogenic injection of diethylnitrosamine (0 or 30 mg/kg) and fed a diet containing none or 5000 ppm CLO for up to 20 months. Necropsies of five rats per groups were performed at 18, 46, 102, 264, 377, 447 (control, DEN, and DEN + CLO rats), 524, and 608 days (for the CLO and control rats). Gross macroscopic and microscopic evaluation and gene expression profiling (on Affymetrix microarrays) were performed in peritumoral and tumoral liver tissues. Bioanalysis of the liver gene expression data revealed that TSC-22 was strongly down-regulated early in the study. Its underexpression was maintained throughout the study but disappeared upon CLO withdrawal. These modulations were confirmed by real-time polymerase chain reaction. However, CYP-R gene expression was not significantly altered in our study. Taken together, our results showed that TSC-22, but not CYP-R, has the potential to be an acute early molecular marker for nongenotoxic hepatocarcinogenesis in rodents.

Case studies of community relations on DOE's Formerly Utilized Sites Remedial Action Program as models for Superfund sites

International Nuclear Information System (INIS)

Plant, S.W.; Adler, D.G.

1995-01-01

Ever since the US Department of Energy (DOE) created its Formerly Utilized Sites Remedial Action Program (FUSRAP) in 1974, there has been a community relations program. The community relations effort has grown as FUSRAP has grown. With 20 of 46 sites now cleaned up, considerable experience in working with FUSRAP stakeholders has been gained. Why not share that experience with others who labor on the Superfund sites? Many similarities exist between the Superfund sites and FUSRAP. FUSRAP is a large, multiple-site environmental restoration program. The challenges range from small sites requiring remedial actions measurable in weeks to major sites requiring the full remedial investigation/feasibility study process. The numerous Superfund sites throughout the United States offer the same diversity, both geographically and technically. But before DOE offers FUSRAP's community relations experience as a model, it needs to make clear that this will be a realistic model. As experiences are shared, DOE will certainly speak of the efforts that achieved its goals. But many of the problems that DOE encountered along the way will also be related. FUSRAP relies on a variety of one- and two-way communication techniques for involving stakeholders in the DOE decision-making process. Some of the techniques and experiences from the case studies are presented

Site and feasibility studies of a first nuclear power plant in Morocco

International Nuclear Information System (INIS)

Righi, M.

1988-11-01

Basing on the estimated studies related to the evaluation of future needs of electrical energy, the launching of a nuclear power program in Morocco is confirmed necessary. A complete study of sites and feasibility has been carried out. Taking into account the site selection factors two sites: BIR ELHAR and SIDI BOULBRA have been kept as candidate sites. The evaluation of seismic and geotechnical risks of the power plant impact on the environment and risks related to human activities has led to privilege the SIDI BOULBRA site. The studies for confirming this site are summarized. 4 figs. (F.M.)

Tuberous sclerosis complex in a child: diagnosis and management

Directory of Open Access Journals (Sweden)

Prastiya Indra Gunawan

2010-06-01

Full Text Available Tuberous sclerosis complex (TSC is a multisystem, autosomal dominant disorder affecting children and adults, resulted from mutations in one of two genes, TSC1 (encoding hamartin or TSC2 (encoding tuberin genes located on chromosomes 9 and 16 respectively.1,2 Synonyms of TSC are Bourneville Pringle syndrome, epiloia, or tuberosclerosis. This disorder is characterized by seizures, mental disability, and small noncancerous tumors on the skin and other body tissues, such as brain, eye, lung, and kidney. The classic triad are seizures, mental retardation, and cutaneous angiofibromas.3

Reduced juvenile long-term depression in tuberous sclerosis complex is mitigated in adults by compensatory recruitment of mGluR5 and Erk signaling.

Directory of Open Access Journals (Sweden)

Wyatt B Potter

Full Text Available Tuberous sclerosis complex (TSC is a multisystem genetic disease that manifests with mental retardation, tumor formation, autism, and epilepsy. Heightened signaling through the mammalian target of rapamycin (mTOR pathway is involved in TSC pathology, however it remains unclear how other signaling pathways are perturbed and contribute to disease symptoms. Reduced long-term depression (LTD was recently reported in TSC mutant mice. We find that although reduced LTD is a feature of the juvenile mutant hippocampus, heightened expression of metabotropic glutamate receptor 5 and constitutively activated Erk signaling in the adult hippocampus drives wild-type levels of LTD. Increased mGluR5 and Erk results in a novel mTOR-independent LTD in CA1 hippocampus of adult mice, and contributes to the development of epileptiform bursting activity in the TSC2(+/- CA3 region of the hippocampus. Inhibition of mGluR5 or Erk signaling restores appropriate mTOR-dependence to LTD, and significantly reduces epileptiform bursting in TSC2(+/- hippocampal slices. We also report that adult TSC2(+/- mice exhibit a subtle perseverative behavioral phenotype that is eliminated by mGluR5 antagonism. These findings highlight the potential of modulating the mGluR5-Erk pathway in a developmental stage-specific manner to treat TSC.

Triboelectric-Based Transparent Secret Code.

Science.gov (United States)

Yuan, Zuqing; Du, Xinyu; Li, Nianwu; Yin, Yingying; Cao, Ran; Zhang, Xiuling; Zhao, Shuyu; Niu, Huidan; Jiang, Tao; Xu, Weihua; Wang, Zhong Lin; Li, Congju

2018-04-01

Private and security information for personal identification requires an encrypted tool to extend communication channels between human and machine through a convenient and secure method. Here, a triboelectric-based transparent secret code (TSC) that enables self-powered sensing and information identification simultaneously in a rapid process method is reported. The transparent and hydrophobic TSC can be conformed to any cambered surface due to its high flexibility, which extends the application scenarios greatly. Independent of the power source, the TSC can induce obvious electric signals only by surface contact. This TSC is velocity-dependent and capable of achieving a peak voltage of ≈4 V at a resistance load of 10 MΩ and a sliding speed of 0.1 m s -1 , according to a 2 mm × 20 mm rectangular stripe. The fabricated TSC can maintain its performance after reciprocating rolling for about 5000 times. The applications of TSC as a self-powered code device are demonstrated, and the ordered signals can be recognized through the height of the electric peaks, which can be further transferred into specific information by the processing program. The designed TSC has great potential in personal identification, commodity circulation, valuables management, and security defense applications.

Status of siting studies for a near surface repository site for radioactive wastes in the Philippines

International Nuclear Information System (INIS)

Valdezco, E.M.; Palattao, M.V.B.; Marcelo, E.A.; Caseria, E.S.; Venida, L.L.; Cruz, J.M. dela

2002-01-01

The Philippines, through the Philippine Nuclear Research Institute (PNRI), decided to conduct a study on siting a low level radioactive waste disposal facility. The infrastructure set up for this purpose, the radioactive waste disposal concept, the overall siting process, the methodology applied and preliminary results obtained are described in this paper. (author)

Site Study Plan for Aesthetics, Deaf Smith County Site, Texas: Environmental Field Program: Preliminary draft

International Nuclear Information System (INIS)

1987-06-01

The Aesthetic Site Study Plan describes a field program consisting of identification of the visually affected area; determination of scenic quality, visual sensitivity, and visual management classes of the site and vicinity; and analysis of the level of visual contrast that would be created by the project. Field ratings of scenic quality, visual sensitivity, and visual contrast will be supplemented by a public perception survey designed to incorporate the views of the public. This plan describes the need for the study, the study design, data management and use, schedule for proposed activities, and quality assurance program. This study will provide data needed to satisfy requirements contained in, or derived from, SRPO Requirement Document (SRP-RD). 35 refs., 6 figs., 2 tabs

Thermally stimulated current of electron beam irradiation cross-linked polyethylene, (3)

International Nuclear Information System (INIS)

Aihara, Mitsugu; Aida, Fumio; Shiono, Takeo

1984-01-01

In the past, electron-beam irradiation was for the most part applied to rather thin insulation electric cables. Considering application to thick insulation, high voltage power cables (6.6 kV or more), the authors experimented on the charge accumulation and crystallizing properties of polyethylene due to irradiation, using three differently crystallizing samples, high and low density polyethylenes (HDPE and LDPE) and straight chain low density polyethylene (LLDPE), and have obtained some findings. Those are summarized as follows. (1) The crystallizing properties (crystallinity, the size of spherulite, etc.) of polyethylene varied according to the cooling condition, and affected the thermally stimulated current (TSC). (2) In HDPE and LDPE, the behaviour of crystallization differed. In HDPE, fine crystals decreased, and spherulites significantly grew in slow cooling, but in LDPE, the generation of fine crystals and the growth of spherulites simultaneously progressed. (3) The TSC peak area for HDPE was scarcely affected by slow cooling, but that for LDPE greatly increased. (4) The TSC of irradiated polyethylene showed peaks corresponding to the melting temperatures of fine crystals and spherulites when collecting voltage Vc was lowered. (5) The above facts suggest that fine crystals and spherulites took part as charge trap sites, but the aspect of participation was different in HDPE and LDPE. (6) LLDPE has the properties of both HDPE and LDPE in view of the crystallinity, charge accumulation was small, and it was hard to be affected by cooling condition. Accordingly, it seemed to be an interesting material as the PE for irradiation. A differential scanning calorimeter and laser small angle scattering method were used for the analysis of the measured results of TSC. (Wakatsuki, Y.)

A study of moult-site fidelity in Egyptian geese, Alopochen ...

African Journals Online (AJOL)

Little is known about moult and moult-site fidelity of African waterfowl. Satellite telemetry and uniquely engraved colour-rings were used to study moult-site fidelity of Egyptian geese marked at two sites in South Africa – Barberspan in the summer-rainfall region and Strandfontein in the winter-rainfall region. Twelve Egyptian ...

Site specific study for possible ongoing salt dome movement

International Nuclear Information System (INIS)

Thoms, R.L.; Manning, T.A.; Paille, L.K.; Gehle, R.M.

1977-01-01

U.S. Gulf Coast salt domes, among other geologic structures, currently are being considered for storage of commercial radioactive wastes. A major concern with dome storage of long lived radioactive wastes lies with the possible tectonic movement of the host dome. Any ongoing movement of a salt dome can be monitored with a site specific complementary system of field instrumentation and finite element modelling. Field instrumentation and accompanying finite element analyses for a study dome in northwest Louisiana are described. Site specific data and early experience associated with tiltmeters over the dome are presented. Also, recommendations are made for modifications and extensions of the field instrumentation and finite element modelling appropriate to the specific site under study

Faslodex inhibits estradiol-induced extracellular matrix dynamics and lung metastasis in a model of lymphangioleiomyomatosis.

Science.gov (United States)

Li, Chenggang; Zhou, Xiaobo; Sun, Yang; Zhang, Erik; Mancini, John D; Parkhitko, Andrey; Morrison, Tasha A; Silverman, Edwin K; Henske, Elizabeth P; Yu, Jane J

2013-07-01

Lymphangioleiomyomatosis (LAM) is a destructive lung disease primarily affecting women. Genetic studies indicate that LAM cells carry inactivating tuberous sclerosis complex (TSC)-2 mutations, and metastasize to the lung. We previously discovered that estradiol increases the metastasis of TSC2-deficient cells in mice carrying xenograft tumors. Here, we investigate the molecular basis underlying the estradiol-induced lung metastasis of TSC2-deficient cells, and test the efficacy of Faslodex (an estrogen receptor antagonist) in a preclinical model of LAM. We used a xenograft tumor model in which estradiol induces the lung metastasis of TSC2-deficient cells. We analyzed the impact of Faslodex on tumor size, the extracellular matrix organization, the expression of matrix metalloproteinase (MMP)-2, and lung metastasis. We also examined the effects of estradiol and Faslodex on MMP2 expression and activity in tuberin-deficient cells in vitro. Estradiol resulted in a marked reduction of Type IV collagen deposition in xenograft tumors, associated with 2-fold greater MMP2 concentrations compared with placebo-treated mice. Faslodex normalized the Type IV collagen changes in xenograft tumors, enhanced the survival of the mice, and completely blocked lung metastases. In vitro, estradiol enhanced MMP2 transcripts, protein accumulation, and activity. These estradiol-induced changes in MMP2 were blocked by Faslodex. In TSC2-deficient cells, estradiol increased MMP2 concentrations in vitro and in vivo, and induced extracellular matrix remodeling. Faslodex inhibits the estradiol-induced lung metastasis of TSC2-deficient cells. Targeting estrogen receptors with Faslodex may be of efficacy in the treatment of LAM.

Faslodex Inhibits Estradiol-Induced Extracellular Matrix Dynamics and Lung Metastasis in a Model of Lymphangioleiomyomatosis

Science.gov (United States)

Li, Chenggang; Zhou, Xiaobo; Sun, Yang; Zhang, Erik; Mancini, John D.; Parkhitko, Andrey; Morrison, Tasha A.; Silverman, Edwin K.; Henske, Elizabeth P.

2013-01-01

Lymphangioleiomyomatosis (LAM) is a destructive lung disease primarily affecting women. Genetic studies indicate that LAM cells carry inactivating tuberous sclerosis complex (TSC)–2 mutations, and metastasize to the lung. We previously discovered that estradiol increases the metastasis of TSC2-deficient cells in mice carrying xenograft tumors. Here, we investigate the molecular basis underlying the estradiol-induced lung metastasis of TSC2-deficient cells, and test the efficacy of Faslodex (an estrogen receptor antagonist) in a preclinical model of LAM. We used a xenograft tumor model in which estradiol induces the lung metastasis of TSC2-deficient cells. We analyzed the impact of Faslodex on tumor size, the extracellular matrix organization, the expression of matrix metalloproteinase (MMP)–2, and lung metastasis. We also examined the effects of estradiol and Faslodex on MMP2 expression and activity in tuberin-deficient cells in vitro. Estradiol resulted in a marked reduction of Type IV collagen deposition in xenograft tumors, associated with 2-fold greater MMP2 concentrations compared with placebo-treated mice. Faslodex normalized the Type IV collagen changes in xenograft tumors, enhanced the survival of the mice, and completely blocked lung metastases. In vitro, estradiol enhanced MMP2 transcripts, protein accumulation, and activity. These estradiol-induced changes in MMP2 were blocked by Faslodex. In TSC2-deficient cells, estradiol increased MMP2 concentrations in vitro and in vivo, and induced extracellular matrix remodeling. Faslodex inhibits the estradiol-induced lung metastasis of TSC2-deficient cells. Targeting estrogen receptors with Faslodex may be of efficacy in the treatment of LAM. PMID:23526212

SITE: a methodology for assessment of energy facility siting patterns. Regional studies program

International Nuclear Information System (INIS)

Frigerio, N.A.; Habegger, L.J.; King, R.F.; Hoover, L.J.; Clark, N.A.; Cobian, J.M.

1975-08-01

The timely development of the nation's energy production capacity in a manner that minimizes potential adverse local and regional impacts associated with energy facilities requires the use of sophisticated techniques for evaluation of siting alternatives and fuel cycle options. This report is a documentation of the computerized SITE methodology that has been developed for evaluating health, environmental, and socioeconomic impacts related to utilization of alternate sites for energy production within a region of interest. The cost, impact, and attribute vectors, which are generated and displayed on density maps, can be used in a multiparameter overlay process to identify preferable siting areas. The assessment of clustered facilities in energy centers is also possible within the SITE analysis framework. An application of the SITE methodology to Northern Illinois is presented. Also included is a description of the ongoing extension of SITE for the accumulative evaluation of alternative regional energy siting patterns and fuel cycle options. An appendix provides documentation and user information for the SITE computer program

Site Study Plan for meteorology/air quality, Deaf Smith County site, Texas: Environmental Field Program: Preliminary draft

International Nuclear Information System (INIS)

1987-06-01

The Meteorological/Air Quality Site Study Plan describes a field program consisting of continuous measurements of surface (10-meter) wind speed and direction, temperature, humidity, dew point, pressure, and sensible heat flux (vertical). Air quality measurements will be limited to suspended particulate matter. After the first year of measurements, a 60-meter tower will be added to incorporate measurements needed for later modeling and dose calculations; these will include upper level winds, vertical temperature structure, and vertical wind speed. All of these measurements will be made at a site located within the 9-mi 2 site area but remote from the ESF. A second site, located near and downwind from the ESF, will monitor only particulate matter. The SSP describes the need for each study; its design and design rationale; analysis, management, and use of data, schedule of field activities, organization of field personnel and sample management, and quality assurance requirements. These studies will provide data needed to satisfy requirements contained in, or derived from the Salt Repository Project Requirements Document. 38 refs., 8 figs., 3 tabs

Imaging of tuberous sclerosis complex: a pictorial review

Energy Technology Data Exchange (ETDEWEB)

Ranke, Felipe Mussi von; Faria, Igor Murad; Zanetti, Glaucia; Marchiori, Edson, E-mail: edmarchiori@gmail.com [Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, RJ, (Brazil); Hochhegger, Bruno [Santa Casa de Porto Alegre, Porto Alegre, RS (Brazil); Souza Junior, Arthur Soares [Faculdade de Medicina de Sao Jose do Rio Preto (FAMERP) , SP (Brazil)

2017-01-15

Tuberous sclerosis complex (TSC) is a genetically determined hamartomatous neurocutaneous disease with high phenotypic variability. TSC is characterized by widespread hamartomas and benign, or rarely malignant, neoplasms distributed in several organs throughout the body, especially in the brain, skin, retina, kidney, heart, and lung. Common manifestations include cortical tubers, subependymal nodules, white matter abnormalities, retinal abnormalities, cardiac rhabdomyoma, lymphangioleiomyomatosis, renal angiomyolipoma, and skin lesions. The wide range of organs affected by the disease implies that TSC1 and TSC2 genes play important roles in the regulation of cell proliferation and differentiation. Although recent advances in treatment have improved morbidity, the prognosis remains quite poor and nearly 40% of patients die by the age of 35 years. Imaging is important in the evaluation of TSC because of its role not only in presumptive diagnosis, but also in defining the full extent of involvement. This information allows a better understanding of the behavioural phenotype, as related to lesion location. Imaging also contributes to treatment planning. This pictorial review describes common and uncommon imaging manifestations of TSC. (author)

mTORC1 Inhibition Corrects Neurodevelopmental and Synaptic Alterations in a Human Stem Cell Model of Tuberous Sclerosis

Directory of Open Access Journals (Sweden)

Veronica Costa

2016-04-01

Full Text Available Hyperfunction of the mTORC1 pathway has been associated with idiopathic and syndromic forms of autism spectrum disorder (ASD, including tuberous sclerosis, caused by loss of either TSC1 or TSC2. It remains largely unknown how developmental processes and biochemical signaling affected by mTORC1 dysregulation contribute to human neuronal dysfunction. Here, we have characterized multiple stages of neurogenesis and synapse formation in human neurons derived from TSC2-deleted pluripotent stem cells. Homozygous TSC2 deletion causes severe developmental abnormalities that recapitulate pathological hallmarks of cortical malformations in patients. Both TSC2+/− and TSC2−/− neurons display altered synaptic transmission paralleled by molecular changes in pathways associated with autism, suggesting the convergence of pathological mechanisms in ASD. Pharmacological inhibition of mTORC1 corrects developmental abnormalities and synaptic dysfunction during independent developmental stages. Our results uncouple stage-specific roles of mTORC1 in human neuronal development and contribute to a better understanding of the onset of neuronal pathophysiology in tuberous sclerosis.

Towards the Study of the Early Medieval Site Bashanta-II

Directory of Open Access Journals (Sweden)

Ochir-Goryaeva Maria A.

2016-12-01

Full Text Available The authors offer preliminary results on study of a new early medieval site discovered in the Gorodovikovo District (Kalmykia during a field survey near Bashanta-I hillfort at Chapaevskoe Lake (or Tsagan nur in Kalmyk language. The new site is found 8 km to the south-west from Bashanta-I hillfort, on the bank of the Egorlyk River. The site is dated by two radiocarbon dates by the middle of the 7th – late 8th centuries, i.e. by the time of Khazar Khanate. Some preliminary studies on the site included topographical mapping, collection of stray finds and a few prospection pits. These activities yielded numerous ceramic sherds, roof tiles and debris of masonry. Judging by the existing cultural stratum, remains of stone structures made of shell stone blocks and numerous ceramic finds of good quality, the new site was a settlement of Saltovo-Mayaki Culture, presumably a satellite hillfort. The identity of ceramics and construction materials allowed the authors to name this new site as Bashanta-II.

Site Study Plan for salt, Deaf Smith County site, Texas: Environmental Field Program: Preliminary draft

International Nuclear Information System (INIS)

1987-06-01

The Salt Site Study Plan (SSP) describes a program for characterizing the existing salt environment in the site vicinity. A step-by-step approach is described which proceeds from published data and planned theoretical studies, to planned laboratory studies, and finally to planned field studies, to provide the necessary data to meet program requirements contained in the Salt Repository Project - Requirements Document (SRP-RD). The plan also draws on the results of other SSP's for certain data; for example, soil salinity data are to be provided under the Soils SSP. The salt studies consist of evaluation of control and mitigation measures, salt monitoring studies, emission factors development, air models development and validation, and risk assessment. For each study, its design and design rationale; analysis, management, and use of data; schedule of activities; organization of personnel and sample management; and quality assurance requirements are described. 90 refs., 9 figs., 7 tabs

The glass block site radionuclide migration study

International Nuclear Information System (INIS)

Killey, R.W.D.; Champ, D.R.

1990-01-01

In 1960 25 nepheline syenite glass blocks containing 14 TBq of mixed fission products in 50 kg of glass were placed below the water table in a shallow sand aquifer at Chalk River Laboratories. Experimental studies undertaken at the site since 1960 have included detailed mapping of the plume of 90 Sr in 1963, 1966 and 1971. Mathematical modeling studies have employed the radiostrontium plume data in determining the split between ion exchange and chemisorption of 90 Sr, and in obtaining reaction rate data for chemisorption. The distribution of 137 Cs on downgradient soils was mapped in 1963 and 1979. An extended plume of low-level 137 Cs contamination observed in the 1979 study prompted an investigation of the role of particulate materials in radionuclide transport. IN 1983, large volume groundwater sampling and separation of cationic, anionic, and neutral dissolved species, as well as particulates, detected anionic and cationic dissolved europium isotopes (154 and 155), and again encountered particulate 137 Cs. A variety of investigations of cesium and strontium sorption have provided a data base on sediment mineralogy, particle surface features, and information on sorption sites and processes. The year 1990 saw the inauguration of a three-year program to update investigations of radionuclide release, transport, and sorption at the glass block site. The first stage of the program has been a detailed definition and simulation of the hydrogeologic setting. Plume mapping and aqueous speciation studies are in progress. This paper summarizes past investigations, reviews the status of the current program, and discusses components of future studies, including investigations of sediment sorption mechanisms. (Author) (17 refs., 8 figs.)

Evaluation of SAMG effectiveness in view of group decision

International Nuclear Information System (INIS)

Huh, Chang Wook; Suh, Nam Duck; Park, Goon Cherl

2012-01-01

We evaluate the technical and organizational aspects of the severe accident management guideline (SAMG), focusing on the decision-making process in the technical support center (TSC). From the technical aspects, we conclude that the present SAMG is a good tool that can assist the TSC in efficiently managing probable severe accidents. However, we suggest that the clear separation of the emergency operating procedure (EOP) and SAMG, which shifts plant control from the main control room (MCR) to the TSC, might not be an effective framework from an organizational perspective. Studies on organizational behavior demonstrate that a group decision made under a risky situation might be polarized in either a risky or cautious way. We recognize that we cannot be free from the polarization effect since the current SAMG recommends that the TSC evaluate the advantages and disadvantages of strategies to be implemented and choose the best one based on a group decision process. Illustrative examples of accident management under risky conditions are recapitulated from previous studies of the authors and we propose that the SAMG should be more proceduralized to remove this polarization from the decision-making process.

Evaluation of SAMG effectiveness in view of group decision

Energy Technology Data Exchange (ETDEWEB)

Huh, Chang Wook; Suh, Nam Duck [Korea Institute of Nuclear Safety, Daejeon (Korea, Republic of); Park, Goon Cherl [Seoul National University, Seoul (Korea, Republic of)

2012-08-15

We evaluate the technical and organizational aspects of the severe accident management guideline (SAMG), focusing on the decision-making process in the technical support center (TSC). From the technical aspects, we conclude that the present SAMG is a good tool that can assist the TSC in efficiently managing probable severe accidents. However, we suggest that the clear separation of the emergency operating procedure (EOP) and SAMG, which shifts plant control from the main control room (MCR) to the TSC, might not be an effective framework from an organizational perspective. Studies on organizational behavior demonstrate that a group decision made under a risky situation might be polarized in either a risky or cautious way. We recognize that we cannot be free from the polarization effect since the current SAMG recommends that the TSC evaluate the advantages and disadvantages of strategies to be implemented and choose the best one based on a group decision process. Illustrative examples of accident management under risky conditions are recapitulated from previous studies of the authors and we propose that the SAMG should be more proceduralized to remove this polarization from the decision-making process.

Inhibition of bovine viral diarrhea virus RNA synthesis by thiosemicarbazone derived from 5,6-dimethoxy-1-indanone.

Science.gov (United States)

Castro, Eliana F; Fabian, Lucas E; Caputto, María E; Gagey, Dolores; Finkielsztein, Liliana M; Moltrasio, Graciela Y; Moglioni, Albertina G; Campos, Rodolfo H; Cavallaro, Lucía V

2011-06-01

In the present work, we described the activity of the thiosemicarbazone derived from 5,6-dimethoxy-1-indanone (TSC), which we previously characterized as a new compound that inhibits bovine viral diarrhea virus (BVDV) infection. We showed that TSC acts at a point of time that coincides with the onset of viral RNA synthesis and that it inhibits the activity of BVDV replication complexes (RCs). Moreover, we have selected five BVDV mutants that turned out to be highly resistant to TSC but still susceptible to ribavirin (RBV). Four of these resistant mutants carried an N264D mutation in the viral RNA-dependent RNA polymerase (RdRp). The remaining mutant showed an A392E mutation within the same protein. Some of these mutants replicated slower than the wild-type (wt) virus in the absence of TSC, whereas others showed a partial reversion to the wt phenotype over several passages in the absence of the compound. The docking of TSC in the crystal structure of the BVDV RdRp revealed a close contact between the indane ring of the compound and several residues within the fingers domain of the enzyme, some hydrophobic contacts, and hydrogen bonds with the thiosemicarbazone group. Finally, in the mutated RdRp from resistant BVDV, these interactions with TSC could not be achieved. Interestingly, TSC inhibited BVDV replication in cell culture synergistically with RBV. In conclusion, TSC emerges as a new nonnucleoside inhibitor of BVDV RdRp that is synergistic with RBV, a feature that turns it into a potential compound to be evaluated against hepatitis C virus (HCV).

Siting study for small platform-mounted industrial energy reactors

International Nuclear Information System (INIS)

1975-07-01

Utilizing an existing 313 MW(t) ship propulsion reactor design, a concept has been formulated for a floating platform-mounted nuclear plant and an evaluation has been made to determine reductions in construction time and cost achievable by repetitive platform construction in a shipyard. Concepts and estimates are presented for siting platform-mounted nuclear plants at the location of industrial facilities where the nuclear plants would furnish industrial process heat and/or electrical power. The representative industrial site designated for this study is considered typical of sites that might be used along the extensive network of navigable canals adjacent to the ocean and is similar to potential sites along the inland waterways of the United States

Archaeometric studies on the Hatahara archaeological site

International Nuclear Information System (INIS)

Nunes, Kelly Placa

2009-01-01

The reconstruction of the past and the understanding of historical and cultural aspects of societies that developed at archaeological sites have been enabled by archaeometric studies undertaken on ceramics located at these areas. This study aims to be a contribution to the elucidation of these aspects with the application of three physical methods of analysis: neutron activation analysis (NAA), thermoluminescence dating (TL) an electronic paramagnetic resonance (EPR) to ceramic fragments from the Hatahara archaeological site, located at central Amazon. The elemental concentrations obtained by NAA for 120 ceramic fragments were interpreted by means of cluster analysis and discriminant analysis. The results showed the existence of five distinct ceramic groups. This information, supported by archaeological interpretation, confirm the existence of four distinct occupation Phases at Hatahara site. In order to establish a chronology for the occupations, the ages of three ceramic fragments were determined by TL. The dating of two fragments did not confirm the archaeological interpretation about their occupation Phases. However, the dating of the third fragment allowed the confirmation that it belongs to the Manacapuru Phase. The determination of the burning temperatures of four ceramic fragments was performed by EPR. It was observed that although the analyzed ceramic samples belong to three distinct groups, there was no significant variation on their burning temperatures. (author)

Site study plan for utilities and solid waste, Deaf Smith County Site, Texas: Environmental Field Program: Preliminary draft

International Nuclear Information System (INIS)

1987-06-01

This site plan describes utilities and solid waste studies to be conducted during the characterization of the Deaf Smith County, Texas, site for the US Department of Energy's Salt Repository Project. After utilities and solid waste information needs derived from Federal, State, and local statutes and regulations and the project specifications are briefly described, the site study plan describes the study design and rationale, the field data collection procedures and equipment, and data analysis methods and application of results, the data management strategy, the schedule of field activities, the management of the study, and the study's quality assurance program. The field data collection activities are organized into programs to characterize electrical power, natural gas, communication, water, wastewater sludge, nonradiological solid waste, nonradiological hazardous waste, and low-level radiological waste. These programs include details for the collection of project needs, identification of utilities and solid waste disposal contractor capabilities, and verification of the obtained data. Utilities and solid waste field activities will begin approximately at the time of site access. Utilities and solid waste characterization will be completed within the first year of activity. 29 refs., 6 figs., 2 tabs

Tokamak Simulation Code modeling of NSTX

International Nuclear Information System (INIS)

Jardin, S.C.; Kaye, S.; Menard, J.; Kessel, C.; Glasser, A.H.

2000-01-01

The Tokamak Simulation Code [TSC] is widely used for the design of new axisymmetric toroidal experiments. In particular, TSC was used extensively in the design of the National Spherical Torus eXperiment [NSTX]. The authors have now benchmarked TSC with initial NSTX results and find excellent agreement for plasma and vessel currents and magnetic flux loops when the experimental coil currents are used in the simulations. TSC has also been coupled with a ballooning stability code and with DCON to provide stability predictions for NSTX operation. TSC has also been used to model initial CHI experiments where a large poloidal voltage is applied to the NSTX vacuum vessel, causing a force-free current to appear in the plasma. This is a phenomenon that is similar to the plasma halo current that sometimes develops during a plasma disruption

Site qualification studies of the UCG-SDB at North Knobs, Wyoming

Energy Technology Data Exchange (ETDEWEB)

Davis, B.E.; Krajewski, S.A.; Ahner, P.F.; Avasthi, J.M.; Dolde, M.E.; Greenman, C.A.; Miranda, J.E.

1979-01-01

The site qualification program for the North Knobs UCG site near Rawlins, Wyoming has been completed. This site will be the location for the field tests of Underground Coal Gasification of Steeply Dipping Beds undertaken by Gulf Research and Development Company for DOE in a cost shared contract. Site characterization included a comprehensive geotechnical analysis along with vegetation, historical, and archeological studies. The G coal seam chosen for these tests is a subbituminous B coal with a true seam thickness of 22 feet and has thin coal benches above and below the main seam. The water table is at 90 feet below the surface. Hydrologic studies have defined the seam as an aquiclude (non-aquifer). The site is deemed restorable to regulatory requirements. Evaluation of this site indicates total acceptability for the three-test program planned by GR and DC.

Feasibility study for siting of a deep repository within the Storuman municipality

International Nuclear Information System (INIS)

1995-01-01

It is SKB's task to gather a broad body of information for the siting of a deep repository for Sweden's spent nuclear fuel and other long-lived radioactive waste. The criteria presented by SKB serve as guidelines for the siting studies. Overviews of siting factors are compiled in general siting studies of all or parts of Sweden. Feasibility studies examine the siting prospects in potentially suitable and interested municipalities. SKB plans to carry out feasibility studies in five to ten municipalities in different parts of the country. General studies and feasibility studies are primarily based on existing material. The feasibility study in Storuman is the first to be conducted. It began during the second half of 1993 with the conclusion of an agreement between the municipality and SKB. SKB has been in charge of the execution of the feasibility study. SKB has been responsible for the summary provided in a status report published in June 1994, as well as for the conclusions and evaluations in this final report. Altogether about 30 reports have been published within the framework of the feasibility study. The purpose of the various studies have been to describe, in as much detail as possible, the prospects for siting a deep repository in the municipality of Storuman, and to shed light on the possible positive and negative consequences of such a siting. The feasibility study has led to a discussion within the municipality and the region concerning the siting of a deep repository. In SKB's opinion, this gives all those concerned ample opportunity to become acquainted with the issues and to assert their interests and present their viewpoints at an early stage. 55 refs

Status report on geochemical field results from Atlantic study sites

International Nuclear Information System (INIS)

Wilson, T.R.S.; Thomson, J.; Hydes, D.J.; Colley, S.

1983-01-01

This report summarises the results of preliminary geochemical investigations at three North Atlantic study areas. The two eastern sites, on the Cape Verde abyssal plain (CV2) and east of Great Meteor Seamount (GME) were visited during 1982. The results presented are preliminary. Studies in the western Atlantic, close to the Nares Abyssal Plain study site are more detailed and are presented in a separate paper. The report shows for the first time the relative redox status of the three sites. The differences are unexpectedly large, the most reduced cores being recovered at GME and the most oxidised at CV2. The sporadic nature of Recent sediment accumulation at these sites is also emphasised. In order to place these preliminary results in context their relevance to the production of mathematical system models is discussed in a closing section. The necessity for such models to rest on sound foundations of geochemical understanding is noted. Suggestions on future research priorities are offered for discussion. (author)

Study on Site Conditions Based on Topographic Slope

Science.gov (United States)

Wu, X.; Wang, X.; Yuan, X.; Chen, M.; Dou, A.

2018-04-01

The travel-time averaged shear-wave velocity to a depth of 30m (Vs30) below the Earth's surface is widely used to classify sites in many building codes. Vs30 is also used to estimate site classification in recent ground-motion prediction equations (GMPEs), and the distribution of Vs30 has been mapped in a region or country. An alternative method has recently been proposed for evaluating global seismic site conditions or Vs30, from the SRTM (Shuttle Radar Topography Mission) DEMs (digital elevation models). The basic premise of the method is that the topographic slope can be used as a reliable proxy for Vs30 in the absence of geologically and geotechnically based site-condition maps through correlations between Vs30 measurements and topographic gradient. Here, we use different resolutions (3 arcsec, 30 arcsec) DEM data to get Vs30 data separately, analyze and compare the difference of Vs30 data and site conditions obtained from different resolution DEM data. Shandong Province in eastern China and Sichuan Province in Western China are studied respectively. It is found that the higher resolution data is better at defining spatial topographic features than the 30c data, but less improvement in its correlation with Vs30.

STUDY ON SITE CONDITIONS BASED ON TOPOGRAPHIC SLOPE

Directory of Open Access Journals (Sweden)

X. Wu

2018-04-01

Full Text Available The travel-time averaged shear-wave velocity to a depth of 30m (Vs30 below the Earth’s surface is widely used to classify sites in many building codes. Vs30 is also used to estimate site classification in recent ground-motion prediction equations (GMPEs, and the distribution of Vs30 has been mapped in a region or country. An alternative method has recently been proposed for evaluating global seismic site conditions or Vs30, from the SRTM (Shuttle Radar Topography Mission DEMs (digital elevation models. The basic premise of the method is that the topographic slope can be used as a reliable proxy for Vs30 in the absence of geologically and geotechnically based site-condition maps through correlations between Vs30 measurements and topographic gradient. Here, we use different resolutions (3 arcsec, 30 arcsec DEM data to get Vs30 data separately, analyze and compare the difference of Vs30 data and site conditions obtained from different resolution DEM data. Shandong Province in eastern China and Sichuan Province in Western China are studied respectively. It is found that the higher resolution data is better at defining spatial topographic features than the 30c data, but less improvement in its correlation with Vs30.

Feasibility study for siting of a deep repository within the Storuman municipality

Energy Technology Data Exchange (ETDEWEB)

NONE

1995-01-01

It is SKB`s task to gather a broad body of information for the siting of a deep repository for Sweden`s spent nuclear fuel and other long-lived radioactive waste. The criteria presented by SKB serve as guidelines for the siting studies. Overviews of siting factors are compiled in general siting studies of all or parts of Sweden. Feasibility studies examine the siting prospects in potentially suitable and interested municipalities. SKB plans to carry out feasibility studies in five to ten municipalities in different parts of the country. General studies and feasibility studies are primarily based on existing material. The feasibility study in Storuman is the first to be conducted. It began during the second half of 1993 with the conclusion of an agreement between the municipality and SKB. SKB has been in charge of the execution of the feasibility study. SKB has been responsible for the summary provided in a status report published in June 1994, as well as for the conclusions and evaluations in this final report. Altogether about 30 reports have been published within the framework of the feasibility study. The purpose of the various studies have been to describe, in as much detail as possible, the prospects for siting a deep repository in the municipality of Storuman, and to shed light on the possible positive and negative consequences of such a siting. The feasibility study has led to a discussion within the municipality and the region concerning the siting of a deep repository. In SKB`s opinion, this gives all those concerned ample opportunity to become acquainted with the issues and to assert their interests and present their viewpoints at an early stage. 55 refs.

SUPERFUND TREATABILITY CLEARINGHOUSE: SOIL STABILIZATION PILOT STUDY, UNITED CHROME NPL SITE PILOT STUDY AND HEALTH AND SAFETY PROGRAM, UNITED CHROME NPL SITE PILOT STUDY

Science.gov (United States)

This document is a project plan for a pilot study at the United Chrome NPL site, Corvallis, Oregon and includes the health and safety and quality assurance/quality control plans. The plan reports results of a bench-scale study of the treatment process as iieasured by the ...

Thermal spray coating for corrosion under insulation (CUI) prevention

Science.gov (United States)

Fuad, Mohd Fazril Irfan Ahmad; Razak, Khalil Abdul; Alias, Nur Hashimah; Othman, Nur Hidayati; Lah, Nik Khairul Irfan Nik Ab

2017-12-01

Corrosion under insulation (CUI) is one of the predominant issues affecting process of Oil and Gas and Petrochemical industries. CUI refers to external corrosion, but it is difficult to be detected as the insulation cover masks the corrosion problem. One of the options to prevent CUI is by utilizing the protective coating systems. Thermal spray coating (TSC) is an advanced coating system and it shows promising performance in harsh environment, which could be used to prevent CUI. However, the application of TSC is not attractive due to the high initial cost. This work evaluates the potential of TSC based on corrosion performance using linear polarization resistance (LPR) method and salt spray test (SST). Prior to the evaluation, the mechanical performance of TSC was first investigated using adhesion test and bend test. Microstructure characterization of the coating was investigated using Scanning Electron Microscope (SEM). The LPR test results showed that low corrosion rate of 0.05 mm/years was obtained for TSC in compared to the bare steel especially at high temperature of 80 °C, where usually normal coating would fail. For the salt spray test, there was no sign of corrosion products especially at the center (fully coated region) was observed. From SEM images, no corrosion defects were observed after 336 hours of continuous exposure to salt fog test. This indicates that TSC protected the steel satisfactorily by acting as a barrier from a corrosive environment. In conclusion, TSC can be a possible solution to minimize the CUI in a long term. Further research should be done on corrosion performance and life cycle cost by comparing TSC with other conventional coating technology.

Inhibition of Bovine Viral Diarrhea Virus RNA Synthesis by Thiosemicarbazone Derived from 5,6-Dimethoxy-1-Indanone▿

Science.gov (United States)

Castro, Eliana F.; Fabian, Lucas E.; Caputto, María E.; Gagey, Dolores; Finkielsztein, Liliana M.; Moltrasio, Graciela Y.; Moglioni, Albertina G.; Campos, Rodolfo H.; Cavallaro, Lucía V.

2011-01-01

In the present work, we described the activity of the thiosemicarbazone derived from 5,6-dimethoxy-1-indanone (TSC), which we previously characterized as a new compound that inhibits bovine viral diarrhea virus (BVDV) infection. We showed that TSC acts at a point of time that coincides with the onset of viral RNA synthesis and that it inhibits the activity of BVDV replication complexes (RCs). Moreover, we have selected five BVDV mutants that turned out to be highly resistant to TSC but still susceptible to ribavirin (RBV). Four of these resistant mutants carried an N264D mutation in the viral RNA-dependent RNA polymerase (RdRp). The remaining mutant showed an A392E mutation within the same protein. Some of these mutants replicated slower than the wild-type (wt) virus in the absence of TSC, whereas others showed a partial reversion to the wt phenotype over several passages in the absence of the compound. The docking of TSC in the crystal structure of the BVDV RdRp revealed a close contact between the indane ring of the compound and several residues within the fingers domain of the enzyme, some hydrophobic contacts, and hydrogen bonds with the thiosemicarbazone group. Finally, in the mutated RdRp from resistant BVDV, these interactions with TSC could not be achieved. Interestingly, TSC inhibited BVDV replication in cell culture synergistically with RBV. In conclusion, TSC emerges as a new nonnucleoside inhibitor of BVDV RdRp that is synergistic with RBV, a feature that turns it into a potential compound to be evaluated against hepatitis C virus (HCV). PMID:21430053

Studies on site characterization methodologies for high level radioactive waste disposal

International Nuclear Information System (INIS)

Wang Ju; Guo Yonghai; Chen Weiming

2008-01-01

This paper presents the final achievement of the project 'Studies of Site-specific Geological Environment for High Level Waste Disposal and Performance Assessment Methodology, Part Ⅰ: Studies on Site Characterization Methodologies for High Level Radioactive Waste Disposal', which is a 'Key Scientific and Technological Pre-Research Project for National Defense' during 2001-2005. The study area is Beishan area, Gansu Province, NW China--the most potential site for China's underground research laboratory and high level radioactive waste repository. The boreholes BS01, BS2, BS03 and BS04 drilled in fractured granite media in Beishan are used to conduct comprehensive studies on site characterization methodologies, including: bore hole drilling method, in situ measurement methods of hydrogeological parameters, underground water sampling technology, hydrogeochemical logging method, geo-stress measurement method, acoustic borehole televiewer measurement method, borehole radar measurement method, fault stability evaluation methods and rock joint evaluation method. The execution of the project has resulted in the establishment of an 'Integrated Methodological System for Site Characterization in Granite Site for High Level Radioactive Waste Repository' and the 8 key methodologies for site characterization: bore hole drilling method with minimum disturbance to rock mass, measurement method for hydrogeological parameters of fracture granite mass, in situ groundwater sampling methods from bore holes in fractured granite mass, fracture measurement methods by borehole televiewer and bore radar system, hydrogeochemical logging, low permeability measurement methods, geophysical methods for rock mass evaluation, modeling methods for rock joints. Those methods are comprehensive, advanced, innovative, practical, reliable and of high accuracy. The comprehensive utilization of those methods in granite mass will help to obtain systematic parameters of

Toward a regional power plant siting method: AEC-Maryland regional siting factors study, FY 1974 progress report

International Nuclear Information System (INIS)

Yaffee, S.L.; Miller, C.A.

1974-11-01

The ''AEC-Maryland Regional Siting Factors Study'' examines the process of siting in a regional context. It is developing an analysis method to delineate candidate areas for siting of several power plant technology packages, including both fossil-fueled and nuclear options. Tools that are being used include simulation modeling, economic and demographic forecasting, spatial analysis, and computer graphics and numerical manipulation. The approach will describe the trade-offs incurred if a power plant is located in one candidate area rather than in another. In FY 1974, a suitability analysis method was developed which uses engineering and environmental parameters to define a level of environmental cost incurred if a segment of land is used to site a specific technology package. (U.S.)

Energy-conserving site-design case study, Radisson, New York. Final report

Energy Technology Data Exchange (ETDEWEB)

1979-12-01

Radisson is a 2,950-acre new community currently being developed by the New York State Urban Development Corporation and located in central New York, 12 miles north-west of Syracuse. Case-study sites selected for this project are a 95-acre residential site and the 51-acre Town Center of the new community. Development on the Residential Site is a low-density (2.8 dwelling units/acre) mixture of single-family, townhouse and multi-family units. Development on the Town Center site is a mixture of small-scale commercial use (144,000 sq. ft.) and 330+ multi-family dwelling units. Energy-conserving plans developed for both sites have focused on passive measures to reduce energy use for space heating. Utility-system options have been identified for both sites, but require further study as to feasibility and cost. This report summarizes energy savings and cost differentials due to passive measures incorporated in both the residential and Town Center Plans. The future implementation schedule, also discussed, summarizes the procedures an schedule required for implementation of the passive measures, as well as further study required for the development of utility-system options. 4 tables.

Thermally stimulated current method applied to highly irradiated silicon diodes

CERN Document Server

Pintilie, I; Pintilie, I; Moll, Michael; Fretwurst, E; Lindström, G

2002-01-01

We propose an improved method for the analysis of Thermally Stimulated Currents (TSC) measured on highly irradiated silicon diodes. The proposed TSC formula for the evaluation of a set of TSC spectra obtained with different reverse biases leads not only to the concentration of electron and hole traps visible in the spectra but also gives an estimation for the concentration of defects which not give rise to a peak in the 30-220 K TSC temperature range (very shallow or very deep levels). The method is applied to a diode irradiated with a neutron fluence of phi sub n =1.82x10 sup 1 sup 3 n/cm sup 2.

Role of CTGF in White Matter Development in Tuberous Sclerosis

Science.gov (United States)

2016-04-01

which lacks Tsc1 expression only in neurons . Here we show that, neurons lacking Tsc1 secrete excessive amounts of connective tissue growth factor...that, neurons lacking Tsc1 secrete excessive amounts of connective tissue growth factor (CTGF), which in turn blocks the maturation of...Columbia University Medical Center New York, NY 2015 Neuronal Connectivity in Tuberous Sclerosis / Keynote Stanley Manne Children’s Research Institute

Hypoxic stress induces, but cannot sustain trophoblast stem cell differentiation to labyrinthine placenta due to mitochondrial insufficiency

Directory of Open Access Journals (Sweden)

Yufen Xie

2014-11-01

Full Text Available Dysfunctional stem cell differentiation into placental lineages is associated with gestational diseases. Of the differentiated lineages available to trophoblast stem cells (TSC, elevated O2 and mitochondrial function are necessary to placental lineages at the maternal–placental surface and important in the etiology of preeclampsia. TSC lineage imbalance leads to embryonic failure during uterine implantation. Stress at implantation exacerbates stem cell depletion by decreasing proliferation and increasing differentiation. In an implantation site O2 is normally ~2%. In culture, exposure to 2% O2 and fibroblast growth factor 4 (FGF4 enabled the highest mouse TSC multipotency and proliferation. In contrast, hypoxic stress (0.5% O2 initiated the most TSC differentiation after 24 h despite exposure to FGF4. However, hypoxic stress supported differentiation poorly after 4–7 days, despite FGF4 removal. At all tested O2 levels, FGF4 maintained Warburg metabolism; mitochondrial inactivity and aerobic glycolysis. However, hypoxic stress suppressed mitochondrial membrane potential and maintained low mitochondrial cytochrome c oxidase (oxidative phosphorylation/OxPhos, and high pyruvate kinase M2 (glycolysis despite FGF4 removal. Inhibiting OxPhos inhibited optimum differentiation at 20% O2. Moreover, adding differentiation-inducing hyperosmolar stress failed to induce differentiation during hypoxia. Thus, differentiation depended on OxPhos at 20% O2; hypoxic and hyperosmolar stresses did not induce differentiation at 0.5% O2. Hypoxia-limited differentiation and mitochondrial inhibition and activation suggest that differentiation into two lineages of the labyrinthine placenta requires O2 > 0.5–2% and mitochondrial function. Stress-activated protein kinase increases an early lineage and suppresses later lineages in proportion to the deviation from optimal O2 for multipotency, thus it is the first enzyme reported to prioritize differentiation.

Dermatologic and dental aspects of the 2012 International Tuberous Sclerosis Complex Consensus Statements.

Science.gov (United States)

Teng, Joyce M C; Cowen, Edward W; Wataya-Kaneda, Mari; Gosnell, Elizabeth S; Witman, Patricia M; Hebert, Adelaide A; Mlynarczyk, Greg; Soltani, Keyoumars; Darling, Thomas N

2014-10-01

The 2012 International Tuberous Sclerosis Complex Clinical Consensus Conference was convened to update the last consensus statement in 1998. Skin and dental lesions are common in tuberous sclerosis complex (TSC) and are a frequent concern for patients. Recognition of these lesions is imperative for early diagnosis, given the treatment advances that may improve patient outcomes. To detail recommendations for the diagnosis, surveillance, and management of skin and dental lesions in TSC. The TSC Dermatology and Dentistry Subcommittee, 1 of 12 subcommittees, reviewed the relevant literature from 1997 to 2012. A consensus on skin and dental issues was achieved within the Dermatology and Dentistry Subcommittee before recommendations were presented, discussed, and agreed on in a group meeting of all subcommittees from June 14 to 15, 2012. Skin and dental findings comprise 4 of 11 major features and 3 of 6 minor features in the diagnostic criteria. A definite diagnosis of TSC is defined as the presence of at least 2 major features or 1 major and 2 or more minor features; in addition, a pathological mutation in TSC1 or TSC2 is diagnostic. Skin and oral examinations should be performed annually and every 3 to 6 months, respectively. Intervention may be indicated for TSC skin or oral lesions that are bleeding, symptomatic, disfiguring, or negatively affecting function. Options presented include surgical excision, laser(s), or use of a mammalian target of rapamycin inhibitor.

Radionuclide migration studies at the Nevada Test Site

International Nuclear Information System (INIS)

Thompson, J.L.

1989-01-01

The United States government routinely tests nuclear devices at the Nevada Test Site (NTS) in southern Nevada. A significant amount of radioactive material exists underground at the NTS with no containers or engineered barriers to inhibit its subsequent migration. The Department of Energy has sponsored for many years a research program on radionuclide movement in the geologic media at this location. Goals of this research program are to measure the extent of movement of radionuclides away from underground explosion sites and to determine the mechanisms by which such movement occurs. This program has acquired significance in another aspect of nuclear waste management because of the Yucca Mountain Project. Yucca Mountain at the NTS is being intensively studied as the possible site for a mined repository for high level nuclear waste. The NTS provides a unique setting for field studies concerning radionuclide migration; there is the potential for greatly increasing our knowledge of the behavior of radioactive materials in volcanogenic media. This review summarizes some of the significant findings made under this research program at the NTS and identifies reports in which the details of the research may be found. 36 refs., 4 figs

Case study: Montclair/West Orange and Glen Ridge Radium Superfund sites

International Nuclear Information System (INIS)

Pezzella, R.; Seppi, P.; Watson, D.

1994-01-01

The Montclair/West Orange and Glen Ridge Radium Sites are located 12 miles west of New York City in three residential communities in Essex County, New Jersey. The sites are contaminated with waste materials from a local radium processing facility which ceased operations in 1926. Houses were subsequently constructed on or near the radium waste disposal areas. The waste material was also used as backfill, which caused contamination to be spread randomly over the communities. There are 769 properties between four townships that comprise the Superfund sites. The Environmental Protection Agency (EPA) conducted an aerial survey in 1981 which identified the boundaries of the sites. In 1985, the New Jersey Department of Environmental Protection (NJDEP) began a pilot study to examine the feasibility of excavation and off-site disposal of contaminated material as a permanent solution. The study was interrupted when the permit for the disposal site was revoked by the state of Nevada. Since 1990 field testing has been completed on over 725 properties and remediation and restoration has been completed on 75 properties

Studies on Labour Safety in Construction Sites

Directory of Open Access Journals (Sweden)

S. Kanchana

2015-01-01

Full Text Available Construction industry has accomplished extensive growth worldwide particularly in past few decades. For a construction project to be successful, safety of the structures as well as that of the personnel is of utmost importance. The safety issues are to be considered right from the design stage till the completion and handing over of the structure. Construction industry employs skilled and unskilled labourers subject to construction site accidents and health risks. A proper coordination between contractors, clients, and workforce is needed for safe work conditions which are very much lacking in Indian construction companies. Though labour safety laws are available, the numerous accidents taking place at construction sites are continuing. Management commitment towards health and safety of the workers is also lagging. A detailed literature study was carried out to understand the causes of accidents, preventive measures, and development of safe work environment. This paper presents the results of a questionnaire survey, which was distributed among various categories of construction workers in Kerala region. The paper examines and discusses in detail the total working hours, work shifts, nativity of the workers, number of accidents, and type of injuries taking place in small and large construction sites.

Studies on Labour Safety in Construction Sites

Science.gov (United States)

Kanchana, S.; Sivaprakash, P.; Joseph, Sebastian

2015-01-01

Construction industry has accomplished extensive growth worldwide particularly in past few decades. For a construction project to be successful, safety of the structures as well as that of the personnel is of utmost importance. The safety issues are to be considered right from the design stage till the completion and handing over of the structure. Construction industry employs skilled and unskilled labourers subject to construction site accidents and health risks. A proper coordination between contractors, clients, and workforce is needed for safe work conditions which are very much lacking in Indian construction companies. Though labour safety laws are available, the numerous accidents taking place at construction sites are continuing. Management commitment towards health and safety of the workers is also lagging. A detailed literature study was carried out to understand the causes of accidents, preventive measures, and development of safe work environment. This paper presents the results of a questionnaire survey, which was distributed among various categories of construction workers in Kerala region. The paper examines and discusses in detail the total working hours, work shifts, nativity of the workers, number of accidents, and type of injuries taking place in small and large construction sites. PMID:26839916

Rare lung manifestation of multifocal micronodular pneumocyte hyperplasia in a teenage girl with tuberous sclerosis complex

Energy Technology Data Exchange (ETDEWEB)

Ha, Seung Mi; Yoon, Hye Kyung; Lee, Seung Koo [Kangwon National University Hospital, Chuncheon (Korea, Republic of)

2016-08-15

Multifocal micronodular pneumocyte hyperplasia (MMPH) is a relatively rare pulmonary disorder that can be associated with tuberous sclerosis complex (TSC). It has been rarely reported in children or adolescents. MMPH is a hamartomatous process of the lung with multiple small nodules, composed of type II pneumocytes. Plain radiography and chest CT in MMPH may demonstrate numerous small nodules measuring 1-10 mm in diameters, distributed randomly throughout both lungs. If MMPH is an initial presentation of TSC, and unless we are familiar with this lung manifestation of TSC, radiologic findings can mimic miliary tuberculosis or metastatic disease. We report a teenage girl with TSC and histologically confirmed MMPH which mimicked miliary tuberculosis at the initial presentation.

Rare lung manifestation of multifocal micronodular pneumocyte hyperplasia in a teenage girl with tuberous sclerosis complex

International Nuclear Information System (INIS)

Ha, Seung Mi; Yoon, Hye Kyung; Lee, Seung Koo

2016-01-01

Multifocal micronodular pneumocyte hyperplasia (MMPH) is a relatively rare pulmonary disorder that can be associated with tuberous sclerosis complex (TSC). It has been rarely reported in children or adolescents. MMPH is a hamartomatous process of the lung with multiple small nodules, composed of type II pneumocytes. Plain radiography and chest CT in MMPH may demonstrate numerous small nodules measuring 1-10 mm in diameters, distributed randomly throughout both lungs. If MMPH is an initial presentation of TSC, and unless we are familiar with this lung manifestation of TSC, radiologic findings can mimic miliary tuberculosis or metastatic disease. We report a teenage girl with TSC and histologically confirmed MMPH which mimicked miliary tuberculosis at the initial presentation

Contaminant transport modeling studies of Russian sites

International Nuclear Information System (INIS)

Tsang, Chin-Fu

1993-01-01

Lawrence Berkeley Laboratory (LBL) established mechanisms that promoted cooperation between U.S. and Russian scientists in scientific research as well as environmental technology transfer. Using Russian experience and U.S technology, LBL developed approaches for field investigations, site evaluation, waste disposal, and remediation at Russian contaminated sites. LBL assessed a comprehensive database as well as an actual, large-scale contaminated site to evaluate existing knowledge of and test mathematical models used for the assessment of U.S. contaminated sites

The pathogenesis and imaging of the tuberous sclerosis complex

International Nuclear Information System (INIS)

Baskin, Henry J.

2008-01-01

Tuberous sclerosis complex (TSC) is an autosomal-dominant disorder characterized by the formation of hamartomatous lesions in multiple organ systems. It is the second most common neurocutaneous syndrome after neurofibromatosis type 1 and has been recognized since the late 1800s. Although the disease has complete penetrance, there is also high phenotypic variability: some patients have obvious signs at birth, while others remain undiagnosed for many years. In addition to skin lesions, TSC patients develop numerous brain lesions, angiomyolipoma (AMLs), lymphangiomyomatosis (LAM) in the lungs, cardiac rhabdomyomas, skeletal lesions, and vascular anomalies, all of which are well seen with medical imaging. Our knowledge of TSC genetics and pathophysiology has expanded dramatically in recent years: two genetic loci were discovered in the 1990s and recent elucidation of TSC's interaction with the mTOR pathway has changed how we manage the disease. Meanwhile, medical imaging is playing an increasingly important role in the diagnosis, management, and treatment of TSC. We provide an update on the genetics and pathophysiology of TSC, review its clinical manifestations, and explore the breadth of imaging features in each organ system, from prenatal detection of cardiac rhabdomyomas to monitoring rapamycin therapy to treatment of AMLs by interventional radiology. (orig.)

Fuzzy Traffic Control with Vehicle-to-Everything Communication.

Science.gov (United States)

Salman, Muntaser A; Ozdemir, Suat; Celebi, Fatih V

2018-01-27

Traffic signal control (TSC) with vehicle-to everything (V2X) communication can be a very efficient solution to traffic congestion problem. Ratio of vehicles equipped with V2X communication capability in the traffic to the total number of vehicles (called penetration rate PR) is still low, thus V2X based TSC systems need to be supported by some other mechanisms. PR is the major factor that affects the quality of TSC process along with the evaluation interval. Quality of the TSC in each direction is a function of overall TSC quality of an intersection. Hence, quality evaluation of each direction should follow the evaluation of the overall intersection. Computational intelligence, more specifically swarm algorithm, has been recently used in this field in a European Framework Program FP7 supported project called COLOMBO. In this paper, using COLOMBO framework, further investigations have been done and two new methodologies using simple and fuzzy logic have been proposed. To evaluate the performance of our proposed methods, a comparison with COLOMBOs approach has been realized. The results reveal that TSC problem can be solved as a logical problem rather than an optimization problem. Performance of the proposed approaches is good enough to be suggested for future work under realistic scenarios even under low PR.

The pathogenesis and imaging of the tuberous sclerosis complex

Energy Technology Data Exchange (ETDEWEB)

Baskin, Henry J. [Cincinnati Children' s Medical Center, Department of Radiology, Cincinnati, OH (United States)

2008-09-15

Tuberous sclerosis complex (TSC) is an autosomal-dominant disorder characterized by the formation of hamartomatous lesions in multiple organ systems. It is the second most common neurocutaneous syndrome after neurofibromatosis type 1 and has been recognized since the late 1800s. Although the disease has complete penetrance, there is also high phenotypic variability: some patients have obvious signs at birth, while others remain undiagnosed for many years. In addition to skin lesions, TSC patients develop numerous brain lesions, angiomyolipoma (AMLs), lymphangiomyomatosis (LAM) in the lungs, cardiac rhabdomyomas, skeletal lesions, and vascular anomalies, all of which are well seen with medical imaging. Our knowledge of TSC genetics and pathophysiology has expanded dramatically in recent years: two genetic loci were discovered in the 1990s and recent elucidation of TSC's interaction with the mTOR pathway has changed how we manage the disease. Meanwhile, medical imaging is playing an increasingly important role in the diagnosis, management, and treatment of TSC. We provide an update on the genetics and pathophysiology of TSC, review its clinical manifestations, and explore the breadth of imaging features in each organ system, from prenatal detection of cardiac rhabdomyomas to monitoring rapamycin therapy to treatment of AMLs by interventional radiology. (orig.)

Fission Yeast Model Study for Dissection of TSC Pathway

Science.gov (United States)

2010-04-01

cerevisiae;Hs, Homo sapiens ;Dm,Drosophilamelanogaster. (C)Themulticopy suppressors ofDtsc2 cpp1-1cells.Dtsc2 cpp1-1cells carrying each of the five...distribution of their cell length was measured (right pan- els ). For each strain, .100 cells were counted. The experi- ments were performed three times with...dependent GAP activity. Taken together, we specu- late that rhb1-DA4 can stimulate its downstream ele - ments regardless of the guanine-nucleotide-binding

Cognitive Challenges

Science.gov (United States)

... Privacy Policy Sitemap Learn Engage Donate About TSC Cognitive Challenges Approximately 45% to 60% of individuals with TSC develop cognitive challenges (intellectual disabilities), although the degree of intellectual ...

Site Study Plan for Acoustics, Deaf Smith County Site, Texas: Environmental Field Program: Preliminary draft

International Nuclear Information System (INIS)

1987-06-01

The Acoustics site study plan describes a field program which characterizes existing sound levels, determines the area's sound propagation characteristics, and monitors the project-related sound emissions. The plan describes for each study: the need for the study, study design, data management and use, schedule, and quality assurance requirements. These studies will provide data needed to satisfy requirements contained in, or derived from, the Salt Repository Requirements Document. 37 refs., 9 figs., 3 tabs

Energy conserving site design case study: Shenandoah, Georgia. Final report

Energy Technology Data Exchange (ETDEWEB)

1980-01-01

The case study examines the means by which energy conservation can be achieved at an aggregate community level by using proper planning and analytical techniques for a new town, Shenandoah, Georgia, located twenty-five miles southwest of Atlanta's Hartsfield International Airport. A potentially implementable energy conservation community plan is achieved by a study team examining the land use options, siting characteristics of each building type, alternate infrastructure plans, possible decentralized energy options, and central utility schemes to determine how community energy conservation can be achieved by use of pre-construction planning. The concept for the development of mixed land uses as a passively sited, energy conserving community is based on a plan (Level 1 Plan) that uses the natural site characteristics, maximizes on passive energy siting requirement, and allows flexibility for the changing needs of the developers. The Level 2 Plan is identical with Level 1 plan plus a series of decentraized systems that have been added to the residential units: the single-family detached, the apartments, and the townhouses. Level 3 Plan is similar to the Level 1 Plan except that higher density dwellings have been moved to areas adjacent to central site. The total energy savings for each plan relative to the conventional plan are indicated. (MCW)

System studies of compact ignition tokamaks

International Nuclear Information System (INIS)

Galambos, J.D.; Peng, Y.K.M.; Blackfield, D.T.

1986-01-01

A new version of the FEDC Tokamak System Code (TSC) has been developed to analyze the Compact Ignition Tokamak (CIT). These proposed experiments have small (major radius F 1.5m) and high magnetic fields (B J 10T), and are characterized by reduced cost. Key design constraints of CIT include limits to the high stress levels in the magnetic coils, limits to the large temperature rises in the coils and on the first wall or divertor plate, minimizing power supply requirements, and assuring adequate plasma performance in fusion ignition and burn time consistent with the latest physics understanding. We present systems code level studies of CIT parameter space here for a range of design options with various design constraints. The present version of the TSC incorporates new models for key components of CIT. For example, new algorithms have been incorporated for calculating stress levels in the TFC and ohmic solenoid, temperature rise in the magnetic coils, peak power requirements, plasma MHD equilibrium and volt-second capability. The code also incorporates a numerical optimizer to find combinations of engineering quantities (device size, coil sizes, coil current densities etc.) and physics quantities (plasma density temperature, and beta, etc.) which satisfy all the constraints and can minimize or maximize a figure of merit (e.g., the major radius). This method was recently used in a mirror reactor system code (3) for the Minimara concept development

Attention Deficit Hyperactivity Disorder and Tuberous Sclerosis Complex

Science.gov (United States)

... Privacy Policy Sitemap Learn Engage Donate About TSC Attention Deficit Hyperactivity Disorder and TSC What is ADHD? Attention Deficit Hyperactivity Disorder (ADHD) is a common neurobehavioral disorder. It is ...

Reactive sites influence in PMMA oligomers reactivity: a DFT study

Science.gov (United States)

Paz, C. V.; Vásquez, S. R.; Flores, N.; García, L.; Rico, J. L.

2018-01-01

In this work, we present a theoretical study of methyl methacrylate (MMA) living anionic polymerization. The study was addressed to understanding two important experimental observations made for Michael Szwarc in 1956. The unexpected effect of reactive sites concentration in the propagation rate, and the self-killer behavior of MMA (deactivating of living anionic polymerization). The theoretical calculations were performed by density functional theory (DFT) to obtain the frontier molecular orbitals values. These values were used to calculate and analyze the chemical interaction descriptors in DFT-Koopmans’ theorem. As a result, it was observed that the longest chain-length species (related with low concentration of reactive sites) exhibit the highest reactivity (behavior associated with the increase of the propagation rate). The improvement in this reactivity was attributed to the crosslinking produced in the polymethyl methacrylate chains. Meanwhile, the self-killer behavior was associated with the intermolecular forces present in the reactive sites. This behavior was associated to an obstruction in solvation, since the active sites remained active through all propagation species. The theoretical results were in good agreement with the Szwarc experiments.

Amplification of the angiogenic signal through the activation of the TSC/mTOR/HIF axis by the KSHV vGPCR in Kaposi's sarcoma.

Directory of Open Access Journals (Sweden)

Bruno C Jham

2011-04-01

Full Text Available Kaposi's sarcoma (KS is a vascular neoplasm characterized by the dysregulated expression of angiogenic and inflammatory cytokines. The driving force of the KS lesion, the KSHV-infected spindle cell, secretes elevated levels of vascular endothelial growth factor (VEGF, essential for KS development. However, the origin of VEGF in this tumor remains unclear.Here we report that the KSHV G protein-coupled receptor (vGPCR upregulates VEGF in KS through an intricate paracrine mechanism. The cytokines secreted by the few vGPCR-expressing tumor cells activate in neighboring cells multiple pathways (including AKT, ERK, p38 and IKKβ that, in turn, converge on TSC1/2, promoting mTOR activation, HIF upregulation, and VEGF secretion. Conditioned media from vGPCR-expressing cells lead to an mTOR-dependent increase in HIF-1α and HIF-2α protein levels and VEGF upregulation. In a mouse allograft model for KS, specific inhibition of the paracrine activation of mTOR in non-vGPCR-expressing cells was sufficient to inhibit HIF upregulation in these cells, and abolished the ability of the vGPCR-expressing cells to promote tumor formation in vivo. Similarly, pharmacologic inhibition of HIF in this model blocked VEGF secretion and also lead to tumor regression.Our findings provide a compelling explanation for how the few tumor cells expressing vGPCR can contribute to the dramatic amplification of VEGF secretion in KS, and further provide a molecular mechanism for how cytokine dysregulation in KS fuels angiogenesis and tumor development. These data further suggest that activation of HIF by vGPCR may be a vulnerable target for the treatment of patients with KS.

Wildlife studies on the Hanford site: 1994 Highlights report

Energy Technology Data Exchange (ETDEWEB)

Cadwell, L.L. [ed.

1995-04-01

The purposes of the project are to monitor and report trends in wildlife populations; conduct surveys to identify, record, and map populations of threatened, endangered, and sensitive plant and animal species; and cooperate with Washington State and federal and private agencies to help ensure the protection afforded by law to native species and their habitats. Census data and results of surveys and special study topics are shared freely among cooperating agencies. Special studies are also conducted as needed to provide additional information that may be required to assess, protect, or manage wildlife resources at Hanford. This report describes highlights of wildlife studies on the Site in 1994. Redd counts of fall chinook salmon in the Hanford Reach suggest that harvest restrictions directed at protecting Snake River salmon may have helped Columbia River stocks as well. The 1994 count (5619) was nearly double that of 1993 and about 63% of the 1989 high of approximately 9000. A habitat map showing major vegetation and land use cover types for the Hanford Site was completed in 1993. During 1994, stochastic simulation was used to estimate shrub characteristics (height, density, and canopy cover) across the previously mapped Hanford landscape. The information provided will be available for use in determining habitat quality for sensitive wildlife species. Mapping Site locations of plant species of concern continued during 1994. Additional sensitive plant species data from surveys conducted by TNC were archived. The 10 nesting pairs of ferruginous hawks that used the Hanford Site in 1993 represented approximately 25% of the Washington State population.

Wildlife studies on the Hanford site: 1994 Highlights report

International Nuclear Information System (INIS)

Cadwell, L.L.

1995-04-01

The purposes of the project are to monitor and report trends in wildlife populations; conduct surveys to identify, record, and map populations of threatened, endangered, and sensitive plant and animal species; and cooperate with Washington State and federal and private agencies to help ensure the protection afforded by law to native species and their habitats. Census data and results of surveys and special study topics are shared freely among cooperating agencies. Special studies are also conducted as needed to provide additional information that may be required to assess, protect, or manage wildlife resources at Hanford. This report describes highlights of wildlife studies on the Site in 1994. Redd counts of fall chinook salmon in the Hanford Reach suggest that harvest restrictions directed at protecting Snake River salmon may have helped Columbia River stocks as well. The 1994 count (5619) was nearly double that of 1993 and about 63% of the 1989 high of approximately 9000. A habitat map showing major vegetation and land use cover types for the Hanford Site was completed in 1993. During 1994, stochastic simulation was used to estimate shrub characteristics (height, density, and canopy cover) across the previously mapped Hanford landscape. The information provided will be available for use in determining habitat quality for sensitive wildlife species. Mapping Site locations of plant species of concern continued during 1994. Additional sensitive plant species data from surveys conducted by TNC were archived. The 10 nesting pairs of ferruginous hawks that used the Hanford Site in 1993 represented approximately 25% of the Washington State population

KAJIAN ZONASI PENGEMBANGAN KAWASAN PUSAKA. STUDI KASUS: SITUS SANGIRAN, SRAGEN (Zoning Study of Heritage Site Development Case Study: Sangiran Site, Sragen

Directory of Open Access Journals (Sweden)

Wiendu Nuryanti

2008-11-01

Full Text Available ABSTRAK Sebagai Warisan Budaya Dunia (World Culture Heritage yang ditetapkan oleh UNESCO pada tanggal 5 Desember 1996, Situs Sangiran merupakan bagian penting dalam sejarah manusia di dunia. Sesuai dengan keputusan Menteri Pendidikan dan Kebudayaan (Nomor 0701011977, situs tersebut telah ditetapkan sebagai cagar budaya (Widianto, et al.,1996. Penelitian ini memiliki dua tujuan utama: (1 menyusun konsepsi dasar pelestarian, dan (2 menyusun arahan desain (guidelines pelestarian Situs Sangiran. Permasalahan utama dari kajian ini adalah perubahan lahan karena faktor alam dan aktivitas manusia (pertanian, pembangunan, penambangan, ancaman pencurian, penggelapan, dan jual beli fosil, rendahnya partisipasi masyarakat dalam upaya pelestarian Situs Sangiran, dan belum adanya panduan/arahan pelestarian dan pemanfaatan wisata yang jelas. Dengan metoda kualitatif naturalistik, dihasilkan konsepsi (prinsip dasar pelestarian Situs Sangiran Sragen, dalanr masing-masing zona (zona I -3 sesuai dengan potensi tiap zona yang perlu dilestarikan dan dikernbangkan. Analisis yang digunakan dalam hal ini adalah analisis makro, meso, dan mikro. Dari kajian ini dapat disimpulkan bahwa dalam merencanakan pelestarian Situs Sangiran, hal terpenting adalah mentaati zonasi dasar situs, di mana setiap zona akan merniliki guidelines  tersendiri. Guidelines terdiri atas pengembangan produk (Klaster Ngebung, Bukuran, Dayu, street furniture, serta pemberdayaan masyarakat.   ABSTRACT  UNESCO has established Sangiran Site as a World Culture Heritage in December 5th 1996. Its present is important to the history of mankind in the world. Widianto, et al., 1996, stated that Sangiran site has become a Cultural Site regarding to the declaration of Minister of Culture and Education (No. 070/0/1977. This Research has two main purposes, (1 to arrange a conception of basic preservation, and (2 to arrange a design guidelines of Sangiran Site Preservation. There are several major issues in

76 FR 81959 - Notice of Proposed Information Collection: Comment Request; Homelessness Prevention Study Site...

Science.gov (United States)

2011-12-29

... Information Collection: Comment Request; Homelessness Prevention Study Site Visits AGENCY: Office of the Chief.... This Notice also lists the following information: Title of Proposal: Homelessness Prevention Study Site... of the Paperwork Reduction Act requirements associated with HUD's Homelessness Prevention Study Site...

A Study of Social Networking Sites for Learners of Japanese

Directory of Open Access Journals (Sweden)

Fusako Ota

2011-01-01

Full Text Available Social Networking Sites (SNSs such as “Facebook” and “MySpace” have been used by many people from different countries around the world, and they have recently been applied to second language (L2 learning, both inside and outside classrooms. A number of researchers have investigated the utility of SNSs, and some language researchers have studied the use of SNSs for L2 learning in language classrooms. However, the study of the usage of SNSs for L2 learning outside the classroom has not yet been studied thoroughly, despite the fact that many communities and groups exist for users who are interested in learning L2 on such sites. This article examines the nature and extent of SNS communities available specifically for L2 learners of Japanese, and describes the usage which is being made of these communities in particular on the SNSs, “mixi” and “Facebook”. Furthermore, the beneficial aspects of using such sites for L2 learning will be discussed.

Site study plan for non-routine laboratory rock mechanics, Deaf Smith County Site, Texas: Revision 1

International Nuclear Information System (INIS)

1987-12-01

This Site Study Plan describes the non-routine rock mechanics and thermal properties laboratory testing program planned for the characterization of site-specific geologic materials for the Deaf Smith County site, Texas. The study design provides for measurements of index, mechanical, thermomechanical, thermal and special properties for the host salt, and where appropriate, for nonhost lithologies. The types of tests which will be conducted are constant stress (creep) tests, constant strain (stress relaxation) tests, constant strain-rate tests, constant stress-rate tests, cyclic loading tests, hollow cylinder tests, uniaxial and triaxial compression tests, direct tension tests, indirect (triaxial) shear tests, thermal property determinations (conductivity, specific heat, expansivity, and diffusivity), fracture healing tests, thermal decrepitation tests, moisture content determinations, and petrographic and micromechanics analyses. Tests will be conducted at confining pressures up to 30 MPa and temperatures up to 300/degree/C. These data are used to construct mathematical models for the phenomenology of salt deformation. The models are then used in finite-element codes to predict repository response. A tentative testing schedule and milestone log are given. The duration of the testing program is expected to be approximately 5 years. 44 refs., 13 figs., 13 tabs

An economic study of the site of Marcoule; Etude economique du site de Marcoule

Energy Technology Data Exchange (ETDEWEB)

Duprat, H [Commissariat a l' Energie Atomique, Marcoule (France). Centre d' Etudes Nucleaires

1964-07-01

The study of this site of the Marcoule Center shows that its regional economical influence is limited to the buying power distributed among its personnel. This entails distortions between first this supplementary buying power and the overall markets within the possible Marcoule influence zone, and secondly the concentration on Bagnols-sur-Ceze and this town's normal extension possibilities. (author) [French] L'etude du site de Marcoule demontre que son influence economique se limite, pour la region, au pouvoir d'achat distribue a son personnel. Ceci entraine une disproportion d'une part entre ce pouvoir d'achat supplementaire et l'ensemble des marches contenus dans l'aire d'influence possible de Marcoule, d'autre part entre la concentration realisee a Bagnole-sur-Ceze et la capacite d'extension normale de cette agglomeration. (auteur)

Web Site of King Faysal for Researches and Islamic Studies :Evaluative Case Study . 2

Directory of Open Access Journals (Sweden)

Hind Badi Aly al-Badi

2007-06-01

Full Text Available This study makes evaluation to the website of King Faisal Islamic Study & Research Center (KFISRC in relation to its site, advanced design, services rendered, public users and easy-to-search including its discharged duties; content, form and scope.The study aims to evaluate the website of KFISRC published in the Internet as well as the volume of its sample users, and to determine the motivations of use.The study was conducted on the Second Semester in 1426H. / 2005G. The researcher has relied in the evaluation case study on "on-line access" in the Internet; a matter which was imposed by the nature and objectives of the study and followed by an evaluation-analysis methodology based on the measures derived from Website & Information Resources Measures in the Internet. The information were gathered using the questionnaire forms which were distributed to a random sample of 15% of the society slide subject of the study totaling 1755 of post-graduate female students; i.e. Master degree; enrolled in Library & Information, Arabic Language and History Departments of Faculty of Arts & Humanities , King Abdulaziz University, Jeddah; and post-graduate female students; i.e. Master degree; enrolled in Faculty of Da'wa & Fundamentals of Religion, Umm Al-Qura University, Makkah Al-Mukarramah, including male and female staff of KFISRC. The study has reached to the number of findings, mainly as follows:The volume of the users has reached to 40.7% of the sampling. One of the reasons of the decreasing percentage in the number of the site users of KFISRC is that 32.4% did not know about the existence of the site in the Internet; whereas, 24.1% of the sampling whose most important motivation to use the site is to know whether the resources searched by them would be available in the data-bases of the KFISRC or not

Web Site of King Faysal for Researches and Islamic Studies :Evaluative Case Study . 1

Directory of Open Access Journals (Sweden)

Hind Badi Aly al-Badi

2007-03-01

Full Text Available This study makes evaluation to the website of King Faisal Islamic Study & Research Center (KFISRC in relation to its site, advanced design, services rendered, public users and easy-to-search including its discharged duties; content, form and scope.The study aims to evaluate the website of KFISRC published in the Internet as well as the volume of its sample users, and to determine the motivations of use.The study was conducted on the Second Semester in 1426H. / 2005G. The researcher has relied in the evaluation case study on "on-line access" in the Internet; a matter which was imposed by the nature and objectives of the study and followed by an evaluation-analysis methodology based on the measures derived from Website & Information Resources Measures in the Internet. The information were gathered using the questionnaire forms which were distributed to a random sample of 15% of the society slide subject of the study totaling 1755 of post-graduate female students; i.e. Master degree; enrolled in Library & Information, Arabic Language and History Departments of Faculty of Arts & Humanities , King Abdulaziz University, Jeddah; and post-graduate female students; i.e. Master degree; enrolled in Faculty of Da'wa & Fundamentals of Religion, Umm Al-Qura University, Makkah Al-Mukarramah, including male and female staff of KFISRC. The study has reached to the number of findings, mainly as follows:The volume of the users has reached to 40.7% of the sampling. One of the reasons of the decreasing percentage in the number of the site users of KFISRC is that 32.4% did not know about the existence of the site in the Internet; whereas, 24.1% of the sampling whose most important motivation to use the site is to know whether the resources searched by them would be available in the data-bases of the KFISRC or not

Feasibility study for the United Heckathorn Superfund Site, Richmond, California

Energy Technology Data Exchange (ETDEWEB)

Lincoff, A.H. [US Environmental Protection Agency, San Francisco, CA (United States). Region IX; Costan, G.P.; Montgomery, M.S.; White, P.J. [Pacific Northwest Lab., Richland, WA (United States)

1994-07-01

The United Heckathom Superfund Site in Richmond, California, was used to formulate pesticides from approximately 1947 to 1966. Soils at the site and sediments in the harbor were contaminated with various chlorinated pesticides, primarily DDT, as a result of these activities. The US Environmental Protection Agency listed the site on the Superfund National Priorities List in 1990. This document is part of the Remedial Investigation and Feasibility Study phase of the Superfund response, which will provide the basis for selection of a final remedy that will protect human health and the environment and achieve compliance with federal and state envirorunental laws.

Feasibility study for the United Heckathorn Superfund Site, Richmond, California

International Nuclear Information System (INIS)

Lincoff, A.H.

1994-07-01

The United Heckathom Superfund Site in Richmond, California, was used to formulate pesticides from approximately 1947 to 1966. Soils at the site and sediments in the harbor were contaminated with various chlorinated pesticides, primarily DDT, as a result of these activities. The US Environmental Protection Agency listed the site on the Superfund National Priorities List in 1990. This document is part of the Remedial Investigation and Feasibility Study phase of the Superfund response, which will provide the basis for selection of a final remedy that will protect human health and the environment and achieve compliance with federal and state envirorunental laws

Sangrado de angiomiolipoma renal en paciente con síndrome de genes contiguos (TSC2/PKD1 tras 17 años de tratamiento renal sustitutivo

Directory of Open Access Journals (Sweden)

Mónica Furlano

2017-01-01

Full Text Available Presentamos el caso de un varón de 32 años, con síndrome de genes contiguos TSC2/PKD1, que le ocasiona esclerosis tuberosa (ET y poliquistosis renal autosómica dominante simultáneamente. Evolucionó a enfermedad renal terminal y se realizó trasplante renal a los 12 años. Los riñones presentaban angiomiolipomas (AML, que son tumores benignos frecuentes en pacientes con ET. A los 17 años postrasplante, presentó un cuadro de dolor abdominal, anemización y hematoma retroperitoneal. Dicho hematoma se produjo por el sangrado de los AML. Como tratamiento se realizó embolización selectiva. Nuestro paciente podría haberse beneficiado en el momento del trasplante renal del tratamiento con inhibidores de mTOR. Este fármaco actúa como inmunosupresor y reductor tumoral en la ET, al disminuir el riesgo de rotura y hemorragia. En este paciente no se administró porque cuando se trasplantó no se conocía la relación de los inhibidores de mTOR con la ET. Este caso confirma que, a pesar de tratarse de pacientes trasplantados o en diálisis, el riesgo de sangrado por los AML persiste, por lo cual se propone realizar controles periódicos de los riñones propios y valorar la nefrectomía.

Geophysical study of the Peinan Archaeological Site, Taiwan

Science.gov (United States)

Tong, Lun-Tao; Lee, Kun-Hsiu; Yeh, Chang-Keng; Hwang, Yan-Tsong; Chien, Jeng-Ming

2013-02-01

The Peinan archaeological site is the most intact Neolithic village with slate coffin burial complexes in Taiwan. However, the area that potentially contains significant ancient remains is covered by dense vegetation. No reliable data show the distribution of the ancient village, and no geophysical investigation has been performed at this site. To evaluate various geophysical methods under the geological setting and surface condition of the site, the physical properties of the remains were measured and four geophysical methods involving magnetic, electromagnetic (EM), electrical resistivity tomography (ERT), and ground-penetrating radar (GPR) were tested along three parallel profiles. The results imply that the EM and magnetic methods are much cost-effective and suitable for investigating the entire area. GPR and ERT methods can provide high resolution subsurface image, which are much suitable for subsequently detail investigation. The EM and magnetic surveys were thus conducted over the entire Peinan Cultural Park to understand the distribution of the ancient building remains at the Peinan site. The results of this study were verified by subsequent excavations, which indicate that the EM survey was successful in delineating the majority of the ancient village because the basements of building are highly resistive in comparison to the background sediment. The results of this investigation suggest that the ancient village was broadly distributed over the eastern part of the Peinan Culture Park and extended to the southeast.

[Study on Tritium Content in Soil at Sites of Nuclear Explosions on the Territory of Semipalatinsk Test Site].

Science.gov (United States)

Timonova, L V; Lyakhova, O N; Lukashenko, S N; Aidarkhanov, A O

2015-01-01

As a result of investigations carried out on the territory of Semipalatinsk Test Site, tritium was found in different environmental objects--surface and ground waters, vegetation, air environment, and snow cover. The analysis of the data obtained has shown that contamination of environmental objects at the Semipalatinsk Test Site with tritium is associated with the places where underground nuclear tests were performed. Since tritium can originate from an activation reaction and be trapped by pock particles during a test, it was decided to examine the soil in the sites where surface and excavation tests took place. It was found that the concentration of tritium in soil correlates with the concentration of europium. Probably, the concentration of tritium in the soil depends on the character and yield of the tests performed. Findings of the study have revealed that tritium can be found in soil in significant amounts not only in sites where underground nuclear tests took place but also in sites where surface and excavation nuclear tests were carried out.

Reversal of tolerance induced by transplantation of skin expressing the immunodominant T cell epitope of rat type II collagen entitles development of collagen-induced arthritis but not graft rejection

DEFF Research Database (Denmark)

Bäcklund, Johan; Treschow, Alexandra; Firan, Mihail

2002-01-01

rejection or instead to tolerance and arthritis protection. Interestingly, TSC grafts were accepted and not even immunization of recipient mice with CII in adjuvant induced graft rejection. Instead, TSC skin recipients displayed a reduced T and B cell response to CII and were also protected from arthritis...... collagen (CI), e.g. in skin, are tolerized against rat CII and resistant to CIA. In this study we transplanted skin from TSC transgenic mice onto non-transgenic CIA-susceptible littermates to investigate whether introduction of this epitope to a naïve immune system would lead to T cell priming and graft....... However, additional priming could break arthritis protection and was accompanied by an increased T cell response to the grafted epitope. Strikingly, despite the regained T cell response, development of arthritis was not accompanied by graft rejection, showing that these immune-mediated inflammatory...

Long-Term Use of Everolimus in Patients with Tuberous Sclerosis Complex: Final Results from the EXIST-1 Study.

Directory of Open Access Journals (Sweden)

David N Franz

Full Text Available Everolimus, a mammalian target of rapamycin (mTOR inhibitor, has demonstrated efficacy in treating subependymal giant cell astrocytomas (SEGAs and other manifestations of tuberous sclerosis complex (TSC. However, long-term use of mTOR inhibitors might be necessary. This analysis explored long-term efficacy and safety of everolimus from the conclusion of the EXIST-1 study (NCT00789828.EXIST-1 was an international, prospective, double-blind, placebo-controlled phase 3 trial examining everolimus in patients with new or growing TSC-related SEGA. After a double-blind core phase, all remaining patients could receive everolimus in a long-term, open-label extension. Everolimus was initiated at a dose (4.5 mg/m2/day titrated to a target blood trough of 5-15 ng/mL. SEGA response rate (primary end point was defined as the proportion of patients achieving confirmed ≥50% reduction in the sum volume of target SEGA lesions from baseline in the absence of worsening nontarget SEGA lesions, new target SEGA lesions, and new or worsening hydrocephalus. Of 111 patients (median age, 9.5 years who received ≥1 dose of everolimus (median duration, 47.1 months, 57.7% (95% confidence interval [CI], 47.9-67.0 achieved SEGA response. Of 41 patients with target renal angiomyolipomas at baseline, 30 (73.2% achieved renal angiomyolipoma response. In 105 patients with ≥1 skin lesion at baseline, skin lesion response rate was 58.1%. Incidence of adverse events (AEs was comparable with that of previous reports, and occurrence of emergent AEs generally decreased over time. The most common AEs (≥30% incidence suspected to be treatment-related were stomatitis (43.2% and mouth ulceration (32.4%.Everolimus use led to sustained reduction in tumor volume, and new responses were observed for SEGA and renal angiomyolipoma from the blinded core phase of the study. These findings support the hypothesis that everolimus can safely reverse multisystem manifestations of TSC in a

Integrated ground-water monitoring strategy for NRC-licensed facilities and sites: Case study applications

Science.gov (United States)

Price, V.; Temples, T.; Hodges, R.; Dai, Z.; Watkins, D.; Imrich, J.

2007-01-01

This document discusses results of applying the Integrated Ground-Water Monitoring Strategy (the Strategy) to actual waste sites using existing field characterization and monitoring data. The Strategy is a systematic approach to dealing with complex sites. Application of such a systematic approach will reduce uncertainty associated with site analysis, and therefore uncertainty associated with management decisions about a site. The Strategy can be used to guide the development of a ground-water monitoring program or to review an existing one. The sites selected for study fall within a wide range of geologic and climatic settings, waste compositions, and site design characteristics and represent realistic cases that might be encountered by the NRC. No one case study illustrates a comprehensive application of the Strategy using all available site data. Rather, within each case study we focus on certain aspects of the Strategy, to illustrate concepts that can be applied generically to all sites. The test sites selected include:Charleston, South Carolina, Naval Weapons Station,Brookhaven National Laboratory on Long Island, New York,The USGS Amargosa Desert Research Site in Nevada,Rocky Flats in Colorado,C-Area at the Savannah River Site in South Carolina, andThe Hanford 300 Area.A Data Analysis section provides examples of detailed data analysis of monitoring data.

The Cottonwood Lake study area, a long-term wetland ecosystem monitoring site

Science.gov (United States)

Mushet, David M.; Euliss, Ned H.

2012-01-01

The Cottonwood Lake study area is one of only three long-term wetland ecosystem monitoring sites in the prairie pothole region of North America; the other two are Orchid Meadows in South Dakota and St. Denis in Saskatchewan. Of the three, Cottonwood Lake has, by far, the longest continuous data-collection record. Research was initiated at the study area in 1966, and intensive investigations of the hydrology, chemistry, and biology of prairie pothole wetlands continue at the site today. This fact sheet describes the study area, provides an overview of wetland ecology research that has been conducted at the site in the past, and provides an introduction to current work being conducted at the study area by USGS scientists.

Early Behavioral Intervention to Improve Social Communication Function in Infants with TSC

Science.gov (United States)

2016-10-01

Disability (ID) using the...infant to complete the JASPER intervention at the UCLA site, acquired during our Social Scenes Paradigm (see Figures 1 & 2). The plot demonstrates that...Theory in Intellectual and Developmental Disabilities San Diego, CA • Jeste, S.S., (2016). Can rare disorders pave the way to

The Agincourt demographic and health study - site description ...

African Journals Online (AJOL)

The Agincourt demographic and health study - site description, baseline findings and implications. Stephen M Tollman, Kobus Herbst, Michel Garenne, John S.S. Gear, KathJeen Kahn. Abstract. No Abstract. Full Text: EMAIL FREE FULL TEXT EMAIL FREE FULL TEXT · DOWNLOAD FULL TEXT DOWNLOAD FULL TEXT.

Safety aspects of geological studies around nuclear installations sites

International Nuclear Information System (INIS)

Faure, J.

1988-01-01

The experience of geological studies of about forty french nuclear sites allows to set out the objectives, the phases and the geographic extensions of workings to be realized for confirming a site. The data to be collected for the safety analysis are specified; they concern the local and regional geology, the geotechnical characteristics and the essential elements for evaluating the hazards related to the soil liquefaction, the surface fracturing and in some cases the volcanic risks. It is necessary to follow up the geology during the installation construction and life. 8 refs. (F.M.)

A crystallographic and spectroscopic study of crystal sites in the 'spinel' structure; Etude cristallographique et spectroscopique des sites cristallins dans la structure 'spinelle'

Energy Technology Data Exchange (ETDEWEB)

Drifford, M [Commissariat a l' Energie Atomique, Saclay (France). Centre d' Etudes Nucleaires

1967-06-15

A crystallographic study on magnesium aluminates led to the determination of crystal site average distortion. A spectroscopic study of transition elements doped spinels gave the position of the doping element in tetrahedral (A) or octahedral (B) sites, and the local distortion of the latter. The comparison of average and local parameters points to differences in the behaviour of doping elements and shows the size of (A) sites are independent of the host crystal composition while the average size of (B) sites varies with the composition. (author) [French] Une etude cristallographique des aluminates de magnesium a permis de determiner la deformation moyenne des sites cristallins. Une etude spectroscopique des aluminates de magnesium dopes avec des elements de transition a permis de localiser les dopants entre les sites tetraedriques (A) et octaedriques (B) et d'etudier la deformation locale de ces sites. La comparaison entre les parametres moyens et locaux a caracterise le comportement du dopant et montre que les dimensions des sites (A) sont independantes de la composition de la matrice, alors que les caracteristiques geometriques moyennes des sites (B) varient continument avec la composition. (auteur)

Behavior-based safety on construction sites: a case study.

Science.gov (United States)

Choudhry, Rafiq M

2014-09-01

This work presents the results of a case study and describes an important area within the field of construction safety management, namely behavior-based safety (BBS). This paper adopts and develops a management approach for safety improvements in construction site environments. A rigorous behavioral safety system and its intervention program was implemented and deployed on target construction sites. After taking a few weeks of safety behavior measurements, the project management team implemented the designed intervention and measurements were taken. Goal-setting sessions were arranged on-site with workers' participation to set realistic and attainable targets of performance. Safety performance measurements continued and the levels of performance and the targets were presented on feedback charts. Supervisors were asked to give workers recognition and praise when they acted safely or improved critical behaviors. Observers were requested to have discussions with workers, visit the site, distribute training materials to workers, and provide feedback to crews and display charts. They were required to talk to operatives in the presence of line managers. It was necessary to develop awareness and understanding of what was being measured. In the process, operatives learned how to act safely when conducting site tasks using the designed checklists. Current weekly scores were discussed in the weekly safety meetings and other operational site meetings with emphasis on how to achieve set targets. The reliability of the safety performance measures taken by the company's observers was monitored. A clear increase in safety performance level was achieved across all categories: personal protective equipment; housekeeping; access to heights; plant and equipment, and scaffolding. The research reveals that scores of safety performance at one project improved from 86% (at the end of 3rd week) to 92.9% during the 9th week. The results of intervention demonstrated large decreases in

Lungs in TSC

Science.gov (United States)

... must be done in the hospital under general anesthesia. In general, lung biopsy is not required to ... be helpful for some LAM patients who have asthma like symptoms of wheezing and intermittent shortness of ...

Test-retest and between-site reliability in a multicenter fMRI study.

Science.gov (United States)

Friedman, Lee; Stern, Hal; Brown, Gregory G; Mathalon, Daniel H; Turner, Jessica; Glover, Gary H; Gollub, Randy L; Lauriello, John; Lim, Kelvin O; Cannon, Tyrone; Greve, Douglas N; Bockholt, Henry Jeremy; Belger, Aysenil; Mueller, Bryon; Doty, Michael J; He, Jianchun; Wells, William; Smyth, Padhraic; Pieper, Steve; Kim, Seyoung; Kubicki, Marek; Vangel, Mark; Potkin, Steven G

2008-08-01

In the present report, estimates of test-retest and between-site reliability of fMRI assessments were produced in the context of a multicenter fMRI reliability study (FBIRN Phase 1, www.nbirn.net). Five subjects were scanned on 10 MRI scanners on two occasions. The fMRI task was a simple block design sensorimotor task. The impulse response functions to the stimulation block were derived using an FIR-deconvolution analysis with FMRISTAT. Six functionally-derived ROIs covering the visual, auditory and motor cortices, created from a prior analysis, were used. Two dependent variables were compared: percent signal change and contrast-to-noise-ratio. Reliability was assessed with intraclass correlation coefficients derived from a variance components analysis. Test-retest reliability was high, but initially, between-site reliability was low, indicating a strong contribution from site and site-by-subject variance. However, a number of factors that can markedly improve between-site reliability were uncovered, including increasing the size of the ROIs, adjusting for smoothness differences, and inclusion of additional runs. By employing multiple steps, between-site reliability for 3T scanners was increased by 123%. Dropping one site at a time and assessing reliability can be a useful method of assessing the sensitivity of the results to particular sites. These findings should provide guidance toothers on the best practices for future multicenter studies.

Clinical report: a rare co-occurrence of tuberous sclerosis complex and Rett syndrome in a girl with mental retardation, epilepsy and autism

OpenAIRE

Belousova, Elena; Sukhorukov, Vladimir; Dorofeeva, Marina; Shagam, Lev; Vlodavetz, Dmitrii V.

2017-01-01

Introduction. There are some genetic disorders with combination of mental retardation, epilepsy and autism in which the abnormal mammalian Target of Rapamycin (m-TOR) signaling is implicated. The most important of them is tuberous sclerosis complex (TSC), but the disturbances of the m-TOR pathway can also be detected in Rett syndrome (RS), Fragile X syndrome and Down syndrome. We describe the rare case of co-occurrence of TSC and RS. Case study. The female child was born at term by normal de...

Webs sites of women`s studies universitary centers in spain: selection and evaluation

Directory of Open Access Journals (Sweden)

Isabel de Torres Ramírez

2006-12-01

Full Text Available After a brief introduction about the level of development of Women’s Studies in Spain and a list of their parent university institutions, Women’s Studies Web sites are assessed by measuring their usability. The aim is to improve use and accessibility of existing Web sites and, above all, provide encouragement and a model to follow by future Web sites in this area.

Sensitization of Cancer Cells through Reduction of Total Akt and Downregulation of Salinomycin-Induced pAkt, pGSk3β, pTSC2, and p4EBP1 by Cotreatment with MK-2206

Directory of Open Access Journals (Sweden)

Ae-Ran Choi

2014-01-01

Full Text Available MK-2206 is an inhibitor of Akt activation. It has been investigated as an anticancer drug in clinical trials assessing the potential of pAkt targeting therapy. The purpose of this study was to identify conditions that increase the sensitivity of cancer cells to MK-2206. We found that the treatment of cancer cells with a high concentration of salinomycin (Sal reduced total Akt protein levels but increased activated Akt levels. When cancer cells were cotreated with MK-2206 and Sal, both pAkt and total Akt levels were reduced. Using microscopic observation, an assessment of cleaved PARP, FACS analysis of pre-G1 region, and Hoechst staining, we found that Sal increased apoptosis of MK-2206-treated cancer cells. These results suggest that cotreatment with MK-2206 and Sal sensitizes cancer cells via reduction of both pAkt and total Akt. Furthermore, cotreatment of cancer cells with Sal and MK-2206 reduced pp70S6K, pmTOR, and pPDK1 levels. In addition, Sal-induced activation of GSK3β, TSC2, and 4EBP1 was abolished by MK-2206 cotreatment. These results suggest that cotreatment using MK-2206 and Sal could be used as a therapeutic method to sensitize cancer cells through targeting of the PI3K/Akt/mTOR pathway. Our findings may contribute to the development of MK-2206-based sensitization therapies for cancer patients.

Innovations in Site Characterization Case Study: The Role of a Conceptual Site Model for Expedited Site Characterization Using the Triad Approach at the Poudre River Site, Fort Collins, Colorado

Science.gov (United States)

This case study examines how systematic planning, an evolving conceptual site model (CSM), dynamic work strategies, and real time measurement technologies can be used to unravel complex contaminant distribution patterns...

Summary of site-characterization studies conducted from 1983 through 1987 at the Waste Isolation Pilot Plant (WIPP) site, southeastern New Mexico

International Nuclear Information System (INIS)

Lappin, A.R.

1988-01-01

The Waste Isolation Pilot Plant (WIPP) is being excavated at a depth of approximately 655 m in bedded halites. Site-characterization activities at the WIPP site began in 1976. Characterization activities since 1983 have had the objective of updating the conceptual model for the geologic and hydrologic behavior of the WIPP site and vicinity. This paper discusses aspects of the general conceptual model significant to both site characterization and performance assessment. The geological and hydrologic behavior of the WIPP site and vicinity is transient, and has been transient since at least deposition of the Permian Salado Formation containing the underground workings of the WIPP facility. The Salado Formation deforms regionally in response to gravity, but is very low in permeability, except within approximately two meters of the WIPP facility. The Culebra Dolomite Member of the Rustler Formation dominates the hydrology at the WIPP site. Hydrologic measurements, geologic studies, major-element and minor-element distributions in Culebra fluids, and the results of isotopic studies (stable-isotope, radiocarbon, uranium-disequilibrium, and 87 Sr/ 86 Sr) are consistent with the interpretations that, although the Culebra dominates flow within the Rustler at the WIPP site and Rustler karst is not present, there has been limited vertical fluid movement within the Rustler and between the Rustler and the overlying Dewey Lake Red Beds

Multi-site Study of Diffusion Metric Variability: Characterizing the Effects of Site, Vendor, Field Strength, and Echo Time using the Histogram Distance

Science.gov (United States)

Helmer, K. G.; Chou, M-C.; Preciado, R. I.; Gimi, B.; Rollins, N. K.; Song, A.; Turner, J.; Mori, S.

2016-01-01

MRI-based multi-site trials now routinely include some form of diffusion-weighted imaging (DWI) in their protocol. These studies can include data originating from scanners built by different vendors, each with their own set of unique protocol restrictions, including restrictions on the number of available gradient directions, whether an externally-generated list of gradient directions can be used, and restrictions on the echo time (TE). One challenge of multi-site studies is to create a common imaging protocol that will result in a reliable and accurate set of diffusion metrics. The present study describes the effect of site, scanner vendor, field strength, and TE on two common metrics: the first moment of the diffusion tensor field (mean diffusivity, MD), and the fractional anisotropy (FA). We have shown in earlier work that ROI metrics and the mean of MD and FA histograms are not sufficiently sensitive for use in site characterization. Here we use the distance between whole brain histograms of FA and MD to investigate within- and between-site effects. We concluded that the variability of DTI metrics due to site, vendor, field strength, and echo time could influence the results in multi-center trials and that histogram distance is sensitive metrics for each of these variables. PMID:27350723

Multi-site Study of Diffusion Metric Variability: Characterizing the Effects of Site, Vendor, Field Strength, and Echo Time using the Histogram Distance.

Science.gov (United States)

Helmer, K G; Chou, M-C; Preciado, R I; Gimi, B; Rollins, N K; Song, A; Turner, J; Mori, S

2016-02-27

MRI-based multi-site trials now routinely include some form of diffusion-weighted imaging (DWI) in their protocol. These studies can include data originating from scanners built by different vendors, each with their own set of unique protocol restrictions, including restrictions on the number of available gradient directions, whether an externally-generated list of gradient directions can be used, and restrictions on the echo time (TE). One challenge of multi-site studies is to create a common imaging protocol that will result in a reliable and accurate set of diffusion metrics. The present study describes the effect of site, scanner vendor, field strength, and TE on two common metrics: the first moment of the diffusion tensor field (mean diffusivity, MD), and the fractional anisotropy (FA). We have shown in earlier work that ROI metrics and the mean of MD and FA histograms are not sufficiently sensitive for use in site characterization. Here we use the distance between whole brain histograms of FA and MD to investigate within- and between-site effects. We concluded that the variability of DTI metrics due to site, vendor, field strength, and echo time could influence the results in multi-center trials and that histogram distance is sensitive metrics for each of these variables.

Construction Site Workers’ Awareness on Using Safety Equipment: Case Study

Directory of Open Access Journals (Sweden)

Ulang N. Md

2014-01-01

Full Text Available Construction sector is an important sector and contributed significantly to national development. However, this sector poses higher risk to accident. This is due to fact that construction site can be considered as a dangerous zone to workers and to the public. Due to the variety of cases occurs on site, the contractor will usually have to pay the cost related to accidents in the form of higher insurance premium. Despite various measures, accidents still occur at construction sites. Personal Protective Equipment (PPE is one of the important means to protect the wearer from hazards in the workplace. Since this equipment is the last frontier of the wearer from worksite hazards, it is important to select it based on the job scope and the intended protection. Therefore, this study was formulated to find out the level of knowledge and awareness of construction workers on PPE usage. It was also important to know what make the workers would want or do not want to use the PPE. It was found in this study that the level of awareness and knowledge among workers on the proper use of PPE is moderate. Construction sites accident can be further be reduced with proper implementation of PPE voluntarily by all workers.

A Comprehensive Mixture of Tobacco Smoke Components Retards Orthodontic Tooth Movement via the Inhibition of Osteoclastogenesis in a Rat Model

Directory of Open Access Journals (Sweden)

Maya Nagaie

2014-10-01

Full Text Available Tobacco smoke is a complex mixture of numerous components. Nevertheless, most experiments have examined the effects of individual chemicals in tobacco smoke. The comprehensive effects of components on tooth movement and bone resorption remain unexplored. Here, we have shown that a comprehensive mixture of tobacco smoke components (TSCs attenuated bone resorption through osteoclastogenesis inhibition, thereby retarding experimental tooth movement in a rat model. An elastic power chain (PC inserted between the first and second maxillary molars robustly yielded experimental tooth movement within 10 days. TSC administration effectively retarded tooth movement since day 4. Histological evaluation disclosed that tooth movement induced bone resorption at two sites: in the bone marrow and the peripheral bone near the root. TSC administration significantly reduced the number of tartrate-resistant acid phosphatase (TRAP-positive osteoclastic cells in the bone marrow cavity of the PC-treated dentition. An in vitro study indicated that the inhibitory effects of TSCs on osteoclastogenesis seemed directed more toward preosteoclasts than osteoblasts. These results indicate that the comprehensive mixture of TSCs might be a useful tool for detailed verification of the adverse effects of tobacco smoke, possibly contributing to the development of reliable treatments in various fields associated with bone resorption.

Atherosclerosis risk factors in pigeon squabs

International Nuclear Information System (INIS)

Klumpp, S.A.; Clarkson, T.B.

1986-01-01

The basis for atherosclerosis susceptibility of White Carneau (WC) and resistance of Show Racer (SR) pigeons is not known. Body weight (BW), total serum cholesterol (TSC), growth of the aorta and replication of endothelial cells of the distal thoracic aorta (lesion prone site) of 1, 2 and 4 week old squabs were studied. Aortic measurements were determined morphometrically, and endothelial cell replication was quantitated by 24-hour 3 H-thymidine labeling and whole-mount SEM autoradiography. From hatching to 4 weeks, BW increased more in WC than SR (22 to 473 gm in WC vs 19 to 416 gm in SR, p 2 ) in WC and 44% (101, 140 and 146 mm 2 ) in SR. Aortic surface area was significantly larger (0 = 0.002) in the 4 week WC than 4 week SR. 3 H-thymidine labeled endothelial cells at 1, 2 and 4 weeks were 783, 387 and 53 in WC and 674, 283 and 27 cells/mm 2 in SR. Endothelial replication in the 4 week WC was twice that of the SR and significantly different between breeds at 2 and 4 weeks (p = 0.04; p = 0.02, respectively). Higher TSC, endothelial cell replication and larger aortic surface area in the WC may be contributing factors to increased atherosclerosis susceptibility

A study plan for determining recharge rates at the Hanford Site using environmental tracers

International Nuclear Information System (INIS)

Murphy, E.M.; Szercsody, J.E.; Phillips, S.J.

1991-02-01

This report presents a study plan for estimating recharge at the Hanford Site using environmental tracers. Past operations at the Hanford Site have led to both soil and groundwater contamination, and recharge is one of the primary mechanisms for transporting contaminants through the vadose zone and into the groundwater. An alternative to using fixed lysimeters for determining recharge rates in the vadose zone is to use environmental tracers. Tracers that have been used to study water movement in the vadose zone include total chloride, 36 Cl, 3 H, and 2 H/ 18 O. Atmospheric levels of 36 Cl and 3 H increased during nuclear bomb testing in the Pacific, and the resulting ''bomb pulse'' or peak concentration can be measured in the soil profile. Locally, past operations at the Hanford Site have resulted in the atmospheric release of numerous chemical and isotopic tracers, including nitrate, 129 I, and 99 Tc. Seven study sites on the Hanford Site have been selected, in two primary soil types that are believed to represent the extremes in recharge, the Quincy sand and the Warden silt loam. An additional background study site upwind of the Hanford facilities has been chosen at the Yakima Firing Center. Six tracer techniques (total chloride, 36 Cl, 3 H, nitrate, 129 I, and 99 Tc) will be tested on at least one site in the Quincy sand, one site in the Warden silt loam, and the background site, to determine which combination of tracers works best for a given soil type. In subsequent years, additional sites will be investigated. The use of environmental tracers is perhaps the only cost-effective method for estimating the spatial variability of recharge at a site as large as Hanford. The tracer techniques used at Hanford have wide applicability at other arid sites. 166 refs., 41 figs., 16 tabs

Site study plan for geochemical analytical requirements and methodologies: Revision 1

International Nuclear Information System (INIS)

1987-12-01

This site study plan documents the analytical methodologies and procedures that will be used to analyze geochemically the rock and fluid samples collected during Site Characterization. Information relating to the quality aspects of these analyses is also provided, where available. Most of the proposed analytical procedures have been used previously on the program and are sufficiently sensitive to yield high-quality analyses. In a few cases improvements in analytical methodology (e.g., greater sensitivity, fewer interferences) are desired. Suggested improvements to these methodologies are discussed. In most cases these method-development activities have already been initiated. The primary source of rock and fluid samples for geochemical analysis during Site Characterization will be the drilling program, as described in various SRP Site Study Plans. The Salt Repository Project (SRP) Networks specify the schedule under which the program will operate. Drilling will not begin until after site ground water baseline conditions have been established. The Technical Field Services Contractor (TFSC) is responsible for conducting the field program of drilling and testing. Samples and data will be handled and reported in accordance with established SRP procedures. A quality assurance program will be utilized to assure that activities affecting quality are performed correctly and that the appropriate documentation is maintained. 28 refs., 9 figs., 14 tabs

Groundwater numerical modelling of the Fjaellveden study site - evaluation of parameter variations

International Nuclear Information System (INIS)

Larsson, N.A.

1987-10-01

The sensitivity/uncertainty of the hydraulic conductivity distribution in crystalline rocks is considered at the Fjaellveden study site - a site included in the Swedish site selection programme for final storage of spent nuclear fuel. A three-dimensional FEM-model assuming steady-state flow with constant fluid properties under saturated conditions is used. The bedrock of the site is divided into three hydraulic units; rock mass, local and regional fracture zones. The data set of hydraulic conductivity of each unit has been treated statistically in various ways, reflecting different aspects of the physical conditions of the site. A total of nine cases have been prepared, all based on 214 data points. (orig./HP)

Evaluation of decision making in technical support center for effective severe accident management

International Nuclear Information System (INIS)

Huh, C.; Suh, N.

2010-01-01

We have evaluated the technical and organizational aspect of the current SAMG focusing on the decision making process in TSC. Technically, we found that the current SAMG is a good software kind of tool which can help operators to manage the severe accident. But the clear cutting of EOP and SAMG, shift of plant control from MCR to TSC seems to have no firm ground to be accepted as it is. Study on the organizational behavior shows that the group decision under risky situation will be inevitably polarized either toward risky or cautious way. Since the current SAMG makes TSC to evaluate the pros and cons of strategies to be implemented and choose one based on group decision, we are not free of this group polarization phenomenon. We propose that the proven organization of EOP needs to be maintained and also that the SAMG needs to be more proceduralized. (authors)

Cognitive Challenges

Science.gov (United States)

... Alliance Our Story Our Vision Our Team Our Leadership Our Results Our Corporate Policies FAQs Careers Contact Us Media Store Privacy Policy Sitemap Learn Engage Donate About TSC Cognitive Challenges Approximately 45% to 60% of individuals with TSC ...

Study on detecting leachate leakage of municipal solid waste landfill site.

Science.gov (United States)

Liu, Jiangang; Cao, Xianxian; Ai, Yingbo; Zhou, Dongdong; Han, Qiting

2015-06-01

The article studies the detection of the leakage passage of leachate in a waste landfill dam. The leachate of waste landfill has its own features, like high conductivity, high chroma and an increasing temperature, also, the horizontal flow velocity of groundwater on the leakage site increases. This article proposes a comprehensive tracing method to identify the leakage site of an impermeable membrane by using these features. This method has been applied to determine two leakage sites of the Yahu municipal solid waste landfill site in Pingshan District, Shenzhen, China, which shows that there are two leachate leakage passages in the waste landfill dam A between NZK-2 and NZK-3, and between NZK-6 and NZK-7. © The Author(s) 2015.

Salt repository project site study plan for water resources: Revision 1

International Nuclear Information System (INIS)

1987-12-01

The Site Study Plan for Water Resources describes a field program consisting of surface-water and ground-water characterization. The surface-water studies will determine the drainage basin characteristics (i.e., topography, soils, land use), hydrometeorology, runoff to streams and playas, and surface-water quality (i.e., offsite pollution sources in playa lakes and in streams). The environmental ground-water studies will focus on ground-water quality characterization. The site study plan describes for each study the need for the study, study design, data management and use, schedule of proposed activities, and quality assurance. These studies will provide data needed to satisfy requirements contained in, or derived from, the Salt Repository Projects Requirements Document. 78 refs., 8 figs., 5 tabs

Systemic disease manifestations associated with epilepsy in tuberous sclerosis complex.

Science.gov (United States)

Jeong, Anna; Wong, Michael

2016-09-01

Epilepsy is one of the most disabling symptoms of tuberous sclerosis complex (TSC) and is a leading cause of morbidity and mortality in affected individuals. The relationship between systemic disease manifestations and the presence of epilepsy has not been thoroughly investigated. This study utilizes a multicenter TSC Natural History Database including 1,816 individuals to test the hypothesis that systemic disease manifestations of TSC are associated with epilepsy. Univariate analysis was used to identify patient characteristics (e.g., age, gender, race, and TSC mutation status) associated with the presence of epilepsy. Individual logistic regression models were built to examine the association between epilepsy and each candidate systemic or neurologic disease variable, controlling for the patient characteristics found to be significant on univariate analysis. Finally, a multivariable logistic regression model was constructed, using the variables found to be significant on the individual analyses as well as the patient characteristics that were significant on univariate analysis. Nearly 88% of our cohort had a history of epilepsy. After adjusting for age, gender, and TSC mutation status, multiple systemic disease manifestations including cardiac rhabdomyomas (odds ratio [OR] 2.3, 95% confidence interval [CI] 1.3-3.9, p = 0.002), retinal hamartomas (OR 2.1, CI 1.0-4.3, p = 0.04), renal cysts (OR 2.1, CI 1.3-3.4, p = 0.002), renal angiomyolipomas (OR 3.0, CI 1.8-5.1, p epilepsy. In the multivariable logistic regression model, cardiac rhabdomyomas (OR 1.9, CI 1.0-3.5, p = 0.04) remained significantly associated with the presence of epilepsy. The identification of systemic disease manifestations such as cardiac rhabdomyomas that confer a higher risk of epilepsy development in TSC could contribute to disease prognostication and assist in the identification of individuals who may receive maximal benefit from potentially novel, targeted, preventative therapies. Wiley

Evaluating Origin of Electron Traps in Tris(8-hydroxyquinoline) Aluminum Thin Films using Thermally Stimulated Current Technique

OpenAIRE

Matsushima, Toshinori; Adachi, Chihaya

2008-01-01

We measured the energy distributions and concentrations of electron traps in O_2-unexposed and O_2-exposed tris(8-hydroxyquinoline) aluminum (Alq_3) films using a thermally stimulated current (TSC) technique to investigate how doping O_2 molecules in Alq_3 films affect the films' electron trap and electron transport characteristics. The results of our TSC studies revealed that Alq_3 films have an electron trap distribution with peak depths ranging from 0.075 to 0.1 eV and peak widths ranging ...

Evaluation of the potential application of 2-acetylpyridine N4- phenyl thiosemicarbazones derivatives for cancer therapy and diagnosis; Avaliacao da potencial aplicacao de derivados de 2-acetilpiridina N-4 fenil tiossemicarbazonas em terapia e diagnostico oncologico

Energy Technology Data Exchange (ETDEWEB)

Soares, Marcella Araugio

2013-08-01

Despite the wide range of antineoplastic agents available, resistance of some types of cancer and toxicity to normal cells have been identified as the main causes of treatment failure and death. The lack of early and precise diagnosis is also responsible for reducing survival of cancer patients. In this context, the development of substances with low toxicity and therapeutic potential and/or diagnosis purpose, is the major tool in an attempt to increase the survival of patients and assure the safety and efficacy of treatment. Thiosemicarbazones (TSC) are a class of synthetic compounds that have several biological activities, including antitumor. Although several studies have shown the great potential of TSC as therapeutic and / or diagnostic agents, different chemical modifications performed on this class of molecules indicate new possibilities for applications and still require further studies. The objective of this study was to evaluate the potential applicability of 2-acetylpyridine N-4-phenyl thiosemicarbazones derivatives for cancer therapy and diagnosis. The results showed that all 13 TSC tested were cytotoxic to breast and glioblastoma tumor cell lines, presenting higher in vitro antitumor activity than etoposide, an antineoplastic and inhibitor of topoisomerase II frequently used for cancer therapy. The TSC that have halogen or nitro on ortho position showed higher antitumor activity in vitro than their isomers with halogen or nitro on meta or para position of the phenyl group. H2Ac4oFPh and H2Ac4oClPh compounds showed the highest antitumor activity among all tested compounds, with IC{sub 50} in nanomolar order. These TSC induced cell death by apoptosis and oxidative stress was responsible, at least in part, for this type of cell death. The 5 mg.kg{sup -1} H2Ac4oFPh dose, administered s.c., for 4 consecutive days, did not induce important toxicity; however, the same treatment protocol was not effective for tumor growth reduction in an animal model of brain

Evaluation of the potential application of 2-acetylpyridine N4- phenyl thiosemicarbazones derivatives for cancer therapy and diagnosis

International Nuclear Information System (INIS)

Soares, Marcella Araugio

2013-01-01

Despite the wide range of antineoplastic agents available, resistance of some types of cancer and toxicity to normal cells have been identified as the main causes of treatment failure and death. The lack of early and precise diagnosis is also responsible for reducing survival of cancer patients. In this context, the development of substances with low toxicity and therapeutic potential and/or diagnosis purpose, is the major tool in an attempt to increase the survival of patients and assure the safety and efficacy of treatment. Thiosemicarbazones (TSC) are a class of synthetic compounds that have several biological activities, including antitumor. Although several studies have shown the great potential of TSC as therapeutic and / or diagnostic agents, different chemical modifications performed on this class of molecules indicate new possibilities for applications and still require further studies. The objective of this study was to evaluate the potential applicability of 2-acetylpyridine N-4-phenyl thiosemicarbazones derivatives for cancer therapy and diagnosis. The results showed that all 13 TSC tested were cytotoxic to breast and glioblastoma tumor cell lines, presenting higher in vitro antitumor activity than etoposide, an antineoplastic and inhibitor of topoisomerase II frequently used for cancer therapy. The TSC that have halogen or nitro on ortho position showed higher antitumor activity in vitro than their isomers with halogen or nitro on meta or para position of the phenyl group. H2Ac4oFPh and H2Ac4oClPh compounds showed the highest antitumor activity among all tested compounds, with IC 50 in nanomolar order. These TSC induced cell death by apoptosis and oxidative stress was responsible, at least in part, for this type of cell death. The 5 mg.kg -1 H2Ac4oFPh dose, administered s.c., for 4 consecutive days, did not induce important toxicity; however, the same treatment protocol was not effective for tumor growth reduction in an animal model of brain tumor

Siting Study for the Remote-Handled Low-Level Waste Disposal Project

Energy Technology Data Exchange (ETDEWEB)

Lisa Harvego; Joan Connolly; Lance Peterson; Brennon Orr; Bob Starr

2010-10-01

The U.S. Department of Energy has identified a mission need for continued disposal capacity for remote-handled low-level waste (LLW) generated at the Idaho National Laboratory (INL). An alternatives analysis that was conducted to evaluate strategies to achieve this mission need identified two broad options for disposal of INL generated remote-handled LLW: (1) offsite disposal and (2) onsite disposal. The purpose of this study is to identify candidate sites or locations within INL boundaries for the alternative of an onsite remote handled LLW disposal facility and recommend the highest-ranked locations for consideration in the National Environmental Policy Act process. The study implements an evaluation based on consideration of five key elements: (1) regulations, (2) key assumptions, (3) conceptual design, (4) facility performance, and (5) previous INL siting study criteria, and uses a five-step process to identify, screen, evaluate, score, and rank 34 separate sites located across INL. The result of the evaluation is identification of two recommended alternative locations for siting an onsite remote-handled LLW disposal facility. The two alternative locations that best meet the evaluation criteria are (1) near the Advanced Test Reactor Complex and (2) west of the Idaho Comprehensive Environmental Response, Compensation, and Liability Act Disposal Facility.

Radiotracers for per studies of neurotransmitter binding sites: Design considerations

International Nuclear Information System (INIS)

Kilbourn, M.R.

1991-01-01

Neurotransmitter binding sites, such as receptors, neuronal uptake systems, and vesicular uptake systems, are important targets for new radiopharmaceutical design. Selection of potential radioligands can be guided by in vitro laboratory data including such characteristics as selectivity and affinity for specific binding sites. However, development of PET radiotracers for use in vivo must include considerations of in vivo pharmacokinetics and metabolism. Introduction of potential radioligands is further narrowed by the demands of the radiochemical synthesis, which must produce radioligands of high chemical and radiochemical purity and of high specific activity. This paper will review examples of previous and current attempts by radiopharmaceutical chemists to meet these demands for new positron emitter-labeled radioligands for PET studies of a wide array of neurotransmitter binding sites

MOCAT project: innovation applied to the improvement of emergency management in the nuclear power Garona

International Nuclear Information System (INIS)

Callejo, J. L.; Caro, R. J.

2009-01-01

With the aim of improving the Emergency Management, the Nuclear Power Station of Santa Maria de Garona has undertaken Technical Support Centre (TSC) Modernization Project MOCAT. Developed by Tecnatom, it applies the new Information Technologies to the management of the information available in the TSC and computerizes the procedures associated to the different areas from the TSC. First stage structures the relevant information in two sheets for Operation and Radiation control Areas and mechanizes the Radiological control Area Manager guideline, the Event Management and the emergency Direct Screen. (Author)

Site study plan for EDBH [Engineering Design Boreholes] seismic surveys, Deaf Smith County site, Texas: Revision 1

International Nuclear Information System (INIS)

Hume, H.

1987-12-01

This site study plan describes seismic reflection surveys to run north-south and east-west across the Deaf Smith County site, and intersecting near the Engineering Design Boreholes (EDBH). Both conventional and shallow high-resolution surveys will be run. The field program has been designed to acquire subsurface geologic and stratigraphic data to address information/data needs resulting from Federal and State regulations and Repository program requirements. The data acquired by the conventional surveys will be common-depth- point, seismic reflection data optimized for reflection events that indicate geologic structure near the repository horizon. The data will also resolve the basement structure and shallow reflection events up to about the top of the evaporite sequence. Field acquisition includes a testing phase to check/select parameters and a production phase. The field data will be subjected immediately to conventional data processing and interpretation to determine if there are any anamolous structural for stratigraphic conditions that could affect the choice of the EDBH sites. After the EDBH's have been drilled and logged, including vertical seismic profiling, the data will be reprocessed and reinterpreted for detailed structural and stratigraphic information to guide shaft development. The shallow high-resulition seismic reflection lines will be run along the same alignments, but the lines will be shorter and limited to immediate vicinity of the EDBH sites. These lines are planned to detect faults or thick channel sands that may be present at the EDBH sites. 23 refs. , 7 figs., 5 tabs

Design of an environmental site assessment template for open radioactive site contamination : a radioecological risk approach and case study

International Nuclear Information System (INIS)

Nguyen, T.

2004-01-01

To reduce redundancy, cost, and time, while at the same time ultimately increasing the effectiveness of the radioactive risk management process, a logical framework incorporating risk assessments (human cancer and environmental risks) into the environmental site assessment process was designed for radioactive open site contamination. Risk-based corrective action is becoming an increasingly more acceptable approach for the remediation of contaminated sites. In the past, cleanup goals were usually established without any regard to the risk involved, by mandating remediation goals based solely on maximum contamination levels. Now, a multi-stage environmental site assessment template has been developed on a radioecological approach. The template gives a framework for making environmentally sound decisions based on relevant regulations and guidelines. The first stage involves the comparison of the background screening activity level to the regulated activity level, the second stage involves the use of site-specific information to determine the risk involved with the contamination, and the third stage provides a remediation decision matrix based on results from the first two stages. This environmental site assessment template is unique because it incorporates the modified Canadian National Classification System for radioactive contaminated sites and two different types of risk assessments (human cancer risks and the newly designed ecological risk) into the decision making process. The template was used to assess a radiologically contaminated site at the Canadian Forces Base at Suffield (Alberta) as a case study, and it reaffirms the Department of National Defence's action as appropriate. This particular site is a Class 3, has an overall insignificant human cancer risk ( -6 ) and a low environmental risk, and conforms to all regulated guidelines. Currently, it is restricted and should be left as is, provided that the subsurface is not disturbed. (author)

Site study plan for ecology, Deaf Smith County Site, Texas: Environmental Field Program: Preliminary Draft

International Nuclear Information System (INIS)

1987-06-01

The Ecology Site Study Plan describes a field program consisting of studies which include surveys for endangered, threatened, and candidate species; vegetation characterization, including mapping and cover typing, plant succession, wetlands description, and preexisting stresses; and wildlife community characterization, including availability and quality of habitats and descriptions of mammal, bird, reptile, amphibian, and invertebrate populations. The plan for each study describes the need for the study, study design, data management and use, schedule and personnel requirements, and quality assurance. These studies will provide data needed to satisfy requirements contained in, or derived from, the Salt Repository Project Requirements Document (SRP-RD). 83 refs., 3 tabs

Luminescence and conductivity studies on CVD diamond exposed to UV light

CERN Document Server

Bizzarri, A; Bruzzi, M; Sciortino, S

1999-01-01

The photoluminescence (PL), thermoluminescence (TL) and thermally stimulated currents (TSC) of four high-quality CVD diamond films have been investigated in the range of temperatures between 300 and 700 K. The sample excitation has been carried out by means of an UV xenon lamp and UV laser lines. The features of the signals have been found equal to those obtained from particle excitation. The TL analysis shows the existence of several deep traps with activation energies between 0.6 and 1.0 eV. The contribution to the TL signal from different traps has been singled out by means of successive annealing processes. The TL results are in good agreement with those obtained from TSC measurements. The combined use of the two techniques allows a precise determination of the trap parameters. The spectral content of the TL response has also been compared with the PL signal in order to investigate the recombination process. This analysis shows that, in this temperature range, the TL signal is likely due to recombination ...

Parameters of importance to determine in geoscientific site studies

International Nuclear Information System (INIS)

Andersson, Johan; Almen, K.E.; Ericsson, L.O.; Karlsson, Fred; Stroem, A.; Fredriksson, Anders; Stanfors, R.

1996-12-01

This report identifies and describes parameters, that may be determined in a site characterization study, for performing functional and safety analyses of a deep rock repository for radioactive wastes. The report discusses data needs for rock engineering and for description of other environmental aspects. It is intended that the report be used as a basis for formulating the criteria of acceptance in evaluating a candidate site. The report describes how different parameters influence the safety function, and how they are evaluated in practice. The logical order of performing measurements, due to the need of in-data and influence on other measurements is also discussed. 65 refs

Engineering study of generic site criteria for selected DOE plutonium facilities

International Nuclear Information System (INIS)

Kingsbury, R.J.; Greenwood, J.M.; Sandoval, M.D.

1980-09-01

The objectives of this study were to identify criteria that would be applied to selection of a site for plutonium facilities such as those at the Rocky Flats Plant, to establish the relative importance of these criteria, and to identify suitable areas within the United States for location of plutonium facilities with respect to these criteria. Sources of the site criteria identified include federal laws, federal agency regulations, state laws and regulations, and requirements associated with operations to be performed at the site. The criteria identified during the study were organized into 14 major categories. The relative importnace of each category and each criterion within the categories were established using group decision-making techniques. The major criteria categories, their assigned weight on a scale of 1 to 10, and their relative priority ranks are as follows: geology/seismicity; public safety; environmental impact; meteorology; hydrology; topography; transportation; utilities; personnel; safeguards/security; land area and availability; land use compatibility; and, public acceptance. A suitability analysis of the continental United States was performed using only those criteria that could be mapped at a national scale. Suitability was assessed with respect to each of these criteria, and individual suitability maps were prepared. A composite suitability map was generated using computerized overlay techniques. This map provides a starting point for identifying specific candidate sites if an actual site selection were to be conducted

A NEW FRAMEWORK FOR GEOSPATIAL SITE SELECTION USING ARTIFICIAL NEURAL NETWORKS AS DECISION RULES: A CASE STUDY ON LANDFILL SITES

Directory of Open Access Journals (Sweden)

S. K. M. Abujayyab

2015-10-01

Full Text Available This paper briefly introduced the theory and framework of geospatial site selection (GSS and discussed the application and framework of artificial neural networks (ANNs. The related literature on the use of ANNs as decision rules in GSS is scarce from 2000 till 2015. As this study found, ANNs are not only adaptable to dynamic changes but also capable of improving the objectivity of acquisition in GSS, reducing time consumption, and providing high validation. ANNs make for a powerful tool for solving geospatial decision-making problems by enabling geospatial decision makers to implement their constraints and imprecise concepts. This tool offers a way to represent and handle uncertainty. Specifically, ANNs are decision rules implemented to enhance conventional GSS frameworks. The main assumption in implementing ANNs in GSS is that the current characteristics of existing sites are indicative of the degree of suitability of new locations with similar characteristics. GSS requires several input criteria that embody specific requirements and the desired site characteristics, which could contribute to geospatial sites. In this study, the proposed framework consists of four stages for implementing ANNs in GSS. A multilayer feed-forward network with a backpropagation algorithm was used to train the networks from prior sites to assess, generalize, and evaluate the outputs on the basis of the inputs for the new sites. Two metrics, namely, confusion matrix and receiver operating characteristic tests, were utilized to achieve high accuracy and validation. Results proved that ANNs provide reasonable and efficient results as an accurate and inexpensive quantitative technique for GSS.

The effect of tobacco smoke exposure on the generation of reactive oxygen species and cellular membrane damage using co-culture model of blood brain barrier with astrocytes.

Science.gov (United States)

Seo, Seung-Beom; Choe, Eun Sang; Kim, Kwang-Sik; Shim, Soon-Mi

2017-06-01

Brain tissue is known to be vulnerable to the exposure by tobacco smoke. Tobacco smoke can induce generation of reactive oxygen species (ROS), causing inflammatory activity and blood-brain barrier (BBB) impairment. The aim of the present study was to investigate the effect of tobacco smoke on cell cytotoxicity, generation of ROS, and cellular membrane damage in astrocytes and BBB using a co-culture system. Cell viability of U373MG cells was reduced in a dose-dependent manner, ranging from 96.7% to 40.3% by tobacco smoke condensate (TSC). Cell viability of U373MG co-cultured with human brain microvascular endothelial cells (HBMECs) was 104.9% at the IC 50 value of TSC. Trans-epithelial electric resistance values drastically decreased 80% following 12-h incubation. The value was maintained until 48 h and then increased at 72-h incubation (85%). It then decreased to 75% at 120 h. Generation of ROS increased in a dose-dependent manner, ranging from 102.7% to 107.9%, when various concentrations of TSC (4-16 mg/mL) were administered to the U373MG monoculture. When TSC was added into U373MG co-cultured with HBMECs, production of ROS ranged from 101.7% to 102.6%, slightly increasing over 12 h. Maximum exposure-generated ROS of 104.8% was reached at 24 h. Cell cytotoxicity and oxidative stress levels in the U373MG co-culture model system with HBMECs were lower than U373MG monoculture. HBMECs effectively acted as a barrier to protect the astrocytes (U373MG) from toxicity of TSC.

Climate change research methods and its significance in the study of choosing candidate site in nuclear waste disposal sites

International Nuclear Information System (INIS)

Zhao Yong; Zhang Zhanshi

2008-01-01

A high-level nuclear waste is the inevitable product accompanies the development of the nuclear power station. How to dispose it properly has become focused by all over the world. Some of the important progresses have been achieved in the fields of site setting, performance assessment and underground laboratory recently. Palaeoclimate patter and the tendency of climate change are very important aspects for the site setting This paper discussed some of the important progresses on the disposal of unclear wastes, the influence of the climate change on the site setting and main methods such as lake sediments, marine sediment, loess, ancient soil and ice core deal with palaeoclimate and palaeo environment study. (authors)

Additional media studies for site suitability criteria

International Nuclear Information System (INIS)

Donich, T.R.; Kaufman, A.M.; Sauter, G.D.; Steinborn, T.L.; Towse, D.F.

1978-01-01

Site suitability studies have been made previously at LLL on bedded salt and shale. In the present study domed salt, basalt, and crystalline rock are compared with bedded salt and shale and with each other as possible repositories. The level of effort required to develop models for these media that are similar in quality to those available for bedded salt and shale is evaluated. The effort necessary to develop data bases on the physical and chemical properties comparable to that available for bedded salt and shale is also estimated. Each medium is evaluated as a suitable repository environment. The funding necessary for model and data base development is estimated

Siting-selection study for the Soyland Power Cooperative, Inc. , compressed-air energy-storage system (CAES)

Energy Technology Data Exchange (ETDEWEB)

1982-01-01

A method used for siting a compressed air energy storage (CAES) system using geotechnical and environmental criteria is explained using the siting of a proposed 220 MW water-compensated CAES plant in Illinois as an example. Information is included on the identification and comparative ranking of 28 geotechnically and environmental sites in Illinois, the examination of fatal flaws, e.g., mitigation, intensive studies, costly studies, permit denials, at 7 sites; and the selection of 3 sites for further geological surveying. (LCL)

Analysis of twenty pediatric cases of tuberous sclerosis complex: Are we doing enough?

Directory of Open Access Journals (Sweden)

Jyoti Nath

2015-01-01

Full Text Available Background: Tuberous sclerosis complex (TSC is a neuro-cutaneous disease characterized by hamartoma formation in various organs particularly the skin, brain, eye, kidney, heart and lungs. Patients usually have multisystem involvement and thus present to different medical specialties with varied complaints while the true nature of the disease and the hidden manifestations may remain unattended. A im: To assess the frequency and characteristics of various cutaneous and systemic manifestations in TSC and the total impact on different system in a cohort of pediatric patients. Methods: The study included 20 patients fulfilling the diagnostic criteria for TSC from dermatology, pediatrics, and neurology department. Detailed history, examination, and investigations such as chest X-ray, electrocardiography (ECG, ultrasonography (USG abdomen, echocardiography, fundoscopy, computed tomography (CT scan of brain and abdomen were done. Results: Dermatological manifestations included ash leaf macules, angiofibromas, shagreen patch, and fibrous plaque. Systemic findings observed were subependymal calcified nodules, subependymal giant cell astrocytoma, cortical tubers, renal cysts, angiomyolipomas, lung cyst, retinal hamartomas, mental deficits and epilepsy. Limitations: Wechsler′s Intelligence Scale for Children would have been more appropriate for assessing the intelligence. Dental check-up was not done in our group of patients. Conclusion: Our study highlights the wide variety of cutaneous and systemic manifestations of TSC. The study emphasizes the need for comprehensive multidisciplinary treatment and periodic follow-up which are necessary for appropriate management of this multisystem disorder. Counseling regarding education and rehabilitation of the patients and genetic counseling of parents are important.

National radioactive waste repository site selection study. Phase 2. A report on public comment

International Nuclear Information System (INIS)

1995-11-01

Agreement was reached in principle between State/Territory and the Commonwealth of Australia Governments that a suitable site for a radioactive wastes repository must be found. The discussion papers resulting from the Phase 1 and Phase 2 of the site selection study were released for public comment. The national repository will be for disposal of low level and short-lived intermediate level radioactive wastes streaming from the medical, research and industrial use of radioisotopes in Australia. The purpose of this report is to summarise and respond in general terms to comment received on the discussion paper -Phase 2 of the study. Forty five submissions were received. Of these: 18 supported the Phase 2 study approach and the concept of a national repository; 13 did not state a clear position but either requested more information or provided constructive comment on the siting process; 7 supported the site selection approach and the repository concept but suggested that the repository should not be sited in a particular area; 3 opposed the siting of the repository in their vicinity but not necessarily the repository concept and site selection approach; 4 opposed the concept of a national repository. This compares with 124 submissions on Phase 1 of the study, of which 57 opposed the national repository concept (52 of these were from letters elicited by Greenpeace) and 48 supported the establishment of a national repository and the site selection approach proposed. 3 figs

Thermophysical Properties of the Phoenix Mars Landing Site Study Regions

Science.gov (United States)

Putzig, N. E.; Mellon, M. T.; Golombek, M. P.; Arvidson, R. E.

2006-03-01

Analysis of Phoenix Mars study regions places 4 of 5 in a previously-identified duricrust-dominated thermophysical unit which also contains the Viking and Spirit landing sites. Extrapolation of lander-observed properties to the study regions may be complicated by surface heterogeneity.

An economic study of the site of Marcoule

International Nuclear Information System (INIS)

Duprat, H.

1964-01-01

The study of this site of the Marcoule Center shows that its regional economical influence is limited to the buying power distributed among its personnel. This entails distortions between first this supplementary buying power and the overall markets within the possible Marcoule influence zone, and secondly the concentration on Bagnols-sur-Ceze and this town's normal extension possibilities. (author) [fr

Stability of the resistance to the thiosemicarbazone derived from 5,6-Dimethoxy-1-indanone, a non-nucleoside polymerase inhibitor of bovine viral diarrhea virus

OpenAIRE

Castro, Eliana Florencia; Campos, Rodolfo Hector; Cavallaro, Lucía Vicenta

2017-01-01

Bovine viral diarrhea virus (BVDV) is the prototype Pestivirus. BVDV infection is distributed worldwide and causes serious problems for the livestock industry. The thiosemicarbazone of 5,6-dimethoxy-1-indanone (TSC) is a non-nucleoside polymerase inhibitor (NNI) of BVDV. All TSC-resistant BVDV variants (BVDV-TSCr T1–5) present an N264D mutation in the NS5B gene (RdRp) whereas the variant BVDV-TSCr T1 also presents an NS5B A392E mutation. In the present study, we carried out twenty passages of...

Coexistence of multiphase superconductivity and ferromagnetism in lithiated iron selenide hydroxide [(L i1 -xF ex) OH ]FeSe

Science.gov (United States)

Urban, Christian; Valmianski, Ilya; Pachmayr, Ursula; Basaran, Ali C.; Johrendt, Dirk; Schuller, Ivan K.

2018-01-01

We present experimental evidence for (a) multiphase superconductivity and (b) coexistence of magnetism and superconductivity in a single structural phase of lithiated iron selenide hydroxide [(L i1 -xF ex )OH]FeSe. Magnetic field modulated microwave spectroscopy data confirms superconductivity with at least two distinct transition temperatures attributed to well-defined superconducting phases at TSC 1=40 ±2 K and TSC 2=35 ±2 K. Magnetometry data for the upper critical fields reveal a change in the magnetic order (TM=12 K) below TSC 1 and TSC 2 that is consistent with ferromagnetism. This occurs because the superconducting coherence length is much smaller than the structural coherence length, allowing for several different electronic and magnetic states on a single crystallite. The results give insight into the physics of complex multinary materials, where several phenomena governed by different characteristic length scales coexist.

KETERKAITAN PERSEPSI DAN PERILAKU PENGUNJUNG TERHADAP KESEJAHTERAAN RUSA TIMOR DI TAMAN SATWA CIKEMBULAN GARUT

Directory of Open Access Journals (Sweden)

Anggita Puspitasari

2016-01-01

Full Text Available Rusa deer management in Taman Satwa Cikembulan (TSC as one of the conservation institution needs to consider animal welfare, perceiption, and behavior of visitors, order to optimize the function of Rusa deer as an exhibition wildlife. The data were collected on March to April 2014 in TSC, Garut, gains by interview and questionnaire. This research shown that animal welfare implementation of Timor deer in TSC rank into sufficient. Health management wasn’t yet optimal and it influence pain and injury aspect has become the lowest value. Visitors behavior wasn’t relate significantly with animal welfare assesment, nevertheless if animal welfare emendation done by manager, it will increase visitors anxiety of Timor deer. Animal welfare emendetaion, management of visitors, additional utilities and infrastructures are some manegement which can minimize the annoying behaviors by visitors in TSC. Keyword : animal welfare, behavior, perception, Rusa deer

Metastatic Group 3 Medulloblastoma in a Patient With Tuberous Sclerosis Complex: Case Description and Molecular Characterization of the Tumor.

Science.gov (United States)

Moavero, Romina; Folgiero, Valentina; Carai, Andrea; Miele, Evelina; Ferretti, Elisabetta; Po, Agnese; Diomedi Camassei, Francesca; Lepri, Francesca Romana; Vigevano, Federico; Curatolo, Paolo; Valeriani, Massimiliano; Colafati, Giovanna S; Locatelli, Franco; Tornesello, Assunta; Mastronuzzi, Angela

2016-04-01

Medulloblastoma is the most common pediatric brain tumor. We describe a child with tuberous sclerosis complex that developed a Group 3, myc overexpressed, metastatic medulloblastoma (MB). Considering the high risk of treatment-induced malignancies, a tailored therapy, omitting radiation, was given. Based on the evidence of mammalian target of rapamycin mTORC, mTOR Complex; RAS, Rat sarcoma; RAF, rapidly accelerated fibrosarcoma (mTOR) pathway activation in the tumor, targeted therapy was applied resulting in complete remission of disease. Although the PI3K/AKT/mTOR signaling pathway plays a role in MB, we did not find TSC1/TSC2 (TSC, tuberous sclerosis complex) mutation in our patient. We speculate that a different pathway resulting in mTOR activation is the basis of both TSC and MB in this child; H&E, haematoxilin and eosin; Gd, gadolinium. © 2015 Wiley Periodicals, Inc.

Metadata in Arabic Libraries' Web Sites in Egypt and Saudi Arabia : An Applied Study

Directory of Open Access Journals (Sweden)

Zain A.Hady

2005-03-01

Full Text Available An Applied study aims at analyzes the metadata of Arabic Libraries' Web Sites in Egypt and Saudi Arabia, it begins with a methodological introduction, then the study analyzes the web sites using Meta Tag Analyzer software, it included the following web sites : Library of Alexandria, Egyptian Libraries, Egyptian National, King Fahd National Library, King Abdel Aziz Public Library, and Mubarak Public Library.

Challenges in radiological impact assessment studies at new sites for nuclear facilities and its safety review and assessment for siting consent

International Nuclear Information System (INIS)

Mukherjee Roy, Susmita; Roshan, A.D.; Bishnoi, L.R.

2018-01-01

One of the basic requirement of site evaluation for a Nuclear Facility (NF) is radiological impact assessment (RIA). This involves evaluation of transportation of radioactive materials discharged from a nuclear facility under normal operational or accidental conditions, through different compartments of environment viz. air, land and water, and finally assessment of its consequences. Amongst others, site characteristics and the site related parameters play major role in evaluation of impact of postulated releases from NPPs. Doses to public from both external and internal exposures are computed to assess potential consequences of a radiological release and acceptability of the site-plant pair is established based on the outcome of this assessment. A comprehensive study of the site characteristics including meteorology, hydrology, hydro-geology and demography of the region along with details of land and water use, bioaccumulation, transfer to and from the environmental matrices is required for accomplishing satisfactory RIA

Site study plan for Upper Aquifer Hydrology Clusters, Deaf Smith County Site, Texas: Surface-based geotechnical field program: Preliminary draft

International Nuclear Information System (INIS)

1988-01-01

As part of site characterization studies, at the Deaf Smith County site, Texas, 15 wells at 5 locations will be completed in the Ogallala Formation and Dockum Group. The purposes of the wells, which are called Upper Aquifer (2) establish background hydrologic and water quality conditions, (3) provide analysis, (4) monitor responses of the shallow hydrologic system to site activities and nearby pumpage for irrigation, (5) collect water samples from both saturated and unsaturated materials to help define recharge rates and ground-water flow patterns, (6) monitor variations on water quality, and (7) define ground-water resources near the site. The test wells will be installed during a 14-month period starting about 1-1/2 years after site characterization activities begin. The Technical Field Services Contractor is responsible for conducting the field program of drilling and testing. Samples and data will be handled and reported in accordance with established Salt Repository Project procedures. A quality assurance program will assure that activities affecting quality are performed correctly and that the appropriate documentation is maintained. 44 refs., 19 figs., 5 tabs

Detailed site characterization for final disposal of spent fuel in Finland - Case study Loviisa

International Nuclear Information System (INIS)

Anttila, P.; Ahokas, H.; Ruotsalainen, P.; Cosma, C.; Keskinen, J.; Hinkkanen, H.; Rouhiainen, P.; Oehberg, A.

1998-01-01

The spent fuel from the Finnish nuclear power plants will be disposed of in the Finnish bedrock. Pos iva Oy is responsible for the site selection programme carried out in accordance with the governmental decisions. Preliminary site investigations were made in five areas in 1987-1992. Based on the results, three areas, Romuvaara in Kuhmo, Kivetty in Aeaenekoski and Olkiluoto in Eurajoki, were selected for the detailed site characterization in 1993-2000. The final site will be selected by the end of the year 2000. The interim reporting of the detailed studies of the three areas was made in 1996. In 1997, the island of Haestholmen, as the host to the Loviisa NPP, was included as a fourth candidate site in the programme for the detailed site investigations. The goal is to characterize this site also in detail by the end of 2000 to attain the same level of knowledge as available from the three other sites. The background information existing from the studies made for the construction of the repository for the low-and intermediate-level wastes will create a good basis to reach the target. The research programme for the detailed site characterization has mainly been focused on groundwater flow and geochemistry due to their importance in terms of long-term safety of the repository. Equipment and methodology development by Posiva has introduced new tools that provide more accurate data on relevant parameters than the ones used in previous stages of site characterization. The programme also contains studies for additional information of the structural and geological properties of the bedrock towards the depth. Also predictive modelling has been made for evaluating the relevance of the assumptions made. The methods applied in the site characterization have comprised, e.g., geological mapping, deep core drilling, groundwater sampling and analyzing, hydraulic testing and geophysical measurements

Detailed site characterization for final disposal of spent fuel in Finland - Case study Loviisa

Energy Technology Data Exchange (ETDEWEB)

Anttila, P. [IVO Power Engineering Ltd. (Finland); Ahokas, H.; Ruotsalainen, P. [Fintact Oy (Finland); Cosma, C.; Keskinen, J. [Vibrometric Oy (Finland); Hinkkanen, H. [Posiva Oy (Finland); Rouhiainen, P. [PRG-Tec Oy (Finland); Oehberg, A. [Saanio and Riekkola Consulting Engineers (Finland)

1998-09-01

The spent fuel from the Finnish nuclear power plants will be disposed of in the Finnish bedrock. Pos iva Oy is responsible for the site selection programme carried out in accordance with the governmental decisions. Preliminary site investigations were made in five areas in 1987-1992. Based on the results, three areas, Romuvaara in Kuhmo, Kivetty in Aeaenekoski and Olkiluoto in Eurajoki, were selected for the detailed site characterization in 1993-2000. The final site will be selected by the end of the year 2000. The interim reporting of the detailed studies of the three areas was made in 1996. In 1997, the island of Haestholmen, as the host to the Loviisa NPP, was included as a fourth candidate site in the programme for the detailed site investigations. The goal is to characterize this site also in detail by the end of 2000 to attain the same level of knowledge as available from the three other sites. The background information existing from the studies made for the construction of the repository for the low-and intermediate-level wastes will create a good basis to reach the target. The research programme for the detailed site characterization has mainly been focused on groundwater flow and geochemistry due to their importance in terms of long-term safety of the repository. Equipment and methodology development by Posiva has introduced new tools that provide more accurate data on relevant parameters than the ones used in previous stages of site characterization. The programme also contains studies for additional information of the structural and geological properties of the bedrock towards the depth. Also predictive modelling has been made for evaluating the relevance of the assumptions made. The methods applied in the site characterization have comprised, e.g., geological mapping, deep core drilling, groundwater sampling and analyzing, hydraulic testing and geophysical measurements 23 refs, 4 figs

Discovering How Students Search a Library Web Site: A Usability Case Study.

Science.gov (United States)

Augustine, Susan; Greene, Courtney

2002-01-01

Discusses results of a usability study at the University of Illinois Chicago that investigated whether Internet search engines have influenced the way students search library Web sites. Results show students use the Web site's internal search engine rather than navigating through the pages; have difficulty interpreting library terminology; and…

Developing methodology for description of biosphere evolution at Olkiluoto disposal site utilising forest studies at other land uplift sites

International Nuclear Information System (INIS)

Ikondn, A.T.K.; Afo, L.

2004-01-01

In Finland, Olkiluoto Island has been selected as the site for final disposal of spent nuclear fuel, in addition to the existing repository for low and intermediate level waste. When creating biosphere models for safety assessments, local main features and processes need to be taken into account. A special characteristic of the site, as well as the coastal area of the Gulf of Bothnia in general, is the land uplift (6-9 mm/a). This continuously exposes new land to soil-formation processes and provides surfaces for colonization by plant communities. The forest vegetation succession on stony, fine-grained till soils starts from deciduous shoreline vegetation and ends in almost pure Norway spruce forests. This has enabled to study ecological and microbiological processes in soils and forests of different developmental stages, to monitor forest condition and the factors affecting it in sites locating close to each other. It has also made possible gradient studies of the succession of boreal mire ecosystems without a need to wait thousands of years. Applying a methodology described in the full paper, a descriptive model on the evolution of the biosphere will be established to indicate possible ecosystem distributions and main characteristics on the area on the basis of above-mentioned studies carried out by Finnish Forest Research Institute, and of results of the site investigations at Olkiluoto. In future, the evolution description will be used as a basis for selection of appropriate ecosystem modules and parameter values in the subsequent coupled assessment model systems. (author)

Thermally stimulated conductivity and thermoluminescence from Al2O3 : C

DEFF Research Database (Denmark)

Agersnap Larsen, N.; Bøtter-Jensen, L.; McKeever, S.W.S.

1999-01-01

Simultaneous measurements of thermoluminescence (TL) and thermally stimulated conductivity (TSC) are reported on single-crystal dosimetry-quality Al2O3:C following gamma irradiation at room temperature. Analysis of the data reveals a superposition of several first-order TL and TSC peaks caused...

Positron annihilation and thermally stimulated current of electron beam irradiated polyetheretherketone

Energy Technology Data Exchange (ETDEWEB)

Fujita, Shigetaka; Shinyama, Katsuyoshi; Baba, Makoto [Hachinohe Inst. of Tech., Hachinohe, Aomori (Japan); Suzuki, Takenori

1997-03-01

Positron lifetime measurements were applied to electron beam irradiated poly(ether-ether-ketone). The lifetime, {tau}{sub 3}, of the ortho-positronium of unirradiated and 5 MGy irradiated specimen became rapidly longer above about 150degC. {tau}{sub 3} of 50 MGy and 100 MGy irradiated specimen was shorter than that of unirradiated one. Thermally stimulated current (TSC) decreased with increasing the dose before voltage application. In the case of voltage application, a TSC peak appeared and the peak value decreased with increased the dose. The correlation between the results of positron annihilation and TSC was investigated. (author)

Modelling of Ohmic discharges in ADITYA tokamak using the Tokamak Simulation Code

International Nuclear Information System (INIS)

Bandyopadhyay, I; Ahmed, S M; Atrey, P K; Bhatt, S B; Bhattacharya, R; Chaudhury, M B; Deshpande, S P; Gupta, C N; Jha, R; Joisa, Y Shankar; Kumar, Vinay; Manchanda, R; Raju, D; Rao, C V S; Vasu, P

2004-01-01

Several Ohmic discharges of the ADITYA tokamak are simulated using the Tokamak Simulation Code (TSC), similar to that done earlier for the TFTR tokamak. Unlike TFTR, the dominant radiation process in ADITYA is through impurity line radiation. TSC can follow the experimental plasma current and position to very good accuracy. The thermal transport model of TSC including impurity line radiation gives a good match of the simulated results with experimental data for the Ohmic flux consumption, electron temperature and Z eff . Even the simulated magnetic probe signals are in reasonably good agreement with the experimental values

Modelling of Ohmic discharges in ADITYA tokamak using the Tokamak Simulation Code

Energy Technology Data Exchange (ETDEWEB)

Bandyopadhyay, I; Ahmed, S M; Atrey, P K; Bhatt, S B; Bhattacharya, R; Chaudhury, M B; Deshpande, S P; Gupta, C N; Jha, R; Joisa, Y Shankar; Kumar, Vinay; Manchanda, R; Raju, D; Rao, C V S; Vasu, P [Institute for Plasma Research, Bhat, Gandhinagar 382428 (India)

2004-09-01

Several Ohmic discharges of the ADITYA tokamak are simulated using the Tokamak Simulation Code (TSC), similar to that done earlier for the TFTR tokamak. Unlike TFTR, the dominant radiation process in ADITYA is through impurity line radiation. TSC can follow the experimental plasma current and position to very good accuracy. The thermal transport model of TSC including impurity line radiation gives a good match of the simulated results with experimental data for the Ohmic flux consumption, electron temperature and Z{sub eff}. Even the simulated magnetic probe signals are in reasonably good agreement with the experimental values.

Second Information Technology in Education Study: SITES 2006 Technical Report

Science.gov (United States)

Carstens, Ralph, Ed.; Pelgrum, Willem J., Ed.

2009-01-01

The International Association for the Evaluation of Educational Achievement (IEA) has been conducting comparative studies for 50 years. SITES 2006 is the fifth wave of surveys related to information and communication technology (ICT), a wave that IEA started with its Computers in Education Study (two studies with data collection in 1989 and 1992),…

A Study Plan for Determining Recharge Rates at the Hanford Site Using Environmental Tracers

Energy Technology Data Exchange (ETDEWEB)

Murphy, E. M.; Szecsody, J. E.; Phillips, S. J.

1991-02-01

This report presents a study plan tor estimating recharge at the Hanford Site using environmental tracers. Past operations at the Hanford Site have led to both soil and groundwater contamination, and recharge is one of the primary mechanisms for transporting contaminants through the vadose zone and into the groundwater. The prediction of contaminant movement or transport is one aspect of performance assessment and an important step in the remedial investigation/feasibility study (RI/FS) process. In the past, recharge has been characterized by collecting lysimeter data. Although lysimeters can generate important and reliable data, their limitations include 1) fixed location, 2) fixed sediment contents, 3) edge effects, 4) low rates, and 5) relatively short duration of measurement. These limitations impact the ability to characterize the spatial distribution of recharge at the Hanford Site, and thus the ability to predict contaminant movement in the vadose zone. An alternative to using fixed lysimeters for determining recharge rates in the vadose zone is to use environmental tracers. Tracers that have been used to study water movement in the vadose zone include total chloride, {sup 36}CI, {sup 3}H, and {sup 2}H/{sup 18}O. Atmospheric levels of {sup 36}CI and {sup 3}H increased during nuclear bomb testing in the Pacific, and the resulting "bomb pulse" or peak concentration can be measured in the soil profile. Locally, past operations at the Hanford Site have resu~ed in the atmospheric release of numerous chemical and isotopic tracers, including nitrate, {sup 129}I, and {sup 99}Tc. The radionuclides, in particular, reached a well-defined atmospheric peak in 1945. Atmospheric releases of {sup 129}I and {sup 99}Tc were greatly reduced by mid-1946, but nitrogen oxides continued to be released from the uranium separations facilities. As a result, the nitrate concentrations probably peaked in the mid-1950s, when the greatest number of separations facilities were operating

Establishing a reference rock site for the site effect study in and around the Kathmandu valley, Nepal

Science.gov (United States)

Bhattarai, Mukunda; Adhikari, Lok Bijaya; Gautam, Umesh Prasad; Bollinger, Laurent; Hernandez, Bruno; Yokoi, Toshiaki; Hayashida, Takumi

2016-05-01

We propose a reference site for the site effect study in and around the Kathmandu valley, Nepal. The used data were the accelerograms recorded at two stations, DMG and KKA, and velocity seismograms co-recorded at the PKIN station during nine shallow local and regional earthquakes of local magnitude equal to or greater than 5.0. The DMG station is located on the thick sediments of the Kathmandu valley, whereas the others are rock sites. The KKA station is located on the granite and gneisses of the Shivapuri Lekh about 10 km northwest of the capital, and the PKIN station is in the tunnel of an old iron mine on the southern slope of the Phulchauki Hill about 15 km southeast. The spectral ratios of the ground motion records of the DMG station compared to those of the PKIN station, for all considered earthquakes, confirm that the DMG station has amplification ranging from 1 to 10 in the frequency range of 0.5-10 Hz, and spectral ratios of the KKA station referenced by the PKIN station show that the KKA station has significant amplification in the frequency range of 4-10 Hz and the peak value of the spectral ratio is at most over 25. Therefore, the site amplification in and around Kathmandu valley would be significantly underestimated in the frequency range from 4 to 10 Hz if the records of the KKA station were used as a proxy for input seismic motions to the sediment. Based on the above analysis, we propose that the PKIN station should be considered as a reliable reference site for the assessment of seismic hazards in and around the Kathmandu valley.

The Influence of Heritage Sites as Filming Locations on Tourists’ Decisions to Visit Sites and Their Perceptions of Them. Case Study: Game of Thrones

Directory of Open Access Journals (Sweden)

Emily Bowyer

2017-10-01

Full Text Available This paper provides an overview of the authors’ master thesis and addresses the effects of World Heritage Sites and heritage sites which are used as filming locations on visitor perceptions of a site and their decision to visit a site. Film-induced tourism is becoming increasingly popular and it is important to assess its impacts on World Heritage Sites and heritage sites used as locations. The integration of the different aspects of heritage and filming at a site including elements and the communication between all the different parties involved are also addressed. The case study used is the popular television series Game of Thrones focusing on various locations in Northern Ireland and Dubrovnik, Croatia. The paper aims to provide a starting platform for future research on heritage sites used as filming locations and the possible impacts that this may have.

Site Study Plan for soils, Deaf Smith County site, Texas: Environmental Field Program: Preliminary draft

International Nuclear Information System (INIS)

1987-06-01

The Soils Site Study Plan describes a field program consisting of a soil characterization survey, impact monitoring of soils, predisturbance soil salinity survey, and a reclamation suitability study. This information will be used to plan for soil stripping, stockpiling, and replacement; reclamation of soils; determining predisturbance chemical and physical characteristics of the soils; including salinity levels; and monitoring for changes in chemical and physical characteristics of the soil. The SSP describes for each study the need for the study, the study design, data management and use, schedule of proposed activities, and the quality assurance program. These studies will provide data needed to satisfy requirements contained in, or derived from, the Salt Repository Project Requirements Document. 75 refs., 10 figs., 5 tabs

Siting of nuclear desalination plants in Saudi Arabia: A seismic study

International Nuclear Information System (INIS)

Aljohani, M.S.; Abdul-Fattah, A.F.; Almarshad, A.I.

2005-01-01

This paper presents the selection criteria generally and seismic criteria specifically to select a suitable site in Saudi Arabia for a nuclear desalination plant. These criteria include geological, meteorological, cooling water supply discharge, transport infrastructure, population, electric grid, water network capacity, environmental impact and airport movement. The seismicity of the Arabian peninsula for the locations of seismic activity along the Red Sea and the Arabian Gulf coastlines from 1973 to 2000 was studied carefully. This study included towns and locations along the east and west coastlines and their distances from the seismic event site. The results showed that Rabigh City along the west coast of Saudi Arabia is a good site to build a nuclear desalination plant. This is because of the following reasons: good seismic stability; good weather statistics; no flooding; mild wave conditions; good supply and discharge; good transportation infrastructure; low population area; very close to the huge electric grid. (author)

Study on site selection of cold chain logistics in northwest territories

Science.gov (United States)

Liu, Yubin; Ren, Zongwei

2017-08-01

In this research, we mainly studied the Site selection problem of cold chain logistics in northwest of China. In the first place, we counted the demands of cold chain products in northwest territories, and then classified it into the Site selection problem in five provinces in northwest territories(Xinjiang, Qinghai, Gansu, Ningxia, Shanxi); Next, we used the Center of gravity Method to select initial location; Finally, we established the location of distribution by using Analytic Hierarchy Process (AHP)and fuzzy comprehensive evaluation method. Comparing with the traditional method, this method not only considered the cost of transportation and distance, but also deliberated the physical condition, social environment and economics condition which associated with Site selection problem.

Feasibility Study of Economics and Performance of Solar Photovoltaics at the Price Landfill Site in Pleasantville, New Jersey. A Study Prepared in Partnership with the Environmental Protection Agency for the RE-Powering America's Land Initiative: Siting Renewable Energy on Potentially Contaminated Land and Mine Sites

Energy Technology Data Exchange (ETDEWEB)

Salasovich, J.; Geiger, J.; Mosey, G.; Healey, V.

2013-05-01

The U.S. Environmental Protection Agency (EPA), in accordance with the RE-Powering America's Land initiative, selected the Price Landfill site in Pleasantville, New Jersey, for a feasibility study of renewable energy production. The National Renewable Energy Laboratory (NREL) provided technical assistance for this project. The purpose of this report is to assess the site for a possible photovoltaic (PV) system installation and estimate the cost, performance, and site impacts of different PV options. In addition, the report recommends financing options that could assist in the implementation of a PV system at the site. This study did not assess environmental conditions at the site.

Feasibility Study of Economics and Performance of Solar Photovoltaics at the Tower Road Site in Aurora, Colorado. A Study Prepared in Partnership with the Environmental Protection Agency for the RE-Powering America's Land Initiative: Siting Renewable Energy on Potentially Contaminated Land and Mine Sites

Energy Technology Data Exchange (ETDEWEB)

Van Geet, O.; Mosey, G.

2013-03-01

The U.S. Environmental Protection Agency (EPA), in accordance with the RE-Powering America's Land initiative, selected the Tower Road site in Aurora, Colorado, for a feasibility study of renewable energy production. The National Renewable Energy Laboratory (NREL) provided technical assistance for this project. The purpose of this report is to assess the site for a possible photovoltaic (PV) system installation and estimate the cost, performance, and site impacts of different PV options. In addition, the report recommends financing options that could assist in the implementation of a PV system at the site. This study did not assess environmental conditions at the site.

Environmental radioactivity survey in site studies

International Nuclear Information System (INIS)

Segovia, N.; Gaso, M.I.; Tejera, A.; Tamez, E.

1989-01-01

Systematic monitoring of Rn in soil, background gamma levels and meteorological measurements was conducted at the site of a nuclear research center. The data cover a time period of several years. The radon in soil gas fluctuations is influenced by seasonal meteorological changes and by local geophysical parameters and they range between 1000 Bq/m 3 and 18000 Bq/m 3 . The observed gamma levels show geographic stability; some seasonal fluctuations were observed. Data analysis indicates that no significant changes occured at the site over the measuring period and radiation levels and meteorological changes are moderate inducing no ecological impact on the surroundings. (author) 8 refs.; 5 figs.; 2 tabs

Thermally stimulated current in PTFE and its application in radiation dosimetry

International Nuclear Information System (INIS)

Ozdemir, S.

1985-01-01

Thermally Stimulated Current (TSC) measurement was made on PTFE (Polytetrafluoro ethylene) in an attempt to develop an integrating radiation dosimeter material and the system. TSC spectra, dose response, energy response, fading and background charge stability characteristics were used as a measure of suitability of various untreated and heat treated PTFE samples for dosimetry applications. For practical TSC dosimetry system, it was discovered that the PTFE samples should be subjected to a specific heat treatment in order to produce samples with better dosimeter characteristics. A treatment at a temperature of 240 C produces a high dose response and low fading characteristics. It was found that the spurious charges due to storage and low sensitivity to irradiation caused the limitation in the measurement of low doses with PTFE samples for personnel protection. However, a TSC Dosimetry system using PTFE is proposed which is suitable for radiation doses in the radiotherapy range from *approx* 50 to *approx* 800 mGy. (author)

Classification of time-series images using deep convolutional neural networks

Science.gov (United States)

Hatami, Nima; Gavet, Yann; Debayle, Johan

2018-04-01

Convolutional Neural Networks (CNN) has achieved a great success in image recognition task by automatically learning a hierarchical feature representation from raw data. While the majority of Time-Series Classification (TSC) literature is focused on 1D signals, this paper uses Recurrence Plots (RP) to transform time-series into 2D texture images and then take advantage of the deep CNN classifier. Image representation of time-series introduces different feature types that are not available for 1D signals, and therefore TSC can be treated as texture image recognition task. CNN model also allows learning different levels of representations together with a classifier, jointly and automatically. Therefore, using RP and CNN in a unified framework is expected to boost the recognition rate of TSC. Experimental results on the UCR time-series classification archive demonstrate competitive accuracy of the proposed approach, compared not only to the existing deep architectures, but also to the state-of-the art TSC algorithms.

Application of thermally stimulated current measurement to the polymorphic characterization of drug substances

International Nuclear Information System (INIS)

Ikeda, Y.; Hirayama, T.; Terada, K.

2005-01-01

The thermal stimulated current measurement was used as an innovative analytical equipment to evaluate the polymorphic properties of terfenadine and Compound A, being developed by Takeda Pharmaceutical Company, Limited. At first, terfenadine, which is known to have polymorphs, was used as a model sample for thermally stimulated current (TSC) analyses. The TSC curves of amorphous and two polymorphs were distinctly different from each other. Therefore, it was considered that TSC measurement could be a useful technique to evaluate the crystalline properties of drug substances. The polymorphs of compound A were difficult to distinguish the characteristics of polymorphs from conventional powder X-ray diffractometry and also differential scanning calorimetry. Forms A and B of compound A were clearly differentiated by the thermal stimulated current properties that were adequate to characterize each form. Thus, it was shown that TSC was extremely useful and powerful tool for identification of complicated polymorphs, which were not distinguished by conventional methods

Site attraction as a representation of heritage tourism: Case study of Gunung Padang Site, Cianjur, West - Java

Science.gov (United States)

Subakti, A. G.; Pratomo, Aditya; Triana, Ika; Khrisnanto, Iwan

2018-03-01

Heritage Tourism is used for experiencing the heritage of city, region, state or country. This travel enables the tourist to learn about local customs, traditions, history and culture. One of the tourist attractions in Cianjur, where an ancient historical megalithic site named Situs Gunung Padang could be found. Situs Gunung Padang which consists of five terraces and five levels, completely built stones. This site is the largest megalithic site in Southeast Asia. There is an enormous tourism potential owned by this site because it expresses and preserving ancient Sundanese cultural in the megalithic era. But the potential of this site seems haven’t made as a tourist attraction compared with Borobudur or Prambanan Temple. The purpose of this research is to analyze the existence of Gunung Padang site as a heritage tourism with SWOT approach to discover what factors need to be repaired. The result of this research is showing that Gunung Padang site has a huge potential if the regional governments and the locals can contribute to preserve and making this site as a tourist attraction.

Identification and reproducibility of diagnostic DNA markers for tuber starch and yield optimization in a novel association mapping population of potato (Solanum tuberosum L.).

Science.gov (United States)

Schönhals, E M; Ortega, F; Barandalla, L; Aragones, A; Ruiz de Galarreta, J I; Liao, J-C; Sanetomo, R; Walkemeier, B; Tacke, E; Ritter, E; Gebhardt, C

2016-04-01

SNPs in candidate genes Pain - 1, InvCD141 (invertases), SSIV (starch synthase), StCDF1 (transcription factor), LapN (leucine aminopeptidase), and cytoplasm type are associated with potato tuber yield, starch content and/or starch yield. Tuber yield (TY), starch content (TSC), and starch yield (TSY) are complex characters of high importance for the potato crop in general and for industrial starch production in particular. DNA markers associated with superior alleles of genes that control the natural variation of TY, TSC, and TSY could increase precision and speed of breeding new cultivars optimized for potato starch production. Diagnostic DNA markers are identified by association mapping in populations of tetraploid potato varieties and advanced breeding clones. A novel association mapping population of 282 genotypes including varieties, breeding clones and Andean landraces was assembled and field evaluated in Northern Spain for TY, TSC, TSY, tuber number (TN) and tuber weight (TW). The landraces had lower mean values of TY, TW, TN, and TSY. The population was genotyped for 183 microsatellite alleles, 221 single nucleotide polymorphisms (SNPs) in fourteen candidate genes and eight known diagnostic markers for TSC and TSY. Association test statistics including kinship and population structure reproduced five known marker-trait associations of candidate genes and discovered new ones, particularly for tuber yield and starch yield. The inclusion of landraces increased the number of detected marker-trait associations. Integration of the present association mapping results with previous QTL linkage mapping studies for TY, TSC, TSY, TW, TN, and tuberization revealed some hot spots of QTL for these traits in the potato genome. The genomic positions of markers linked or associated with QTL for complex tuber traits suggest high multiplicity and genome wide distribution of the underlying genes.

Management of the Cs/Sr Capsule Project at the Hanford Site. Technology Readiness Assessment Report

Energy Technology Data Exchange (ETDEWEB)

None, None

2018-01-01

The Federal Project Director (FPD) for the U.S. Department of Energy (DOE), Richland Operations Office (RL) Waste Management and D&D Division (WMD) requested a Technology Readiness Assessment (TRA) for the Management of the Cesium/Strontium Capsule Storage Project (MCSCP) at the Waste Encapsulation and Storage Facility (WESF) on the Hanford Site in Washington State. The MCSCP CD-1 TRA was performed by a team selected in collaboration between the Office of Environmental Management (EM) Chief Engineer (EM-3.3) and RL, WMD FPD. The TRA Team included subject matter and technical experts having experience in cask storage, process engineering, and system design who were independent of the MCSCP, and the team was led by the Director of Operations and Processes from the EM Chief Engineer's Office (EM-3.32). Movement of the Cs/Sr capsules to dry storage, based on information from the conceptual design, involves (1) capsule packaging, (2) capsule transfer, and (3) capsule storage. The project has developed a conceptual process, described in 30059-R-02, "NAC Conceptual Design Report for the Management of the Cesium and Strontium Capsules Project", which identifies the five major activities in the process to complete the transfer from storage pool to pad-mounted cask storage. The process, shown schematically in Figure 1, is comprised of the following process steps: (1) loading capsules into the UCS; (2) UCS processing; (3) UCS insertion into the TSC Basket; (4) cask transport from WESF to CSA and (5) extended storage at the CSA.

The Violence Prevention Community Meeting: A Multi-Site Study.

Science.gov (United States)

Lanza, Marilyn; Ridenour, Marilyn; Hendricks, Scott; Rierdan, Jill; Zeiss, Robert; Schmidt, Satu; Lovelace, Jeff; Amandus, Harlan

2016-06-01

The Violence Prevention Community Meeting (VPCM) is a specialized form of community meeting in which avoiding violence and promoting non-violent problem solving and interpersonal civility are focal points. A nationwide study to assess the VPCM as an effective intervention to reduce workplace violence was undertaken. Seven acute locked psychiatric units of the Veterans Health Administration (VHA) throughout the United States participated in the study. All patients and all staff on the seven in-patient locked psychiatry units participated in the intervention (VPCM) or as a control (treatment as usual). The study was 21weeks at each site. The three time periods were pre-treatment weeks 1-3, treatment weeks 4-18, and post-treatment weeks 19-21. The VPCM was conducted during the treatment weeks. Overall rates of aggression declined by 0.6% (95% CI: -5.6%, 6.5%; nonsignificant) per week in the intervention hospitals and by 5.1% (95% CI: 0.4%, 9.6%; significant) per week for the control hospitals. Aggression decreased for both the intervention and control hospitals which could be due to enrollment in a research study and thus being more aware of their ability to address workplace violence at their site. Published by Elsevier Inc.

Ground Characterization Studies in Canakkale Pilot Site of LIQUEFACT Project

Science.gov (United States)

Ozcep, F.; Oztoprak, S.; Aysal, N.; Bozbey, I.; Tezel, O.; Ozer, C.; Sargin, S.; Bekin, E.; Almasraf, M.; Cengiz Cinku, M.; Ozdemir, K.

2017-12-01

The our aim is to outline the ground characterisation studies in Canakkale test site. Study is based on the EU H2020 LIQUEFACT project entitled "Liquefact: Assessment and mitigation of liquefaction potential across Europe: a holistic approach to protect structures / infrastructures for improved resilience to earthquake-induced liquefaction disasters". Objectives and extent of ground characterization for Canakkale test site includes pre-existing soil investigation studies and complementary field studies. There were several SPT and geophysical tests carried out in the study area. Within the context of the complementary tests, six (6) study areas in the test site were chosen and complementary tests were carried out in these areas. In these areas, additional boreholes were opened and SPT tests were performed. It was decided that additional CPT (CPTU and SCPT) and Marchetti Dilatometer (DMT) tests should be carried out within the scope of the complementary testing. Seismic refraction, MASW and micro tremor measurements had been carried out in pre-existing studies. Shear wave velocities obtained from MASW measurements were evaluated to the most rigorous level. These tests were downhole seismic, PS-logging, seismic refraction, 2D-ReMi, MASW, micro tremor (H/V Nakamura method), 2D resistivity and resonance acoustic profiling (RAP). RAP is a new technique which will be explained briefly in the relevant section. Dynamic soil properties had not been measured in pre-existing studies, therefore these properties were investigated within the scope of the complementary tests. Selection of specific experimental tests of the complementary campaign was based on cost-benefit considerations Within the context of complementary field studies, dynamic soil properties were measured using resonant column and cyclic direct shear tests. Several sieve analyses and Atterberg Limits tests which were documented in the pre-existing studies were evaluated. In the complementary study carried out

Summary of some feasibility studies for site-specific solar industrial process heat

Energy Technology Data Exchange (ETDEWEB)

None

1982-01-01

Some feasibility studies for several different site specific solar industrial process heat applications are summarized. The followng applications are examined. Leather Tanning; Concrete Production: Lumber and Paper Processing; Milk Processing; Molding, Curing or Drying; Automobile Manufacture; and Food Processing and Preparation. For each application, site and process data, system design, and performance and cost estimates are summarized.

The part played by applied geology in nuclear power plant site studies

International Nuclear Information System (INIS)

Giafferi, J.L.

1994-01-01

Site-related geological problems are one of the constraints affecting the environment of nuclear power plants. The natural features (soil and subsoil) at the nuclear power plant site affect numerous factors in the design, construction and operation of the civil engineering structures. The site geological criteria are not solely restricted to the soil as a static support for the structures. Earth tremors in France are of moderate intensity but the likelihood of their occurrence must nevertheless be taken into account for each site. Studies must concern the geological and seismic features of the region as well as the soil and subsoil configurations and composition in the immediate vicinity of the site in order to determine the physical characteristics of the earthquakes so that the safety of the plant can be guaranteed; in many cases, water tables have also to be taken into consideration. Geologic survey techniques are discussed. 13 figs., 7 refs

Correlated analysis of 2 MeV proton-induced radiation damage in CdZnTe crystals using photoluminescence and thermally stimulated current techniques

Energy Technology Data Exchange (ETDEWEB)

Gu, Yaxu [State Key Laboratory of Solidification Processing, School of Materials Science and Engineering, Northwestern Polytechnical University, Xi’an 710072 (China); Key Laboratory of Radiation Detection Materials and Devices of Ministry of Industry and Information Technology, Northwestern Polytechnical University, Xi’an 710072 (China); Jie, Wanqi, E-mail: jwq@nwpu.edu.cn [State Key Laboratory of Solidification Processing, School of Materials Science and Engineering, Northwestern Polytechnical University, Xi’an 710072 (China); Key Laboratory of Radiation Detection Materials and Devices of Ministry of Industry and Information Technology, Northwestern Polytechnical University, Xi’an 710072 (China); Rong, Caicai [Institute of Modern Physics, Applied Ion Beam Physics Laboratory, Fudan University, Shanghai 200433 (China); Wang, Yuhan; Xu, Lingyan; Xu, Yadong [State Key Laboratory of Solidification Processing, School of Materials Science and Engineering, Northwestern Polytechnical University, Xi’an 710072 (China); Key Laboratory of Radiation Detection Materials and Devices of Ministry of Industry and Information Technology, Northwestern Polytechnical University, Xi’an 710072 (China); Lv, Haoyan; Shen, Hao [Institute of Modern Physics, Applied Ion Beam Physics Laboratory, Fudan University, Shanghai 200433 (China); Du, Guanghua [Materials Research Center, Institute of Modern Physics, Chinese Academy of Sciences, Lanzhou 730000 (China); Fu, Xu [State Key Laboratory of Solidification Processing, School of Materials Science and Engineering, Northwestern Polytechnical University, Xi’an 710072 (China); Key Laboratory of Radiation Detection Materials and Devices of Ministry of Industry and Information Technology, Northwestern Polytechnical University, Xi’an 710072 (China); and others

2016-11-01

Highlights: • 2 MeV proton-induced radiation damage in CdZnTe crystals is investigated by PL and TSC techniques. • The influence of radiation damage on the luminescent and electrical properties of CdZnTe crystals is studied. • Intensity of PL spectrum is found to decrease significantly in irradiated regions, suggesting the increase of non-radiative recombination centers. • A correlated analysis of PL and TSC spectra suggests that the density of dislocations and A-centers increase after proton irradiation. - Abstract: Radiation damage induced by 2 MeV protons in CdZnTe crystals has been studied by means of photoluminescence (PL) and thermally stimulated current (TSC) techniques. A notable quenching of PL intensity is observed in the regions irradiated with a fluence of 6 × 10{sup 13} p/cm{sup 2}, suggesting the increase of non-radiative recombination centers. Moreover, the intensity of emission peak D{sub complex} centered at 1.48 eV dominates in the PL spectrum obtained from irradiated regions, ascribed to the increase of interstitial dislocation loops and A centers. The intensity of TSC spectra in irradiated regions decreases compared to the virgin regions, resulting from the charge collection inefficiency caused by proton-induced recombination centers. By comparing the intensity of identified traps obtained from numerical fitting using simultaneous multiple peak analysis (SIMPA) method, it suggests that proton irradiation under such dose can introduce high density of dislocation and A-centers in CdZnTe crystals, consistent with PL results.

Modeling study on geological environment at Horonobe URL site

International Nuclear Information System (INIS)

Shimo, Michito; Yamamoto, Hajime; Kumamoto, Sou; Fujiwara, Yasushi; Ono, Makoto

2005-02-01

The Horonobe underground research project has been operated by Japan Nuclear Cycle Development Institute to study the geological environment of sedimentary rocks in deep underground. The objectives of this study are to develop a geological environment model, which incorporate the current findings and the data obtained through the geological, geophysical, and borehole investigations at Horonobe site, and to predict the hydrological and geochemical impacts caused by the URL shaft excavation to the surrounding area. A three-dimensional geological structure model was constructed, integrating a large-scale model (25km x 15km) and a high-resolution site-scale model (4km x 4km) that have been developed by JNC. The constructed model includes surface topography, geologic formations (such as Yuchi, Koetoi, Wakkanai, and Masuporo Formations), and two major faults (Ohomagari fault and N1 fault). In hydrogeological modeling, water-conductive fractures identified in Wakkanai Formation are modeled stochastically using EHCM (Equivalent Heterogeneous Continuum Model) approach, to represent hydraulic heterogeneity and anisotropy in the fractured rock mass. Numerical code EQUIV FLO (Shimo et al., 1996), which is a 3D unsaturated-saturated groundwater simulator capable of EHCM, was used to simulate the regional groundwater flow. We used the same model and the code to predict the transient hydrological changes caused by the shaft excavations. Geochemical data in the Horonobe site such as water chemistries, mineral compositions of rocks were collected and summarized into digital datasets. M3 (Multivariate, Mixing and Mass-balance) method developed by SKB (Laaksoharju et al., 1999) was used to identify waters of different origins, and to infer the mixing ratio of these end-members to reproduce each sample's chemistry. Thermodynamic code such as RHREEQC, GWB, and EQ3/6 were used to model chemical reactions that explain the present minerals and aqueous concentrations observed in the site

Background complementary hydrogeochemical studies. SDM-Site Forsmark

Energy Technology Data Exchange (ETDEWEB)

Kalinowski, Birgitta E. (ed.)

2008-08-15

The overall objectives of the hydrogeochemical description for Forsmark are to establish a detailed understanding of the hydrogeochemical conditions at the site and to develop models that fulfil the needs identified by the safety assessment groups during the site investigation phase. Issues of concern to safety assessment are radionuclide transport and technical barrier behaviour, both of which are dependent on the chemistry of groundwater and pore water and their evolution with time. The work has involved the development of descriptive and mathematical models for groundwaters in relation to rock domains, fracture domains and deformation zones. Past climate changes are one of the major driving forces for hydrogeochemical changes and therefore of fundamental importance for understanding the palaeohydrogeological, palaeohydrogeochemical and present evolution of groundwater in the crystalline bedrock of the Fennoscandian Shield. Understanding current undisturbed hydrochemical conditions at the proposed repository site is important when predicting future changes in groundwater chemistry. The causes of copper corrosion and/or bentonite degradation are of particular interest as they may jeopardise the long-term integrity of the planned SKB repository system. Thus, the following variables are considered for the hydrogeochemical site descriptive modelling: pH, Eh, sulphur species, iron, manganese, uranium, carbonate, phosphate, nitrogen species, total dissolved solids (TDS), isotopes, colloids, fulvic and humic acids and microorganisms. In addition, dissolved gases (e.g. carbon dioxide, methane and hydrogen) are of interest because of their likely participation in microbial reactions. In this series of reports, the final hydrogeochemical evaluation work of the site investigation at the Forsmark site, is presented. The work was conducted by SKB's hydrogeochemical project group, ChemNet, which consists of independent consultants and university researchers with expertise

Giving Back: Collaborations with Others in Ecological Studies on the Nevada National Security Site

Energy Technology Data Exchange (ETDEWEB)

Scott A. Wade (NFO); Kathryn S. Knapp (NFO); Cathy A. Wills (NSTec)

2013-02-24

Formerly named the Nevada Test Site, the Nevada National Security Site (NNSS) was the historical site for nuclear weapons testing from the 1950s to the early 1990s. The site was renamed in 2010 to reflect the diversity of nuclear, energy, and homeland security activities now conducted at the site. Biological and ecological programs and research have been conducted on the site for decades to address the impacts of radiation and to take advantage of the relatively undisturbed and isolated lands for gathering basic information on the occurrence and distribution of native plants and animals. Currently, the Office of the Assistant Manager for Environmental Management of the U.S. Department of Energy (DOE), National Nuclear Security Administration Nevada Site Office (NNSA/NSO) oversees the radiological biota monitoring and ecological compliance programs on the NNSS. The top priority of these programs are compliance with federal and state regulations. They focus on performing radiological dose assessments for the public who reside near the NNSS and for populations of plants and animals on the NNSS and in protecting important species and habitat from direct impacts of mission activities. The NNSS serves as an invaluable outdoor laboratory. The geographic and ecological diversity of the site offers researchers many opportunities to study human influences on ecosystems. NNSA/NSO has pursued collaborations with outside agencies and organizations to be able to conduct programs and studies that enhance radiological biota monitoring and ecosystem preservation when budgets are restrictive, as well as to provide valuable scientific information to the human health and natural resource communities at large. NNSA/NSO is using one current collaborative study to better assess the potential dose to the off-site public from the ingestion of game animals, the most realistic pathway for off-site public exposure at this time from radionuclide contamination on the NNSS. A second

Fetal and Postnatal Magnetic Resonance Imaging of Unilateral Cystic Renal Dysplasia in a Neonate with Tuberous Sclerosis

Directory of Open Access Journals (Sweden)

Vineet Tyagi

2016-10-01

Full Text Available Tuberous sclerosis (TS is an autosomal dominant condition associated with mutations in the TSC1 and/or TSC2 genes. Clinical manifestations are multisystemic, and they often include lesions in the brain, skin, heart, kidneys, and bones. TSC2 gene mutations can be seen concomitantly with autosomal dominant polycystic kidney disease gene mutations. We present a case of a fetus with prenatal diagnosis of TS that had unique asymmetrical distribution of renal cystic disease. We describe the extensive work up with both fetal and neonatal magnetic resonance imaging with correlating images of the unilateral polycystic renal disease in addition to typical TS brain findings.

β-Cyclodextrin hydrogels for the ocular release of antibacterial thiosemicarbazones.

Science.gov (United States)

Glisoni, Romina J; García-Fernández, María J; Pino, Marylú; Gutkind, Gabriel; Moglioni, Albertina G; Alvarez-Lorenzo, Carmen; Concheiro, Angel; Sosnik, Alejandro

2013-04-02

Two types of hydrophilic networks with conjugated beta-cyclodextrin (β-CD) were developed with the aim of engineering useful platforms for the localized release of an antimicrobial 5,6-dimethoxy-1-indanone N4-allyl thiosemicarbazone (TSC) in the eye and its potential application in ophthalmic diseases. Poly(2-hydroxyethyl methacrylate) soft contact lenses (SCLs) displaying β-CD, namely pHEMA-co-β-CD, and super-hydrophilic hydrogels (SHHs) of directly cross-linked hydroxypropyl-β-CD were synthesized and characterized regarding their structure (ATR/FT-IR), drug loading capacity, swelling and in vitro release in artificial lacrimal fluid. Incorporation of TSC to the networks was carried out both during polymerization (DP method) and after synthesis (PP method). The first method led to similar drug loads in all the hydrogels, with minor drug loss during the washing steps to remove unreacted monomers, while the second method evidenced the influence of structural parameters on the loading efficiency (proportion of CD units, mesh size, swelling degree). Both systems provided a controlled TSC release for at least two weeks, TSC concentrations (up to 4000μg/g dry hydrogel) being within an optimal therapeutic window for the antimicrobial ocular treatment. Microbiological tests against P. aeruginosa and S. aureus confirmed the ability of TSC-loaded pHEMA-co-β-CD network to inhibit bacterial growth. Copyright © 2012 Elsevier Ltd. All rights reserved.

Site-selection studies for final disposal of spent fuel in Finland

International Nuclear Information System (INIS)

Vuorela, P.; Aeikaes, T.

1984-02-01

In the management of waste by the Industrial Power Company Ltd. (TVO) preparations are being made for the final disposal of unprocessed spent fuel into the Finnish bedrock. The site selection program will advance in three phases. The final disposal site must be made at the latest by the end of the year 2000, in accordance with a decision laid down by the Finnish Government. In the first phase, 1983-85, the main object is to find homogeneous stable bedrock blocks surrounded by fracture zones located at a safe distance from the planned disposal area. The work usually starts with a regional structural analysis of mosaics of Landsat-1 winter and summer imagery. Next an assortment of different maps, which cover the whole country, is used. Technical methods for geological and hydrogeological site investigations are being developed during the very first phase of the studies, and a borehole 1000 meters deep will be made in southwestern Finland. Studies for the final disposal of spent fuel or high-level reprocessing waste have been made since 1974 in Finland. General suitability studies of the bedrock have been going on since 1977. The present results indicate that suitable investigation areas for the final disposal of highly active waste can be found in Finland

Site specific information in site selection

International Nuclear Information System (INIS)

Aeikaes, T.; Hautojaervi, A.

1998-01-01

The programme for the siting of a deep repository for final disposal of spent nuclear fuel was started already in 1983 and is carried out today by Posiva Oy which continues the work started by Teollisuuden Voima Oy. The programme aims at site selection by the end of the year 2000. The programme has progressed in successive interim stages with defined goals. After an early phase for site identification, five sites were selected in 1987 for preliminary site characterisation. Three of these were selected and judged to be best suited for the more detailed characterisation in 1992. An additional new site was included into the programme based on a separate feasibility study in the beginning of 1997. Since the year 1983 several safety assessments together with technical plans of the facility have been completed. When approaching the site selection the needs for more detailed consideration of the site specific properties in the safety assessment have been increased. The Finnish regulator STUK has published a proposal for general safety requirements for the final disposal of spent nuclear fuel in Finland. This set of requirements has been projected to be used in conjunction of the decision making by the end 2000. Based on the site evaluation all sites can provide a stable environment and there is evidence that the requirements for the longevity of the canister can be fulfilled at each site. In this manner the four candidate sites do not differ too much from each other. The main difference between the sites is in the salinity of the deep groundwater. The significance of differences in the salinity for the long-term safety cannot be defined yet. The differences may contribute to the discussion of the longevity of the bentonite buffer and also to the modelling of the groundwater flow and transport. The use of the geosphere as a transport barrier is basically culminated on the questions about sparse but fast flow routes and 'how bad channeling can be'. To answer these questions

Social Neworking Sites Usage of Students Studying in the S.P.E.S.

Directory of Open Access Journals (Sweden)

Levent Atali

2014-05-01

Full Text Available The purpose of the study is the determination of the intended use of social networking sites of students studying in the School of Physical Education and Sports (S.P.E.S. and demonstration of the current situation. In line with this purpose, answers were sought for the questions 'What are the intentions and aims of students studying in the School of Physical Education and Sports to use social networking sites?' and 'Do intended uses of students studying in the School of Physical Education and Sports differentiate in accordance with their demographic characteristics (gender, department, grade?' The study population consists of students of the Schools of Physical Education and Sports of Kocaeli University and Sakarya University who have been studying in these schools in 2013-2014 academic year. 672 students were chosen from the study population by use of convenience sampling method. "Social Networking Sites Intended Use Scale" is the general screening model because of the reason that the existing situation was determined as a result of analysis of the obtained data with quantitative method. Frequency measurement, Mann-Whitney U and Kruskal Wallis test techniques were used for the analysis of data obtained from students. Consequently, the findings of this study have shown that gender and grade do not create a significant difference in the intended use of social networking sites of students who are studying in the Schools of Physical Education and Sports and the intended use of students can be classified in the issues including communication, shar- ing, publicity, recognition, education and enlightenment.

Comparative study of the active sites in zeolites by different probe molecules

Directory of Open Access Journals (Sweden)

ALINE AUROUX

2005-03-01

Full Text Available This review summarizes some of the recently published results concerning the acid sites in the zeolites ZSM-5 and Y studied by temperature-programmed desorption (TPD and adsorption calorimetry using different probe molecules NH3, CO, N2O and n-hexane. For the first time it has been shown that the acid sites in hydrated zeolites are accessible for n-hexane adsorption

Federated Search and the Library Web Site: A Study of Association of Research Libraries Member Web Sites

Science.gov (United States)

Williams, Sarah C.

2010-01-01

The purpose of this study was to investigate how federated search engines are incorporated into the Web sites of libraries in the Association of Research Libraries. In 2009, information was gathered for each library in the Association of Research Libraries with a federated search engine. This included the name of the federated search service and…

Multi-site study of diffusion metric variability: effects of site, vendor, field strength, and echo time on regions-of-interest and histogram-bin analyses.

Science.gov (United States)

Helmer, K G; Chou, M-C; Preciado, R I; Gimi, B; Rollins, N K; Song, A; Turner, J; Mori, S

2016-02-27

It is now common for magnetic-resonance-imaging (MRI) based multi-site trials to include diffusion-weighted imaging (DWI) as part of the protocol. It is also common for these sites to possess MR scanners of different manufacturers, different software and hardware, and different software licenses. These differences mean that scanners may not be able to acquire data with the same number of gradient amplitude values and number of available gradient directions. Variability can also occur in achievable b-values and minimum echo times. The challenge of a multi-site study then, is to create a common protocol by understanding and then minimizing the effects of scanner variability and identifying reliable and accurate diffusion metrics. This study describes the effect of site, scanner vendor, field strength, and TE on two diffusion metrics: the first moment of the diffusion tensor field (mean diffusivity, MD), and the fractional anisotropy (FA) using two common analyses (region-of-interest and mean-bin value of whole brain histograms). The goal of the study was to identify sources of variability in diffusion-sensitized imaging and their influence on commonly reported metrics. The results demonstrate that the site, vendor, field strength, and echo time all contribute to variability in FA and MD, though to different extent. We conclude that characterization of the variability of DTI metrics due to site, vendor, field strength, and echo time is a worthwhile step in the construction of multi-center trials.

Nuclear installations sites safety

International Nuclear Information System (INIS)

Barber, P.; Candes, P.; Duclos, P.; Doumenc, A.; Faure, J.; Hugon, J.; Mohammadioun, B.

1988-11-01

This report is divided into ten parts bearing: 1 Safety analysis procedures for Basis Nuclear Installations sites (BNI) in France 2 Site safety for BNI in France 3 Industrial and transport activities risks for BNI in France 4 Demographic characteristics near BNI sites in France 5 Meteorologic characteristics of BNI sites in France 6 Geological aspects near the BNI sites in France 7 Seismic studies for BNI sites in France 8 Hydrogeological aspects near BNI sites in France 9 Hydrological aspects near BNI sites in France 10 Ecological and radioecological studies of BNI sites in France [fr

Summary of 1990 eolian characterization studies, Hanford Site, Washington

Energy Technology Data Exchange (ETDEWEB)

Gaylord, D.R.; Stetler, L.D.; Smith, G.D. [Washington State Univ., Pullman, WA (United States); Mars, R.W. [Wyoming Univ., Laramie, WY (United States)

1993-12-01

A study of eolian activity was initiated to improve understanding of past climate change and the likely effect of wind on engineered protective barriers at the Hanford Site. Eolian features from a Holocene sand dune field located in the southeastern portion of the Hanford Site were investigated using a variety of field and laboratory techniques including stratigraphic examinations of hand-dug pits, textural and compositional analyses of dune sand and potential source detritus, and air photo interpretations. These investigations were undertaken to evaluate the provenance and eolian dynamics of the sand dunes. Interpretations of sand dune migration using archival air photo stereopairs document a 20% reduction in the volume of active sand dunes (measured from an approximate 15-km{sup 2} test area) between 1948 and 1987. Changes in annual precipitation appear to have influenced active dune migration strongly.

Density functional study the interaction of oxygen molecule with defect sites of graphene

Energy Technology Data Exchange (ETDEWEB)

Qi Xuejun [State Key Laboratory of Coal Combustion, Wuhan 430074 (China); Guo Xin, E-mail: guoxin@mail.hust.edu.cn [State Key Laboratory of Coal Combustion, Wuhan 430074 (China); Zheng Chuguang [State Key Laboratory of Coal Combustion, Wuhan 430074 (China)

2012-10-15

Highlights: Black-Right-Pointing-Pointer The defect sites existed on the graphite surface create active sites and enhance the reactivity of carbonaceous material. Black-Right-Pointing-Pointer Oxygen molecule more favor chemisorbed on the graphene surface contains defect sites than the perfect surface. Black-Right-Pointing-Pointer The single active oxygen atom adsorbed on the defect surfaces, it completely insert into the surface. - Abstract: The present article reports a theoretical study of oxygen interacted with graphene surface containing defect sites on the atomic level by employing the density functional theory combined with the graphene cluster model. It was founded that oxygen molecule prefers to be chemisorbed on the graphene surface containing defect sites compared to the perfect surface. The adsorption energy of O{sub 2} on the double defect site is about 2.5 times as large as that on the perfect graphene surface. Moreover, the oxygen molecule interacts with S-W defect site gives rise to stable epoxy structure, which pulling the carbon atom outward from the original site in the direction perpendicular to the surface. If the oxygen molecule is adsorbed on the single vacancy site, two C-O bonds are formed on the graphene surface. However, when the oxygen molecule is chemisorbed on the double vacancy site, the oxygen atoms substitute the missing carbon atom's position in the carbon plane and form a hexagonal structure on the graphene network. The results indicate that single active oxygen atom approaches the defect site, it's completely adsorbed in the plane and high energy is released. In all cases, the interaction of an oxygen atom with defect surface involves an exothermic process. The defect site creates active sites on the surface of graphene and produces catalytic effects during the process of oxidation of carbonaceous materials.

A crystallographic and spectroscopic study of crystal sites in the 'spinel' structure; Etude cristallographique et spectroscopique des sites cristallins dans la structure 'spinelle'

Energy Technology Data Exchange (ETDEWEB)

Drifford, M. [Commissariat a l' Energie Atomique, Saclay (France). Centre d' Etudes Nucleaires

1967-06-15

A crystallographic study on magnesium aluminates led to the determination of crystal site average distortion. A spectroscopic study of transition elements doped spinels gave the position of the doping element in tetrahedral (A) or octahedral (B) sites, and the local distortion of the latter. The comparison of average and local parameters points to differences in the behaviour of doping elements and shows the size of (A) sites are independent of the host crystal composition while the average size of (B) sites varies with the composition. (author) [French] Une etude cristallographique des aluminates de magnesium a permis de determiner la deformation moyenne des sites cristallins. Une etude spectroscopique des aluminates de magnesium dopes avec des elements de transition a permis de localiser les dopants entre les sites tetraedriques (A) et octaedriques (B) et d'etudier la deformation locale de ces sites. La comparaison entre les parametres moyens et locaux a caracterise le comportement du dopant et montre que les dimensions des sites (A) sont independantes de la composition de la matrice, alors que les caracteristiques geometriques moyennes des sites (B) varient continument avec la composition. (auteur)

WAG 2 remedial investigation and site investigation site-specific work plan/health and safety checklist for the ecological assessment task, Kingfisher Study

International Nuclear Information System (INIS)

Holt, V.L.; Baron, L.A.

1994-05-01

This report provides specific details and requirements for the WAG 2 remedial investigation and site investigation Ecological Assessment Task, Kingfisher Study, including information that will contribute to safe completion of the project. The report includes historical background; a site map; project organization; task descriptions and hazard evaluations; controls; and monitoring, personal protective equipment, decontamination, and medical surveillance program requirements. The report also includes descriptions of site personnel and their certifications as well as suspected WAG 2 contaminants and their characteristics. The primary objective of the WAG 2 Kingfisher Study is to assess the feasibility of using kingfishers as biological monitors of contaminants on the Oak Ridge Reservation (ORR). Kingfisher sample collection will be used to determine the levels of contaminants and degree of bioaccumulation within a common piscivorous bird feeding on contaminated fish from streams on the ORR

Site Specific Ground Response Analysis for Quantifying Site Amplification at A Regolith Site

Directory of Open Access Journals (Sweden)

Bambang Setiawan

2017-08-01

Full Text Available DOI: 10.17014/ijog.4.3.159-167A numerical model has demonstrated that it can simulate reasonably well earthquake motions at the ground level during a seismic event. The most widely used model is an equivalent linear approach. The equivalent linear model was used to compute the free-field response of Adelaide regolith during the 1997 Burra earthquake. The aim of this study is to quantify the amplification at the investigated site. The model computed the ground response of horizontally layered soil deposits subjected to transient and vertically propagating shear waves through a one-dimensional-soil column. Each soil layer was assumed to be homogeneous, visco-elastic, and infinite in the horizontal extent. The results of this study were compared to other studies and forward computation of the geotechnical dynamic parameters of the investigated site. The amplification triggered by the 1997 Burra seismic event was deduced. This study reveals the amplification factor up to 3.6 at the studied site.

Coaching at the Building Site – A Feasibility Study

DEFF Research Database (Denmark)

Koch, Christian; Alsdorf, Morten; Sander, Dag

2006-01-01

successfully at a building site, in certain specific situations. The feeling of security is important for the focus persons at the building site and is best obtained in the open air. The way the leader acts towards the employee/focus person is decisive. Consciousness and responsibility is developed......Coaching has become widely used in business, and is well described. However it is rarely used in construction. This makes a contrast to the poor performance of site interaction, leading to a vast amount of reported cooperation and coordination problems. This paper investigates the feasibility...... observations in a building site operating with lean construction, where the managers did not use coaching consciously in their interaction with employees. 29 participant observations of five of the managers at the building site were carried out, at individual- and group meetings. Coaching can be used...

Yucca Mountain Site Characterization Project exploratory studies facilities construction status

International Nuclear Information System (INIS)

Allan, J.N.; Leonard, T.M.

1993-01-01

This paper discusses the progress to date on the construction planning and development of the Yucca Mountain Site Characterization Project (YMP) Exploratory Studies Facilities (ESF). The purpose of the ESF is to determine early site suitability and to characterize the subsurface of the Yucca Mountain site to assess its suitability for a potential high level nuclear waste repository. The present ESF configuration concept is for two main ramps to be excavated by tunnel boring machines (TBM) from the surface to the Topopah Spring Member of the Paintbrush Tuff Formation. From the main ramps, slightly above Topopah Spring level, supplemental ramps will be penetrated to the Calico Hills formation below the potential repository. There will be exploratory development drifts driven on both levels with the Main Test Area being located on the Topopah Spring level, which is the level of the proposed repository. The Calico Hills formation lies below the Topopah Spring member and is expected to provide the main geo-hydrologic barrier between the potential repository and the underlying saturated zones in the Crater Flat Tuff

Regulation of mTORC1 Signaling by Src Kinase Activity Is Akt1-Independent in RSV-Transformed Cells

Directory of Open Access Journals (Sweden)

Martina Vojtěchová

2008-02-01

Full Text Available Increased activity of the Src tyrosine protein kinase that has been observed in a large number of human malignancies appears to be a promising target for drug therapy. In the present study, a critical role of the Src activity in the deregulation of mTOR signaling pathway in Rous sarcoma virus (RSV-transformed hamster fibroblasts, H19 cells, was shown using these cells treated with the Src-specific inhibitor, SU6656, and clones of fibroblasts expressing either the active Src or the dominant-negative Src kinase-dead mutant. Disruption of the Src kinase activity results in substantial reduction of the phosphorylation and activity of the Akt/protein kinase B (PKB, phosphorylation of tuberin (TSC2, mammalian target of rapamycin (mTOR, S6K1, ribosomal protein S6, and eukaryotic initiation factor 4E-binding protein 4E-BP1. The ectopic, active Akt1 that was expressed in Src-deficient cells significantly enhanced phosphorylation of TSC2 in these cells, but it failed to activate the inhibited components of the mTOR pathway that are downstream of TSC2. The data indicate that the Src kinase activity is essential for the activity of mTOR-dependent signaling pathway and suggest that mTOR targets may be controlled by Src independently of Akt1/TSC2 cascade in cells expressing hyperactive Src protein. These observations might have an implication in drug resistance to mTOR inhibitor-based cancer therapy in certain cell types.

Stability and morphology of Ag nanoplatelets probed by depolarized dynamic light scattering

Science.gov (United States)

Zimbone, M.; Contino, A.; Maccarrone, G.; Musumeci, P.; Lo Faro, M. J.; Calcagno, L.

2018-06-01

The stability of silver nanoplatelet (NP) suspensions prepared with different concentrations of trisodium citrate (TSC) was studied by depolarized dynamic light scattering (DDLS) and UV–vis spectrometry. The morphology of the nanoparticles, as well as the color and stability of the sols, are tuned by the concentration of the capping agent. The nanoparticles prepared with high TSC concentration (>10‑4 M) are blue triangular NPs showing a slight truncation of the tips with aging. When low TSC concentrations are used, the color of the sols changes from blue to yellow with aging time and a strong modification of the morphology occurs: the nanoparticle shape changes from triangular to spherical. Remarkably, they show a high degree of anisotropy. The aging process was followed by the UV–vis spectra and by measuring the rotational diffusion coefficient by DDLS, providing information on the nanoparticle size and shape evolution. The high intensity of depolarized signal and the high value of rotational diffusion coefficient suggest that the aging process increases the thickness and the roughness of the nanoparticles

Extensive homology of chicken macrochromosomes in the karyotypes of Trachemys scripta elegans and Crocodylus niloticus revealed by chromosome painting despite long divergence times.

Science.gov (United States)

Kasai, F; O'Brien, P C M; Martin, S; Ferguson-Smith, M A

2012-01-01

We report extensive chromosome homology revealed by chromosome painting between chicken (Gallus gallus domesticus, GGA, 2n = 78) macrochromosomes (representing 70% of the chicken genome) and the chromosomes of a turtle, the red-eared slider (Trachemys scripta elegans, TSC, 2n = 50), and the Nile crocodile (Crocodylus niloticus, CNI, 2n = 32). Our data show that GGA1-8 arms seem to be conserved in the arms of TSC chromosomes, GGA1-2 arms are separated and homologous to CNI1p, 3q, 4q and 5q. In addition to GGAZ homologues in our previous study, large-scale GGA autosome syntenies have been conserved in turtle and crocodile despite hundreds of millions of years divergence time. Based on phylogenetic hypotheses that crocodiles diverged after the divergence of birds and turtles, our results in CNI suggest that GGA1-2 and TSC1-2 represent the ancestral state and that chromosome fissions followed by fusions have been the mechanisms responsible for the reduction of chromosome number in crocodiles. Copyright © 2012 S. Karger AG, Basel.

SWOT Analysis of Total Sanitation Campaign in Yavatmal District of Maharashtra.

Science.gov (United States)

Pardeshi, Geeta; Shirke, Avinash; Jagtap, Minal

2008-10-01

To study the strengths, weaknesses, opportunities, and threats of the Total Sanitation Campaign (TSC) in the Yavatmal district of Maharashtra. Data was collected in December 2006 through interviews with stakeholders, house-to-house surveys, focus group discussions, and transect walks. Information in each category was finalized in a meeting after brainstorming and discussion with the TSC cell members. The strengths of the campaign were innovations in Information Education and Communication, motivation through incentives, competitive spirit, active participation and partnerships, involvement of women, and universal coverage. The main weaknesses of the program were the absence of Rural Sanitary Marts/Production Centers, poor maintenance of Women Sanitary Complexes, lack of facilities for monitoring/ follow-up and a temporary focus of the campaign approach. There is an opportunity to tap additional resources, learn from other experiences, and institute back-up agencies to support and guide the community in the post-TSC phase. A change in administration and local leadership and loss of priority and interest needed to sustain the momentum while scaling up the interventions are possible threats for the program.

A Security Level Classification Method for Power Systems under N-1 Contingency

Directory of Open Access Journals (Sweden)

Zhigang Lu

2017-12-01

Full Text Available Security assessment is crucial for the reliable and secure operation of power systems. This paper proposes a security level classification (SLC method to analyze the security level of power systems both qualitatively and quantitatively. In this SLC method, security levels are graded according to a comprehensive safety index (CSI, which is defined by integrating the system margin index (SMI and load entropy. The SMI depends on the operating load and the total supply capacity (TSC under N-1 contingency, and the load entropy reflects the heterogeneity of load distribution calculated from entropy theory. In order to calculate the TSC under N-1 contingency considering both of the computational accuracy and speed, the TSC is converted into an extended conic quadratic programming (ECQP model. In addition, the load boundary vector (LBV model is established to obtain the capacity limit of each load bus, and thus detect potential risks of power systems. Finally, two modified practical power systems and the IEEE 118-bus test system are studied to validate the feasibility of the proposed SLC method.

Recurrent meningitis associated with frontal sinus tuber encephalocele in a patient with tuberous sclerosis.

Science.gov (United States)

Elbabaa, Samer K; Riggs, Angela D; Saad, Ali G

2011-07-01

Tuberous sclerosis complex (TSC) is a genetic neurocutaneous disorder that commonly affects the CNS. The most commonly associated brain tumors include cortical tubers, subependymal nodules, and subependymal giant cell astrocytomas (SEGAs). The authors report an unusual case of recurrent meningitis due to a tuber-containing encephalocele via the posterior wall of the frontal sinus. An 11-year-old girl presented with a history of TSC and previous SEGA resection via interhemispheric approach. She presented twice within 4 months with classic bacterial meningitis. Cerebrospinal fluid cultures revealed Streptococcus pneumoniae. Computed tomography and MR imaging of the brain showed a right frontal sinus encephalocele via a posterior frontal sinus wall defect. Both episodes of meningitis were treated successfully with standard regimens of intravenous antibiotics. The neurosurgical service was consulted to discuss surgical options. Via a bicoronal incision, a right basal frontal craniotomy was performed. A large frontal encephalocele was encountered in the frontal sinus. The encephalocele was herniating through a bony defect of the posterior sinus wall. The encephalocele was ligated and resected followed by removing frontal sinus mucosa and complete cranialization of frontal sinus. Repair of the sinus floor was conducted with fat and pericranial grafts followed by CSF diversion via lumbar drain. Histopathology of the resected encephalocele showed a TSC tuber covered with respiratory (frontal sinus) mucosa. Tuber cells were diffusely positive for GFAP. The patient underwent follow-up for 2 years without evidence of recurrent meningitis or CSF rhinorrhea. This report demonstrates that frontal tubers of TSC can protrude into the frontal sinus as acquired encephaloceles and present with recurrent meningitis. To the authors' knowledge, recurrent meningitis is not known to coincide with TSC. Careful clinical and radiographic follow-up for frontal tubers in patients with TSC is

Identification of Site Selection Factors in the U.S. Franchise Restaurant Industry: An Exploratory Study

OpenAIRE

Park, Kunsoon

2002-01-01

The purpose of this study was to identify and rank the importance of the site selection factors that influence the U.S. franchise restaurant industry as well as rank the confidence level of the experts. To identify the site selection factors, this study sought assistance and support from restaurant professionals. The Delphi technique was used to elicit the opinions of a panel of experts regarding the site selection factors. The panel was composed of restaurant professionals of restaurant c...

Site Study Plan for background environmental radioactivity, Deaf Smith County site, Texas: Environmental Field Program: Preliminary draft

International Nuclear Information System (INIS)

1987-06-01

The Background Environmental Radioactivity Site Study Plan describes a field program consisting of an initial radiological survey and a radiological sampling program. The field program includes measurement of direct radiation and collection and analysis of background radioactivity samples of air, precipitation, soil, water, milk, pasture grass, food crops, meat, poultry, game, and eggs. The plan describes for each study: the need for the study, the study design, data management and use, schedule of proposed activities, and quality assurance requirements. These studies will provide data needed to satisfy requirements contained in, or derived from, the Salt Repository Project (SRP) Requirements Document. 50 refs., 11 figs., 7 tabs

Preliminary studies on trace element contamination in dumping sites of municipal wastes in India and Vietnam

Science.gov (United States)

Agusa, T.; Kunito, T.; Nakashima, E.; Minh, T. B.; Tanabe, S.; Subramanian, A.; Viet, P. H.

2003-05-01

The disposal of wastes in dumping sites has increasingly caused concem about adverse health effects on the populations living nearby. However, no investigation has been conducted yet on contamination in dumping sites of municipal wastes in Asian developing countries. In this study, concentrations of 11 trace elements (V, Cr, Mn, Co, Cu, Zn, Mo, Ag, Cd, Sb and Pb) were detennined in scalp hair from the population living nearby and in soil from dumping sites and control sites of India and Vietnam. Soil samples in dumping site in India showed significantly higher concentrations of some trace elements than soils in control site, whereas this trend was not notable in Vietnam. This is probably due to the fact that the wastes were covered with the soil in the dumping site of Vietnam. Cadmium concentrations in some hair samples of people living near dumping site in India and Vietnam exceeded the level associated with learning disorder in children. Levels of most of the trace elements in hair were significantly higher in dumping site than those in control site in India and Vietnam, suggesting direct or indirect exposure to those elements from dumping wastes. To our knowledge, this is the first study of trace element contamination in dumping sites in India and Vietnam.

Diagnóstico Tardio de Esclerose Tuberosa

Directory of Open Access Journals (Sweden)

Catarina Oliveira

2016-03-01

Full Text Available Tuberous sclerosis (also referred to as Tuberous Sclerosis Complex is an autosomal dominant neurocutaneous syndrome that can involve multiple organs such as the brain, heart, kidney, lung, liver, skin and eye. The diagnosis is clinical and most patients are identified during childhood, in the context of a severe epileptic or neuropsychiatric disorder. Here we report a case of TSC diagnosed in an adult woman with dermatological and vascular involvement that were only recognized as manifestations of TSC after the diagnosis of her infant daughter. This case highlights how important it is that physicians recognize the full spectrum of manifestations of TSC, including the most unusual, so not to miss the diagnosis

X-ray and synchrotron methods in studies of cultural heritage sites

Energy Technology Data Exchange (ETDEWEB)

Koval’chuk, M. V.; Yatsishina, E. B.; Blagov, A. E.; Tereshchenko, E. Yu., E-mail: elenatereschenko@yandex.ru; Prosekov, P. A.; Dyakova, Yu. A. [National Research Centre “Kurchatov Institute” (Russian Federation)

2016-09-15

X-ray and synchrotron methods that are most widely used in studies of cultural heritage objects (including archaeological sites)—X-ray diffraction analysis, X-ray spectroscopy, and visualization techniques— have been considered. The reported examples show high efficiency and informativeness of natural science studies when solving most diverse problems of archaeology, history, the study of art, museology, etc.

X-ray and synchrotron methods in studies of cultural heritage sites

International Nuclear Information System (INIS)

Koval’chuk, M. V.; Yatsishina, E. B.; Blagov, A. E.; Tereshchenko, E. Yu.; Prosekov, P. A.; Dyakova, Yu. A.

2016-01-01

X-ray and synchrotron methods that are most widely used in studies of cultural heritage objects (including archaeological sites)—X-ray diffraction analysis, X-ray spectroscopy, and visualization techniques— have been considered. The reported examples show high efficiency and informativeness of natural science studies when solving most diverse problems of archaeology, history, the study of art, museology, etc.

Study of iron valence state and position in sub-site by Moessbauer spectroscopy

International Nuclear Information System (INIS)

Uhm, Young Rang; Lim, Jae Cheong; KIm, Chul Sung; Son, Kwang Jae

2014-01-01

The magnetic ordering temperature and the magnitude of the magnetic fields at the iron sites of YIG can be influenced by substituting, either partially or totally, the Fe 3+ ions at the octahedral and/or the tetrahedral sites with magnetic or diamagnetic ions, and/or by substitution the Y 3+ ions at the dodecahedral sites with magnetic rare earth ions. It has been known for some time that Moessbauer spectroscopy is a powerful method by which iron-containing garnets can be studied. We report here on the synthesis of the compounds with garnet-related structures of composition Y 3 Fe 4.5 Cr 0.5 O 12 and its examination by 57 Fe Moessbauer spectroscopy. The chromium in compounds of the Y 3 Fe 4.5 Cr 0.5 O 12 is distributed at an octahedral site. The Moessbauer spectra can be analyzed using 3 or 4 sets of six Lorentzians with increasing amount of Cr 3+ compounds in this system. It results from the distribution ( 4 C n ) of Fe 3+ and Cr 3+ at an octahedral site. A comparative study of ferrous tablets of Dynabi was carried out using Moessbauer spectroscopy. The obtained results revealed the presence of ferrous (Fe 2+ ) gluconate and ferrous fumarate in a sample. This observation is important to better control the iron state in such medicaments because their pharmaceutical effect in the body is related to the form and valence of iron. The Cr-containing yttrium iron garnet (YIG), and the exchange interactions and site distributions were studied using 57 Fe Moessbauer spectroscopy. The obtained results revealed the presence of ferrous (Fe 2+ ) gluconate and ferrous fumarate in the sample. This observation is important better control the iron state in such medicaments because their pharmaceutical effect in the body is related to the form and valence of iron

The Study on Policy Options for Siting Hazardous Energy Facility

Energy Technology Data Exchange (ETDEWEB)

Kim, Jin Oh [Korea Energy Economics Institute, Euiwang (Korea)

2000-10-01

The problem of site allocation on locally unwanted land uses related to energy utilities that extended most recently is becoming a new energy policy issue due to the improvement of national standard of living and livelihood quality. Residents do not generally agree on establishing the construction of public energy utilities in their village due to NIMBY syndrome while they basically agree to have them. These circumstances made a big problem against mass production of industry society and the improvement of the national welfare. Locally unwanted land use related to energy utilities includes waste incineration system, nuclear power plant, coal fired power plant, oil and Gas storage tank, briquette manufacturing plant and etc. Opportunity for SOC projects carried out by central and local government is lost because of the regional egoism. The site dispute between government and residents obstructs optimal energy supply to be necessary for industry growth and the national welfare. The main objective of this study is to propose the policy option for finding a solution after surveying theory and background of site troubles and dispute factors. Final results of this study propose a solution on structural and institutional dispute. The former introduces three kinds of approaches such as tradition, compensation and negotiation. The transition of an environmentally sound energy consumption pattern and the improvement of energy efficiency could be carried out by traditional approaches. To claim the damage and offer the accommodation facilities could be settled by compensational approaches. The establishment of regional decentralization on NIMBY facilities could be settled by negotiatory approaches through fair share criteria. The latter proposes 1) 'polluter pays principle', 2) internalization of social cost and benefit on air or water pollution, 3) the behind - the - scene negotiation in a bid to settle a site dispute, 4) and supporting system for peripheral areas

Site characterization quality assurance for the California LLRW Disposal Site Project

International Nuclear Information System (INIS)

Hanrahan, T.P.; Ench, J.E.; Serlin, C.L.; Bennett, C.B.

1988-01-01

In December of 1985 US Ecology was chosen as the license designee for the State of California's low-level radioactive waste disposal facility. In early 1987, three candidate sites were selected for characterization studies in preparation for identifying the preferred site. The geotechnical characterization activities along with studies of the ecological and archaeological attributes, as well as assessments of the socio-economic impacts and cultural resources all provide input towards selection of the proposed site. These technical studies in conjunction with comments from local citizen committees and other interested parties are used as a basis for determining the proposed site for which full site characterization as required by California licensing requirements are undertaken. The purpose of this paper is to present an overview of the program for Quality Assurance and Quality Control for the site characterization activities on the California LLRW Disposal Site Project. The focus is on three major perspectives: The composite QA Program and two of the primary characterization activities, the geotechnical and the meteorological investigations

Mapping Suitable Sites for Setting up Wind Farms: A Case Study of ...

African Journals Online (AJOL)

Mapping Suitable Sites for Setting up Wind Farms: A Case Study of Nyanga District. ... Ethiopian Journal of Environmental Studies and Management ... sources and continuous power shortages make wind energy a very attractive alternative to ...

A study on development of monitoring and assessment module for sites

International Nuclear Information System (INIS)

Park, Se Moon; Yoon, Bong Yo; Kim, Dae Jung; Park, Joo Wan; Kim, Chang Lak

2006-01-01

As the development of total management systems for sites along with site environmental information is becoming standard, the system known as the Site Information and Total Environmental database management System (SITES) has been developed over the last two years. The first result was a database management system for storing data obtained from facilities, and a site characterization in addition to an environmental assessment of a site. The SITES database is designed to be effective and practical for use with facility management and safety assessment in relation to Geographic Information Systems. SITES is a total management program, which includes its database, its data analysis system required for site characterization, a safety assessment modeling system and an environment monitoring system. I can contribute to the institutional management of the facility and to its safety reassessment. SITES is composed of two main modules: the SITES Database Module (SDM) and the Monitoring and Assessment (M and A) module. The M and A module is subdivided into two sub-modules: the Safety Assessment System (SAS) and the Site Environmental Monitoring System (SEMS). SAS controls the data (input and output) from the SITES DB for the site safety assessment, whereas SEMS controls the data obtained from the records of the measuring sensors and facilities. The on-line site and environmental monitoring data is managed in SEMS. The present paper introduces the procedure and function of the M and A modules

An integrated approach for estimating oil volume in petroleum-contaminated sites: a North American case study

International Nuclear Information System (INIS)

Chen, Z.; Huang, G.H.; Chakma, A.

1999-01-01

An integrated approach for estimating the distribution of light nonaqueous phase liquids (LNAPLs) such as oil spill and leakage in a porous media is proposed, based on a study at a site located in western Canada. The site has one original release source that is a flare pit, with on-site soil and groundwater seriously contaminated by petroleum products spilled over the past two decades. Results of the study show that soil properties and site characteristics have significant impact on the spreading of contaminants which affect the estimation of contaminant volume. Although the LNAPLs in the subsurface do not appear as a distinct layer, and the volume and distribution differ from site to site, the proposed method offers insight into the contamination details and is, therefore, considered to be an effective and convenient tool for obtaining a reasonable estimate of residual oil volume in the subsurface. Results could also be used in designing an enhanced recovery scheme for the site under study, as well as in designing multi-component models of the subsurface contamination for the purpose of risk assessment. 13 refs., 2 tabs., 2 figs

Tetragonal Lysozyme Interactions Studied by Site Directed Mutagenesis

Science.gov (United States)

Crawford, Lisa; Karr, Laurel J.; Nadarajah, Arunan; Pusey, Marc

1999-01-01

A number of recent experimental and theoretical studies have indicated that tetragonal lysozyme crystal growth proceeds by the addition of aggregates, formed by reversible self association of the solute molecules in the bulk solution. Periodic bond chain and atomic force microscopy studies have indicated that the probable growth unit is at minimum a 43 tetramer, and most likely an octamer composed of two complete turns about the 43 axis. If these results are correct, then there are intermolecular interactions which are only formed in the solution and others only formed at the joining of the growth unit to the crystal surface. We have set out to study these interactions, and the correctness of this hypothesis, using site directed mutagenesis of specific amino acid residues involved in the different bonds. We had initially expressed wild type lysozyme in S. cervasiae with yields of approximately 5 mg/L, which were eventually raised to approximately 40 mg/L. We are now moving the expression to the Pichia system, with anticipated yields of 300 to (3)500 mg/L, comparable to what can be obtained from egg whites. An additional advantage of using recombinant protein is the greater genetic homogeneity of the material obtained and the absence of any other contaminating egg proteins. The first mutation experiments are TYR 23 (Registered) PHE or ALA and ASN 113 (Registered) ALA or ASP. Both TYR 23 and ASN 113 form part of the postulated dimerization intermolecular binding site which lead to the formation of the 43 helix. Tyrosine also participates in an intermolecular hydrogen bond with ARG 114. The results of these and subsequent experiments will be discussed.

CD/MCD/VTVH-MCD Studies of Escherichia coli Bacterioferritin Support a Binuclear Iron Cofactor Site.

Science.gov (United States)

Kwak, Yeonju; Schwartz, Jennifer K; Huang, Victor W; Boice, Emily; Kurtz, Donald M; Solomon, Edward I

2015-12-01

Ferritins and bacterioferritins (Bfrs) utilize a binuclear non-heme iron binding site to catalyze oxidation of Fe(II), leading to formation of an iron mineral core within a protein shell. Unlike ferritins, in which the diiron site binds Fe(II) as a substrate, which then autoxidizes and migrates to the mineral core, the diiron site in Bfr has a 2-His/4-carboxylate ligand set that is commonly found in diiron cofactor enzymes. Bfrs could, therefore, utilize the diiron site as a cofactor rather than for substrate iron binding. In this study, we applied circular dichroism (CD), magnetic CD (MCD), and variable-temperature, variable-field MCD (VTVH-MCD) spectroscopies to define the geometric and electronic structures of the biferrous active site in Escherichia coli Bfr. For these studies, we used an engineered M52L variant, which is known to eliminate binding of a heme cofactor but to have very minor effects on either iron oxidation or mineral core formation. We also examined an H46A/D50A/M52L Bfr variant, which additionally disrupts a previously observed mononuclear non-heme iron binding site inside the protein shell. The spectral analyses define a binuclear and an additional mononuclear ferrous site. The biferrous site shows two different five-coordinate centers. After O2 oxidation and re-reduction, only the mononuclear ferrous signal is eliminated. The retention of the biferrous but not the mononuclear ferrous site upon O2 cycling supports a mechanism in which the binuclear site acts as a cofactor for the O2 reaction, while the mononuclear site binds the substrate Fe(II) that, after its oxidation to Fe(III), migrates to the mineral core.

A Comparative Study of Blood Culture Sampling from Umbilical Catheter Line versus Peripheral Site

Directory of Open Access Journals (Sweden)

Abdolkarim Hamedi

2010-08-01

Full Text Available Neonatal sepsis is an important cause of death and morbidity in newborns and is diagnosed by isolation of organism in blood culture. In several reports,reliablity of blood cultures were done from umbi lical catheters,have been demonstrated. The objective of the present study was to determine,wether an inde welling umbilical catheter, could be an alternative site for blood culture. In a prospective study over 6 months during 2006,141 paired blood cultures from 134 infant,were done simultaneously from peripheral site and umbilical catheter (mostly U. V. C,during the first four days of life. Majority of these infants were preterm and admitted to NICU for special care. these infants had indwelling umbilical line and had indication of sepsis workup. A total of 141 pairs of blood cultures were obtained from 134 infants. In 16 infants blood culture pairs were positive for one organism in both peripheral vein and umbilical site. 71. 6% of total cultures (n=11pairs were negative in boths site. A total of 22 pairs were positive in one site only,with 5 positive from peripheral vein only and the other 17 from umblical site. Two pairs were positve in boths site with two different organism. In over all 16 infant (11%of blood were considered to be contaminated. Contamination rate were 2. 4% and 9. 2% for peripheral and umbilical catheter site. Contamination rate increased after 48 hours of age in umbilical catheter. The result showed that after 2 days contamination rate for blood culture taken from catheter line increased and specifity decreased. We recommended that blood culture via umblical catheter in first 2 days in sick neonates with indwelling catheter can be a alternate site of blood culture sampelling.

Giving Back: Collaborations with Others in Ecological Studies on the Nevada National Security Site - 13058

International Nuclear Information System (INIS)

Wade, Scott A.; Knapp, Kathryn S.; Wills, Cathy A.

2013-01-01

Formerly named the Nevada Test Site, the Nevada National Security Site (NNSS) was the historical site for nuclear weapons testing from the 1950's to the early 1990's. The site was renamed in 2010 to reflect the diversity of nuclear, energy, and homeland security activities now conducted at the site. Biological and ecological programs and research have been conducted on the site for decades to address the impacts of radiation and to take advantage of the relatively undisturbed and isolated lands for gathering basic information on the occurrence and distribution of native plants and animals. Currently, the Office of the Assistant Manager for Environmental Management of the U.S. Department of Energy (DOE), National Nuclear Security Administration Nevada Site Office (NNSA/NSO) oversees the radiological biota monitoring and ecological compliance programs on the NNSS. The top priority of these programs are compliance with federal and state regulations. They focus on performing radiological dose assessments for the public who reside near the NNSS and for populations of plants and animals on the NNSS and in protecting important species and habitat from direct impacts of mission activities. The NNSS serves as an invaluable outdoor laboratory. The geographic and ecological diversity of the site offers researchers many opportunities to study human influences on ecosystems. NNSA/NSO has pursued collaborations with outside agencies and organizations to be able to conduct programs and studies that enhance radiological biota monitoring and ecosystem preservation when budgets are restrictive, as well as to provide valuable scientific information to the human health and natural resource communities at large. NNSA/NSO is using one current collaborative study to better assess the potential dose to the off-site public from the ingestion of game animals, the most realistic pathway for off-site public exposure at this time from radionuclide contamination on the NNSS. A second

Giving Back: Collaborations with Others in Ecological Studies on the Nevada National Security Site - 13058

Energy Technology Data Exchange (ETDEWEB)

Wade, Scott A.; Knapp, Kathryn S. [U.S. Department of Energy National Nuclear Security Agency, Nevada Site Office, P.O. Box 98518, Las Vegas, NV 89193-8518 (United States); Wills, Cathy A. [National Nuclear Security Technologies, LLC, P.O. Box 98521, M/S 260, Las Vegas, NV 89193-8521 (United States)

2013-07-01

Formerly named the Nevada Test Site, the Nevada National Security Site (NNSS) was the historical site for nuclear weapons testing from the 1950's to the early 1990's. The site was renamed in 2010 to reflect the diversity of nuclear, energy, and homeland security activities now conducted at the site. Biological and ecological programs and research have been conducted on the site for decades to address the impacts of radiation and to take advantage of the relatively undisturbed and isolated lands for gathering basic information on the occurrence and distribution of native plants and animals. Currently, the Office of the Assistant Manager for Environmental Management of the U.S. Department of Energy (DOE), National Nuclear Security Administration Nevada Site Office (NNSA/NSO) oversees the radiological biota monitoring and ecological compliance programs on the NNSS. The top priority of these programs are compliance with federal and state regulations. They focus on performing radiological dose assessments for the public who reside near the NNSS and for populations of plants and animals on the NNSS and in protecting important species and habitat from direct impacts of mission activities. The NNSS serves as an invaluable outdoor laboratory. The geographic and ecological diversity of the site offers researchers many opportunities to study human influences on ecosystems. NNSA/NSO has pursued collaborations with outside agencies and organizations to be able to conduct programs and studies that enhance radiological biota monitoring and ecosystem preservation when budgets are restrictive, as well as to provide valuable scientific information to the human health and natural resource communities at large. NNSA/NSO is using one current collaborative study to better assess the potential dose to the off-site public from the ingestion of game animals, the most realistic pathway for off-site public exposure at this time from radionuclide contamination on the NNSS. A second

Phosphatidylcholine transfer protein interacts with thioesterase superfamily member 2 to attenuate insulin signaling.

Science.gov (United States)

Ersoy, Baran A; Tarun, Akansha; D'Aquino, Katharine; Hancer, Nancy J; Ukomadu, Chinweike; White, Morris F; Michel, Thomas; Manning, Brendan D; Cohen, David E

2013-07-30

Phosphatidylcholine transfer protein (PC-TP) is a phospholipid-binding protein that is enriched in liver and that interacts with thioesterase superfamily member 2 (THEM2). Mice lacking either protein exhibit improved hepatic glucose homeostasis and are resistant to diet-induced diabetes. Insulin receptor substrate 2 (IRS2) and mammalian target of rapamycin complex 1 (mTORC1) are key effectors of insulin signaling, which is attenuated in diabetes. We found that PC-TP inhibited IRS2, as evidenced by insulin-independent IRS2 activation after knockdown, genetic ablation, or chemical inhibition of PC-TP. In addition, IRS2 was activated after knockdown of THEM2, providing support for a role for the interaction of PC-TP with THEM2 in suppressing insulin signaling. Additionally, we showed that PC-TP bound to tuberous sclerosis complex 2 (TSC2) and stabilized the components of the TSC1-TSC2 complex, which functions to inhibit mTORC1. Preventing phosphatidylcholine from binding to PC-TP disrupted interactions of PC-TP with THEM2 and TSC2, and disruption of the PC-TP-THEM2 complex was associated with increased activation of both IRS2 and mTORC1. In livers of mice with genetic ablation of PC-TP or that had been treated with a PC-TP inhibitor, steady-state amounts of IRS2 were increased, whereas those of TSC2 were decreased. These findings reveal a phospholipid-dependent mechanism that suppresses insulin signaling downstream of its receptor.

Bedrock neutralization study for the Bruin Lagoon Superfund Site

International Nuclear Information System (INIS)

Patelunas, G.M.; Lenhardt, D.R.; Niece, J.E.

1989-01-01

The Bruin Lagoon site is located in Bruin Butler County, Pennsylvania, and is listed as No. 3 on the U.S. Environmental Protection Agencies' National Priority List. The Lagoon contains waste petroleum tars, sulfuric acid, coal combustion ash, spent bauxite and other waste materials. This paper reports on the bedrock neutralization study, conducted to assess the feasibility of injecting caustic solutions into acid-contaminated bedrock beneath the lagoon. The site is underlain by a fine to medium grain quartz sandstone which is contaminated with acid to depths in excess of 30 feet. For this investigation, Nx-cores were obtained and pressure tests conducted to a depth of 30 feet below the top of rock. Leach tests were conducted on contaminated core sections using sodium hydroxide and sodium carbonate solutions. A total of 12 core sections were exposed in 3-inch diameter test cylinders and permeated under a positive pressure of 25 to 50 psi. Measurements of leachate volume, temperature, pH, and hydraulic conductivity were recorded

Case study of manufactured gas plant site remediations using thermal desorption

Energy Technology Data Exchange (ETDEWEB)

Vogel, R.G.; Hayes, T.; Slimon, K.F.; Unites, D. [Southern California Gas Company, Los Angeles, CA (United States)

1995-12-31

Southern California Gas Company (SoCal Gas) has recently remediated five of its former manufactured gas plant (MGP) sites using on-site and off-site thermal desorption. This technology has proven effective in the treatment of PAH-contaminated soils with widely variable concentrations. At two of the five sites, MGP-contaminated materials were excavated and thermally treated on site. At the other sites, MGP-contaminated materials were excavated and transported directly to an off-site thermal desorber. Much of the production was of oil-gas, giving lampblack contamination, but some coal tar was also present.

Quantitative Study of Biogas Generation Potential from Different Landfill Sites of Nepal

Directory of Open Access Journals (Sweden)

Bikash Adhikari

2015-01-01

Full Text Available This research paper was study of waste composition and quantitative analysis of biogas generation potential with its recovery at Sisdole, Pokhara and Karaute Dada landfill sites (LFS of Nepal. The waste management practice in LFS are significant deciding factors for the assessment of environmental impacts caused including the release of green house gases like methane, carbondioxide etc to the atmosphere, that could contribute significantly to global warming and climate change. The total waste disposed to Sisdole LFS, Pokhara LFS and Karaute Dada LFS are 410, 80 and 7.8 tons respectively.  The waste composition was studied onsite with waste reduction method and analyzed for their composition. The organic component of wastes was found high as 61.6%, 52.5% and 65% at Sisdole, Pokhara and Karaute Dada LFS respectively. The biogas potential at these landfill sites were 12157.78 cum, 851.99 cum and 169 cum of biogas per day in Sisdole, Pokhara and Karaute Dada LFS respectively. 4.68, 0.33 and 0.07 MW energy per day can be generated from these amounts of biogas produced in Sisdole, Pokhara and Karaute Dada LFS respectively. Proper gas collection system can be the source of income from these landfill sites and help to mitigate the adverse impact of methane that is being released from these landfill sites

Renewable energy potential on brownfield sites: A case study of Michigan

International Nuclear Information System (INIS)

Adelaja, Soji; Shaw, Judy; Beyea, Wayne; Charles McKeown, J.D.

2010-01-01

Federal priorities are increasingly favoring the replacement of conventional sources of energy with renewable energy. With the potential for a federal Renewable Electricity Standard (RES) legislation, many states are seeking to intensify their renewable energy generation. The demand for wind, solar, geothermal and bio-fuels-based energy is likely to be rapidly expressed on the landscape. However, local zoning and NIMBYism constraints slow down the placement of renewable energy projects. One area where land constraints may be lower is brownfields; whose development is currently constrained by diminished housing, commercial, and industrial property demand. Brownfield sites have the potential for rapid renewable energy deployment if state and national interests in this area materialize. This study investigates the application of renewable energy production on brownfield sites using Michigan as a case study. Wind and solar resource maps of Michigan were overlaid with the brownfield locations based on estimates of brownfield land capacity. The total estimated energy potential available on Michigan's brownfield sites is 4320 megawatts (MW) of plate capacity for wind and 1535for solar, equating to 43% of Michigan's residential electricity consumption (using 30% capacity factor). Estimated economic impacts include over $15 billion in investments and 17,500 in construction and long-term jobs.

Offal dumping sites influence the relative abundance and roosting site selection of Black Kites (Milvus migrans govinda) in urban landscape: a study from Kolkata metropolis, India.

Science.gov (United States)

Mazumdar, Subhendu; Ghose, Dipankar; Saha, Goutam Kumar

2017-12-14

Although Black Kites (Milvus migrans govinda) serve as major scavenging raptor in most of the urban areas, scientific studies on this important ecosystem service provider are almost non-existent in Indian context. The present study was carried out in a metropolis in eastern India to find out the factors influencing relative abundance and roosting site selection of Black Kites. Separate generalized linear models (GLMs) were performed considering encounter rate and roosting Black Kite abundance as response variables. The study conclusively indicated that encounter rates of Black Kites were significantly influenced by the presence of garbage dumps in its vicinity. Numbers of Black Kites were also higher in the roosting sites situated closer to garbage dumps and open spaces. In addition, expected counts of Black Kites significantly increased in roosting sites situated away from buildings and water bodies. However, built-up area and tree cover around the roosting sites had no influence on the abundance of Black Kites therein. With rapid urbanization and changing offal disposal patterns, our findings would be useful to ensure continued availability of food and roosting sites of Black Kites in urban areas.

The Effects of Social Networking Sites on Students' Studying and Habits

Science.gov (United States)

Gok, Tolga

2016-01-01

Social media is important to communicate with people, share/ask information, and follow/evaluate/interpret the events, etc. for everyone at the present time. The positive and negative effects of social networking sites on students' studying and habits were examined in this research. The study was conducted on 220 students in vocational school of…

Risk factors for acute surgical site infections after lumbar surgery: a retrospective study.

Science.gov (United States)

Lai, Qi; Song, Quanwei; Guo, Runsheng; Bi, Haidi; Liu, Xuqiang; Yu, Xiaolong; Zhu, Jianghao; Dai, Min; Zhang, Bin

2017-07-19

Currently, many scholars are concerned about the treatment of postoperative infection; however, few have completed multivariate analyses to determine factors that contribute to the risk of infection. Therefore, we conducted a multivariate analysis of a retrospectively collected database to analyze the risk factors for acute surgical site infection following lumbar surgery, including fracture fixation, lumbar fusion, and minimally invasive lumbar surgery. We retrospectively reviewed data from patients who underwent lumbar surgery between 2014 and 2016, including lumbar fusion, internal fracture fixation, and minimally invasive surgery in our hospital's spinal surgery unit. Patient demographics, procedures, and wound infection rates were analyzed using descriptive statistics, and risk factors were analyzed using logistic regression analyses. Twenty-six patients (2.81%) experienced acute surgical site infection following lumbar surgery in our study. The patients' mean body mass index, smoking history, operative time, blood loss, draining time, and drainage volume in the acute surgical site infection group were significantly different from those in the non-acute surgical site infection group (p operative type in the acute surgical site infection group were significantly different than those in the non-acute surgical site infection group (p operative type, operative time, blood loss, and drainage time were independent predictors of acute surgical site infection following lumbar surgery. In order to reduce the risk of infection following lumbar surgery, patients should be evaluated for the risk factors noted above.

Feasibility Study of Economics and Performance of Solar Photovoltaics at the Brisbane Baylands Brownfield Site in Brisbane, California. A Study Prepared in Partnership with the Environmental Protection Agency for the RE-Powering America's Land Initiative: Siting Renewable Energy on Potentially Contaminated Land and Mine Sites

Energy Technology Data Exchange (ETDEWEB)

Salasovich, J.; Geiger, J.; Healey, V.; Mosey, G.

2013-04-01

The U.S. Environmental Protection Agency (EPA), in accordance with the RE-Powering America's Land initiative, selected the Brisbane Baylands site in Brisbane, California, for a feasibility study of renewable energy production. The National Renewable Energy Laboratory (NREL) provided technical assistance for this project. The purpose of this report is to assess the site for a possible photovoltaic (PV) system installation and estimate the cost, performance, and site impacts of different PV options. In addition, the report recommends financing options that could assist in the implementation of a PV system at the site.

Impact of Site Selection and Study Conduct on Outcomes in Global Clinical Trials.

Science.gov (United States)

Sarwar, Chaudhry M S; Vaduganathan, Muthiah; Butler, Javed

2017-08-01

There are over 25 million patients living with heart failure globally. Overall, and especially post-discharge, clinical outcomes have remained poor in heart failure despite multiple trials, with both successes and failures over the last two decades. Matching therapies to the right patient population, identifying high-quality sites, and ensuring optimal trial design and execution represent important considerations in the development of novel therapeutics in this space. While clinical trials have undergone rapid globalization, this has come with regional variation in comorbidities, clinical parameters, and even clinical outcomes and treatment effects across international sites. These issues have now highlighted knowledge gaps about the conduct of trials, selection of study sites, and an unmet need to develop and identify "ideal" sites. There is a need for all stakeholders, including academia, investigators, healthcare organizations, patient advocacy groups, industry sponsors, research organizations, and regulatory authorities, to work as a multidisciplinary group to address these problems and develop practical solutions to improve trial conduct, efficiency, and execution. We review these trial-level issues using examples from contemporary studies to inform and optimize the design of future global clinical trials in heart failure.

Uranium mineral - groundwater equilibrium at the Palmottu natural analogue study site, Finland

International Nuclear Information System (INIS)

Ahonen, L.; Ruskeeniemi, T.; Blomqvist, R.; Ervanne, H.; Jaakkola, T.

1993-01-01

The redox-potential, pH, chemical composition of fracture waters, and uraninite alteration associated with the Palmottu uranium mineralization (a natural analogue study site for radioactive waste disposal in southwestern Finland), have been studied. The data have been interpreted by means of thermodynamic calculations. The results indicate equilibrium between uraninite, ferric hydroxide and groundwater in the bedrock of the study site. Partially oxidized uraninite (UO 2 .33) and ferric hydroxide are in equilibrium with fresh, slightly acidic and oxidized water type, while primary uraninite is stable with deeper waters that have a higher pH and lower Eh. Measured Eh-pH values of groundwater cluster within a relatively narrow range indicating buffering by heterogenous redox-processes. A good consistency between measured Eh and analyzed uranium oxidation states was observed

Veteran Teacher Engagement in Site-Based Professional Development: A Mixed Methods Study

Science.gov (United States)

Houston, Biaze L.

2016-01-01

This research study examined how teachers self-report their levels of engagement, which factors they believe contribute most to their engagement, and which assumptions of andragogy most heavily influence teacher engagement in site-based professional development. This study employed a convergent parallel mixed methods design to study veteran…

Feasibility Study of Economics and Performance of Biomass Power Generation at the Former Farmland Industries Site in Lawrence, Kansas. A Study Prepared in Partnership with the Environmental Protection Agency for the RE-Powering America's Land Initiative: Siting Renewable Energy on Potentially Contaminated Land and Mine Sites

Energy Technology Data Exchange (ETDEWEB)

Tomberlin, G.; Mosey, G.

2013-03-01

Under the RE-Powering America's Land initiative, the U.S. Environmental Protection Agency (EPA) provided funding to the National Renewable Energy Laboratory (NREL) to support a feasibility study of biomass renewable energy generation at the former Farmland Industries site in Lawrence, Kansas. Feasibility assessment team members conducted a site assessment to gather information integral to this feasibility study. Information such as biomass resources, transmission availability, on-site uses for heat and power, community acceptance, and ground conditions were considered.

Basic Study for Active Nucleation Site Density Evaluation in Subcooled Flow Boiling

International Nuclear Information System (INIS)

Chu, In Cheol; Song, Chul Hwa

2008-01-01

Numerous studies have been performed on a active nucleation site density (ANSD) due to its governing influence on a heat transfer. However, most of the studies were focused on pool boiling conditions. Kocamustafaogullari and Ishii developed an ANSD correlation from a parametric study of the existing pool boiling data. Also, they extended the correlation to a convective flow boiling condition by adopting the nucleation suppression factor of Chen's heat transfer correlation. However, the appropriateness of applying the Chen's suppression factor to an ANSD correlation was not fully validated because there was not enough experimental data on ANSD in the forced convective flow boiling. Basu et al. performed forced convective boiling experiments and proposed a correlation of ANSD which is the only correlation based on experimental data for a forced convective boiling. They concluded that the ANSD is only dependent on the static contact angle and the wall superheat, and is independent of the flow rate and the subcooling, which contradict the general acceptance of the nucleation suppression in the forced convective boiling. It seems that no reliable ANSD correlation or model is available for a forced convective boiling. In the present study, the effect of the flow velocity on the suppression of the nucleation site was examined, and the effectiveness of a Brewster reflection technique for the identification of the nucleation site was also examined

Clinical Characteristics of Connective Tissue Nevi in Tuberous Sclerosis Complex With Special Emphasis on Shagreen Patches.

Science.gov (United States)

Bongiorno, Michelle A; Nathan, Neera; Oyerinde, Oyetewa; Wang, Ji-An; Lee, Chyi-Chia Richard; Brown, G Thomas; Moss, Joel; Darling, Thomas N

2017-07-01

Patients with tuberous sclerosis complex (TSC) frequently develop collagenous connective tissue nevi. The prototypical lesion is a large shagreen patch located on the lower back, but some patients only manifest small collagenomas or have lesions elsewhere on the body. The ability to recognize these variable presentations can be important for the diagnosis of TSC. To describe the clinical characteristics of connective tissue nevi on the trunk and extremities of patients with tuberous sclerosis complex. A retrospective analysis of patient medical records and skin photography was performed; 104 adult patients with TSC were enrolled in an observational cohort study that was enriched for those with pulmonary lymphangioleiomyomatosis, and was therefore composed mostly of women (99 women, 5 men). All patients included were examined at the National Institutes of Health (NIH) in Bethesda, Maryland, from 1998 to 2013. Connective tissue nevi were categorized per anatomic location and size. Lesions less than 1 cm in diameter were termed collagenomas. Shagreen patches were characterized as small (1 to connective tissue nevi in patients with TSC. Overall, 58 of 104 patients (median [range] age, 42 [19-70] years) with TSC (56%) had at least 1 connective tissue nevus on the trunk or thighs; of these, 28 of 58 patients (48%) had a solitary lesion, and 30 of 58 patients (52%) had 2 or more lesions. Overall, 120 lesions from 55 patients were classified by size; 46 lesions (38%) were collagenomas; 39 lesions (32%) were small shagreen patches; 21 lesions (18%), medium shagreen patches; and 14 lesions (12%), large shagreen patches. The distribution of lesions was 9% (n = 11), upper back; 29% (n = 35), middle back; 51% (n = 61), lower back; and 11% (n = 13), other locations. All 26 shagreen patches that were analyzed histopathologically had coarse collagen fibers and 24 of 26 stained with Miller elastic stain had decreased elastic fibers. On immunoblot analysis

Cytokine-induced loss of glucocorticoid function: effect of kinase inhibitors, long-acting β(2-adrenoceptor [corrected] agonist and glucocorticoid receptor ligands.

Directory of Open Access Journals (Sweden)

Christopher F Rider

Full Text Available Acting on the glucocorticoid receptor (NR3C1, glucocorticoids are widely used to treat inflammatory diseases. However, glucocorticoid resistance often leads to suboptimal asthma control. Since glucocorticoid-induced gene expression contributes to glucocorticoid activity, the aim of this study was to use a 2 × glucocorticoid response element (GRE reporter and glucocorticoid-induced gene expression to investigate approaches to combat cytokine-induced glucocorticoid resistance. Pre-treatment with tumor necrosis factor-α (TNF or interleukin-1β inhibited dexamethasone-induced mRNA expression of the putative anti-inflammatory genes RGS2 and TSC22D3, or just TSC22D3, in primary human airway epithelial and smooth muscle cells, respectively. Dexamethasone-induced DUSP1 mRNA was unaffected. In human bronchial epithelial BEAS-2B cells, dexamethasone-induced TSC22D3 and CDKN1C expression (at 6 h was reduced by TNF pre-treatment, whereas DUSP1 and RGS2 mRNAs were unaffected. TNF pre-treatment also reduced dexamethasone-dependent 2×GRE reporter activation. This was partially reversed by PS-1145 and c-jun N-terminal kinase (JNK inhibitor VIII, inhibitors of IKK2 and JNK, respectively. However, neither inhibitor affected TNF-dependent loss of dexamethasone-induced CDKN1C or TSC22D3 mRNA. Similarly, inhibitors of the extracellular signal-regulated kinase, p38, phosphoinositide 3-kinase or protein kinase C pathways failed to attenuate TNF-dependent repression of the 2×GRE reporter. Fluticasone furoate, fluticasone propionate and budesonide were full agonists relative to dexamethasone, while GSK9027, RU24858, des-ciclesonide and GW870086X were partial agonists on the 2×GRE reporter. TNF reduced reporter activity in proportion with agonist efficacy. Full and partial agonists showed various degrees of agonism on RGS2 and TSC22D3 expression, but were equally effective at inducing CDKN1C and DUSP1, and did not affect the repression of CDKN1C or TSC22D3

Development of severe accident management guidance for Younggwang units 5 and 6

International Nuclear Information System (INIS)

Lee, K. W.; Beon, C. S.; Kim, M. K.; Hong, S. Y.; Park, K. S.

2001-01-01

Severe Accident Management Guidance (SAMG) has been developed for Younggwang Units 5 and 6. It is consisted of Severe Accident Control Room Guideline, Diagnostic Flow Chart, Severe Accident Guideline, Severe Challenge Guideline, TSC Long Term Monitoring, SAMG Termination. Severe Accident Control Room Guideline, which deals with severe accident after finishing Emergency Operation Procedure, consists of acitions before and after TSC actuation. Seven servere accident management strategies are developed. Diagnostic Flow Chart, Severe Accident Guideline, and Severe Challenge Guideline are developed for each strategy, which enables the users to the implementation of strategy easily and systematically. TSC Long Term Monitoring is also developed to monitor long term activities after a particular strategy. Total of 45 set points are developed for decision making during the implementation of the SAMG

Effect of Silica Fume on two-stage Concrete Strength

Science.gov (United States)

Abdelgader, H. S.; El-Baden, A. S.

2015-11-01

Two-stage concrete (TSC) is an innovative concrete that does not require vibration for placing and compaction. TSC is a simple concept; it is made using the same basic constituents as traditional concrete: cement, coarse aggregate, sand and water as well as mineral and chemical admixtures. As its name suggests, it is produced through a two-stage process. Firstly washed coarse aggregate is placed into the formwork in-situ. Later a specifically designed self compacting grout is introduced into the form from the lowest point under gravity pressure to fill the voids, cementing the aggregate into a monolith. The hardened concrete is dense, homogeneous and has in general improved engineering properties and durability. This paper presents the results from a research work attempt to study the effect of silica fume (SF) and superplasticizers admixtures (SP) on compressive and tensile strength of TSC using various combinations of water to cement ratio (w/c) and cement to sand ratio (c/s). Thirty six concrete mixes with different grout constituents were tested. From each mix twenty four standard cylinder samples of size (150mm×300mm) of concrete containing crushed aggregate were produced. The tested samples were made from combinations of w/c equal to: 0.45, 0.55 and 0.85, and three c/s of values: 0.5, 1 and 1.5. Silica fume was added at a dosage of 6% of weight of cement, while superplasticizer was added at a dosage of 2% of cement weight. Results indicated that both tensile and compressive strength of TSC can be statistically derived as a function of w/c and c/s with good correlation coefficients. The basic principle of traditional concrete, which says that an increase in water/cement ratio will lead to a reduction in compressive strength, was shown to hold true for TSC specimens tested. Using a combination of both silica fume and superplasticisers caused a significant increase in strength relative to control mixes.

Stability of the resistance to the thiosemicarbazone derived from 5,6-dimethoxy-1-indanone, a non-nucleoside polymerase inhibitor of bovine viral diarrhea virus.

Science.gov (United States)

Castro, Eliana F; Campos, Rodolfo H; Cavallaro, Lucía V

2014-01-01

Bovine viral diarrhea virus (BVDV) is the prototype Pestivirus. BVDV infection is distributed worldwide and causes serious problems for the livestock industry. The thiosemicarbazone of 5,6-dimethoxy-1-indanone (TSC) is a non-nucleoside polymerase inhibitor (NNI) of BVDV. All TSC-resistant BVDV variants (BVDV-TSCr T1-5) present an N264D mutation in the NS5B gene (RdRp) whereas the variant BVDV-TSCr T1 also presents an NS5B A392E mutation. In the present study, we carried out twenty passages of BVDV-TSCr T1-5 in MDBK cells in the absence of TSC to evaluate the stability of the resistance. The viral populations obtained (BVDV R1-5) remained resistant to the antiviral compound and conserved the mutations in NS5B associated with this phenotype. Along the passages, BVDV R2, R3 and R5 presented a delay in the production of cytopathic effect that correlated with a decrease in cell apoptosis and intracellular accumulation of viral RNA. The complete genome sequences that encode for NS2 to NS5B, Npro and Erns were analyzed. Additional mutations were detected in the NS5B of BVDV R1, R3 and R4. In both BVDV R2 and R3, most of the mutations found were localized in NS5A, whereas in BVDV R5, the only mutation fixed was NS5A V177A. These results suggest that mutations in NS5A could alter BVDV cytopathogenicity. In conclusion, the stability of the resistance to TSC may be due to the fixation of different compensatory mutations in each BVDV-TSCr. During their replication in a TSC-free medium, some virus populations presented a kind of interaction with the host cell that resembled a persistent infection: decreased cytopathogenicity and viral genome synthesis. This is the first report on the stability of antiviral resistance and on the evolution of NNI-resistant BVDV variants. The results obtained for BVDV-TSCr could also be applied for other NNIs.

Study of iron valence state and position in sub-site by Moessbauer spectroscopy

Energy Technology Data Exchange (ETDEWEB)

Uhm, Young Rang; Lim, Jae Cheong; KIm, Chul Sung [Korea Atomic Energy Research Institute, Daejeon (Korea, Republic of); Son, Kwang Jae [Kookmin Univ., Seoul (Korea, Republic of)

2014-05-15

The magnetic ordering temperature and the magnitude of the magnetic fields at the iron sites of YIG can be influenced by substituting, either partially or totally, the Fe{sup 3+} ions at the octahedral and/or the tetrahedral sites with magnetic or diamagnetic ions, and/or by substitution the Y{sup 3+} ions at the dodecahedral sites with magnetic rare earth ions. It has been known for some time that Moessbauer spectroscopy is a powerful method by which iron-containing garnets can be studied. We report here on the synthesis of the compounds with garnet-related structures of composition Y{sub 3}Fe{sub 4.5}Cr{sub 0.5}O{sub 12} and its examination by {sup 57}Fe Moessbauer spectroscopy. The chromium in compounds of the Y{sub 3}Fe{sub 4.5}Cr{sub 0.5}O{sub 12} is distributed at an octahedral site. The Moessbauer spectra can be analyzed using 3 or 4 sets of six Lorentzians with increasing amount of Cr{sup 3+} compounds in this system. It results from the distribution ({sub 4}C{sub n}) of Fe{sup 3+} and Cr{sup 3+} at an octahedral site. A comparative study of ferrous tablets of Dynabi was carried out using Moessbauer spectroscopy. The obtained results revealed the presence of ferrous (Fe{sup 2+}) gluconate and ferrous fumarate in a sample. This observation is important to better control the iron state in such medicaments because their pharmaceutical effect in the body is related to the form and valence of iron. The Cr-containing yttrium iron garnet (YIG), and the exchange interactions and site distributions were studied using {sup 57}Fe Moessbauer spectroscopy. The obtained results revealed the presence of ferrous (Fe{sup 2+}) gluconate and ferrous fumarate in the sample. This observation is important better control the iron state in such medicaments because their pharmaceutical effect in the body is related to the form and valence of iron.

Site study plan for exploratory shaft monitoring wells, Deaf Smith County Site, Texas: Preliminary Draft

International Nuclear Information System (INIS)

1988-01-01

As part of site characterization studies, two exploratory shafts will be constructed at the Deaf Smith County site, Texas. Twelve wells at five locations have been proposed to monitor potential impacts of shaft construction on water-bearing zones in the Ogallala Formation and the Dockum Group. In addition, tests have been proposed to determine the hydraulic properties of the water-bearing zones for use in design and construction of the shafts. Samples of the Blackwater Draw Formation, Ogallala Formation, and Dockum Group will be obtained during construction of these wells. Visual indentification, laboratory testing, and in situ testing will yield data necessary for Exploratory Shaft Facility design and construction. This activity provides the earliest data on the Blackwater Drew Formation, Ogallala Formation, and Dockum Group near the exploratory shaft locations. Drilling and hydrologic testing are scheduled prior to other subsurface activity at the Exploratory Shaft Facility to establish ground-water baseline conditions. The Technical Field Services Contractor is responsible for conducting the field program of drilling and testing. Samples and data will be handled and reported in accordance with established Salt Repository Project procedures. A quality assurance program will be utilized to assure that activities affecting quality are performed correctly and that the appropriate documentation is maintained. 45 refs., 11 figs., 4 tabs

Transport sensitivity studies for SITE-94: Time-dependent site-scale modelling of future glacial impact

International Nuclear Information System (INIS)

King-Clayton, L.; Smith, Paul

1996-10-01

The report details the methodology and preliminary results from the modelling of radionuclide transport from a hypothetical repository based at the Aespoe site in Sweden. The work complements and utilizes the results from regional-scale, variable density flow modelling by Provost, in which the groundwater flow field is time dependent, reflecting the impact of climate evolution over the next 130,000 years. The climate evolution include development of permafrost conditions and ice sheet advance and retreat. The results indicate that temporal changes in flow conditions owing to future climate changes have a significant effect on the transport of radionuclides from a repository. In all cases modelled with time-dependent boundary conditions, the greatest radionuclide fluxes occur towards the end of the main glacial periods, and correspond to periods of high groundwater discharge at the margin of the modelled ice sheets. Fluxes to the biosphere may, for limited periods (2000 years or less), be three times higher than those from the near field. The study provides a quantitative way of illustrating the possible effects of future glaciations on radionuclide transport from the repository. Such effects are likely to be significant in any potential siting area predicted to be affected by future periods of ice cover. 8 refs, 22 tabs, 119 figs

Multivariate approach to assessing ecotoxicity on abandoned oil refinery environments: Study site description

International Nuclear Information System (INIS)

Lochmiller, R.L.; Yates, G.W.

1993-01-01

Ecology risk assessment requires a clear understanding of how complex mixtures of environmental contaminants impact terrestrial ecosystems. This assessment process could be more universally applied to terrestrial ecosystems with better defined assay systems for evaluating impacts of exposure on organisms, populations, and communities. The authors objective was to define and assess the efficacy of a battery of assays incorporating both standard biological toxicity test and in situ biomonitors for evaluating ecological risks on terrestrial environments contaminated with complex mixtures of petrochemicals on a 160 acre abandoned oil refinery in central Oklahoma. Three suspected contaminated and three uncontaminated reference sites were selected for intensive study. Habitat on each study site is representative of disturbed tall-grass prairie and supports dense, diverse small mammal communities. Soil samples were subjected to aqueous extraction and resulting leachates analyzed for heavy metals, selected ions, and organics. Analytical results support their initial assessment that toxic study sites were contaminated with complex mixtures including lead, zinc, arsenic, chloride, sulfate, potassium, and complex mixtures of polar and non-polar organics

Experimental Studies on Earthen Architecture Sites Consolidated with BS Materials in Arid Regions

Directory of Open Access Journals (Sweden)

Dong Zhao

2016-01-01

Full Text Available This paper takes the preservation works on the archaeological sites of Gaochang Ruins, Xinjiang, as background. Based on the soil characteristics analysis on the archaeological sites, experimental studies were conducted on the consolidation effect of the BS-10 consolidation material on the archaeological sites of adobe and rammed earth. The results showed the following:, after consolidation, the wind erosion resistance of the soil on the archaeological sites was substantially increased, and the wind erosion modulus was reduced by 5~8 times; the soil exhibited fine grid structure and significantly reduced degree of permeability, while still maintaining the moisture exchange between inside and outside the soil; there is excellent ageing resistance; the resistance to freezing and thawing was closely related to the soil water content and had little influence in arid regions; the water resistance was improved and could satisfy the requirements for consolidating the sites in arid regions; the unconfined compressive strength was improved moderately, which was the key direction of improvement in the future, and the consolidated soil did not form duricrust on the surface and had good bonding strength with the internal parts. The BS-10 material can meet the consolidation requirements for the earthen archaeological sites in arid regions of northwest China.

Central repository for low- and intermediate-level waste (ALMA) conceptual design, siting and safety study

International Nuclear Information System (INIS)

Kjellbert, N.; Haeggblom, H.; Cederstroem, M.; Lundgren, T.

1980-07-01

A generic design, siting and safety study of a proposed repository for low- and intermediate-level waste has been made. Special emphasis has been placed on safety characterostics. The conceptual design and the generic site, on which the study is based, are realistically chosen in accordance with present construction techniques and the existing geohydrological conditions in Sweden. (Auth.)

Direct detection and quantification of abasic sites for in vivo studies of DNA damage and repair

International Nuclear Information System (INIS)

Wang Yanming; Liu Lili; Wu Chunying; Bulgar, Alina; Somoza, Eduardo; Zhu Wenxia; Gerson, Stanton L.

2009-01-01

Use of chemotherapeutic agents to induce cytotoxic DNA damage and programmed cell death is a key strategy in cancer treatments. However, the efficacy of DNA-targeted agents such as temozolomide is often compromised by intrinsic cellular responses such as DNA base excision repair (BER). Previous studies have shown that BER pathway resulted in formation of abasic or apurinic/apyrimidinic (AP) sites, and blockage of AP sites led to a significant enhancement of drug sensitivity due to reduction of DNA base excision repair. Since a number of chemotherapeutic agents also induce formation of AP sites, monitoring of these sites as a clinical correlate of drug effect will provide a useful tool in the development of DNA-targeted chemotherapies aimed at blocking abasic sites from repair. Here we report an imaging technique based on positron emission tomography (PET) that allows for direct quantification of AP sites in vivo. For this purpose, positron-emitting carbon-11 has been incorporated into methoxyamine ([ 11 C]MX) that binds covalently to AP sites with high specificity. The binding specificity of [ 11 C]MX for AP sites was demonstrated by in vivo blocking experiments. Using [ 11 C]MX as a radiotracer, animal PET studies have been conducted in melanoma and glioma xenografts for quantification of AP sites. Following induction of AP sites by temozolomide, both tumor models showed significant increase of [ 11 C]MX uptake in tumor regions in terms of radioactivity concentration as a function of time, which correlates well with conventional aldehyde reactive probe (ARP)-based bioassays for AP sites.

A Study On The Applicability Of SERVQUAL Dimensions Of Web Sites

NARCIS (Netherlands)

A. van der Wiele (Ton); J.D. van Iwaarden (Jos)

2002-01-01

textabstractA survey has been undertaken amongst students of two universities (Erasmus University Rotterdam, The Netherlands, and Northeastern University Boston, USA) to study what quality factors are perceived as important in relation to their use of web sites. The results of the questionnaire

Feasibility Study of Economics and Performance of Solar Photovoltaics at the Kerr McGee Site in Columbus, Mississippi. A Study Prepared in Partnership with the Environmental Protection Agency for the RE-Powering America's Land Initiative: Siting Renewable Energy on Potentially Contaminated Land and Mine Sites

Energy Technology Data Exchange (ETDEWEB)

Simon, J.; Mosey, G.

2013-01-01

The U.S. Environmental Protection Agency (EPA), in accordance with the RE-Powering America's Land initiative, selected the Kerr McGee site in Columbus, Mississippi, for a feasibility study of renewable energy production. The National Renewable Energy Laboratory (NREL) provided technical assistance for this project. The purpose of this report is to assess the site for a possible photovoltaic (PV) system installation and estimate the cost, performance, and site impacts of different PV options. In addition, the report recommends financing options that could assist in the implementation of a PV system at the site.

Feasibility Study of Economics and Performance of Solar Photovoltaics at the Crazy Horse Landfill Site in Salinas, California. A Study Prepared in Partnership with the Environmental Protection Agency for the RE-Powering America's Land Initiative: Siting Renewable Energy on Potentially Contaminated Land and Mine Sites

Energy Technology Data Exchange (ETDEWEB)

Stoltenberg, B.; Konz, C.; Mosey, G.

2013-03-01

The U.S. Environmental Protection Agency (EPA), in accordance with the RE-Powering America's Land initiative, selected the Crazy Horse Landfill site in Salinas, California, for a feasibility study of renewable energy production. The National Renewable Energy Laboratory (NREL) was contacted to provide technical assistance for this project. The purpose of this report is to assess the site for a possible photovoltaic (PV) system installation and estimate the cost, performance, operation and maintenance requirements, and site impacts of different PV options. In addition, the report recommends financing options that could assist in the implementation of a PV system at the site.

Potential of semiautomated, synoptic geologic studies for characterization of hazardous waste sites

International Nuclear Information System (INIS)

Foley, M.G.; Beaver, D.E.; Glennon, M.A.; Eliason, J.R.

1988-01-01

Siting studies for licensing hazardous facilities require three-dimensional characterization of site geology including lithology, structure, and tectonics. The scope of these studies depends on the type of hazardous facility and its associated regulations. This scope can vary from a pro forma literature review to an extensive, multiyear research effort. Further, the regulatory environment often requires that the credibility of such studies be established in administrative and litigative proceedings, rather than solely by technical peer review. Pacific Northwest Laboratory (PNL) has developed a technology called remote geologic analysis (RGA). This technology provides reproducible photogeologic maps, determinations of three- dimensional faults and fracture sets expressed as erosional lineaments or planar topographic features, planar feature identification in seismic hypocenter data, and crustal- stress/tectonic analyses. Results from the RGA establish a foundation for interpretations that are defensible in licensing proceedings

An integrated study of spatial multicriteria analysis and mathematical modelling for managed aquifer recharge site suitability mapping and site ranking at Northern Gaza coastal aquifer.

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Rahman, Mohammad Azizur; Rusteberg, Bernd; Uddin, Mohammad Salah; Lutz, Annegret; Saada, Muath Abu; Sauter, Martin

2013-07-30

This paper describes an integrated approach of site suitability mapping and ranking of the most suitable sites, for the implementation of Managed Aquifer Recharge (MAR) projects, using spatial multicriteria decision analysis (SMCDA) techniques and mathematical modelling. The SMCDA procedure contains constraint mapping, site suitability analysis with criteria standardization and weighting, criteria overlay by analytical hierarchy process (AHP) combined with weighted linear combination (WLC) and ordered weighted averaging (OWA), and sensitivity analysis. The hydrogeological impacts of the selected most suitable sites were quantified by using groundwater flow and transport modelling techniques. Finally, ranking of the selected sites was done with the WLC method. The integrated approach is demonstrated by a case study in the coastal aquifer of North Gaza. Constraint mapping shows that 50% of the total study area is suitable for MAR implementation. About 25% of the total area is "very good" and 25% percent is "good" for MAR, according to the site suitability analysis. Six locations were selected and ranked against six representative decision criteria. Long term (year 2003 to year 2040) groundwater flow and transport simulations were performed to quantify the selected criteria under MAR project operation conditions at the selected sites. Finally, the suitability mapping and hydrogeological investigation recommends that the location of the existing infiltration ponds, constructed near the planned North Gaza Wastewater Treatment Plant (NGWWTP) is most suitable for MAR project implementation. This paper concludes that mathematical modelling should be combined with the SMCDA technique in order to select the best location for MAR project implementation. Besides MAR project implementation, the generalised approach can be applicable for any other water resources development project that deals with site selection and implementation. Copyright © 2013 Elsevier Ltd. All rights

A case study of risk assessment in contaminated site remediation in China

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