Relevant bibliographies by topics / Tsc1, Tsc2

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Academic literature on the topic 'Tsc1, Tsc2'

Author: Grafiati
Published: 4 June 2021
Last updated: 16 February 2022

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Journal articles on the topic "Tsc1, Tsc2"

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Kwiatkowski, David. "Cancer Genetics: TSC1, TSC2, TSC3? or mosaicism?" European Journal of Human Genetics 13, no. 6 (May 24, 2005): 695–96. http://dx.doi.org/10.1038/sj.ejhg.5201412.

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Goncharova, Elena, Dmitry Goncharov, Daniel Noonan, and Vera P. Krymskaya. "TSC2 modulates actin cytoskeleton and focal adhesion through TSC1-binding domain and the Rac1 GTPase." Journal of Cell Biology 167, no. 6 (December 20, 2004): 1171–82. http://dx.doi.org/10.1083/jcb.200405130.

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Tuberous sclerosis complex (TSC) 1 and TSC2 are thought to be involved in protein translational regulation and cell growth, and loss of their function is a cause of TSC and lymphangioleiomyomatosis (LAM). However, TSC1 also activates Rho and regulates cell adhesion. We found that TSC2 modulates actin dynamics and cell adhesion and the TSC1-binding domain (TSC2-HBD) is essential for this function of TSC2. Expression of TSC2 or TSC2-HBD in TSC2−/− cells promoted Rac1 activation, inhibition of Rho, stress fiber disassembly, and focal adhesion remodeling. The down-regulation of TSC1 with TSC1 siRNA in TSC2−/− cells activated Rac1 and induced loss of stress fibers. Our data indicate that TSC1 inhibits Rac1 and TSC2 blocks this activity of TSC1. Because TSC1 and TSC2 regulate Rho and Rac1, whose activities are interconnected in a reciprocal fashion, loss of either TSC1 or TSC2 function may result in the deregulation of cell motility and adhesion, which are associated with the pathobiology of TSC and LAM.
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Wang, Yanye, Song Xu, Shikang Zhao, Shuai Zhu, Xiongfei Li, Xi Lei, Jun Chen, and Gang Chen. "Clinical and molecular characteristics of TSC1/2 mutant lung cancer." Journal of Clinical Oncology 38, no. 15_suppl (May 20, 2020): e21647-e21647. http://dx.doi.org/10.1200/jco.2020.38.15_suppl.e21647.

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e21647 Background: Tumor suppressor genes TSC1 and TSC2 inhibit cell growth through inactivation the function of mTORC1. Previous studies have demonstrated that loss of function mutation of either TSC1 or TSC2 gene result in formation of neoplasm in multiple tissues. However, the clinical significance of TSC1 and TSC2 in non-small-cell lung cancer (NSCLC) remains unknown. This study aimed to investigate the clinical and molecular characteristics of TSC1 and TSC2 mutation in NSCLC patients. Methods: We retrieved the clinical and genomic information of 1144 NSCLC from the Pan-Lung cancer dataset through the cBioportal ( www.cbioportal.org) . The cohorts of TSC1 and TSC2 mutant patients were identified. We compared baseline characteristics of patients with the Fisher exact test for categorical data and the Mann-Whitney U test for continuous variables. Overall survival (OS) was estimated with Kaplan-Meier curves, and differences were compared with the log-rank test. Results: Among 1144 patients, 27(2.36%) of them had TSC1 mutation and 40 (3.50%) had TSC2 mutation. Most patients with TSC1 and TSC2 mutations coexisted with other oncogenic gene alterations. TP53 was the most frequent concurrent gene (n = 53), followed with ERBB family genes (n = 24) and KRAS (n = 15). Compared to squamous cell carcinoma, TSC1/2 mutation was slightly more common in adenocarcinoma (53.7% vs 46.3%). 61.2% TSC1/2 mutant patients were male and 88.1% patients had former/current smoking history. Kaplan–Meier analysis showed that the patients harboring TSC1 mutation had a median OS of 14.1 months, whereas patients with TSC2 mutation had a median OS 110.6 months. However, there was not a statistically difference (P = 0.201). Conclusions: TSC1/2 mutation may define a unique population of NSCLC, which often coexists with other oncogenic gene alterations such as TP53 mutation. The function of TSC1/2 mutation and the value of TSC1/2 as therapeutic target in NSCLC are under investigation.
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Hoogeveen-Westerveld, Marianne, Leontine van Unen, Ans van den Ouweland, Dicky Halley, Andre Hoogeveen, and Mark Nellist. "The TSC1-TSC2 complex consists of multiple TSC1 and TSC2 subunits." BMC Biochemistry 13, no. 1 (2012): 18. http://dx.doi.org/10.1186/1471-2091-13-18.

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Gan, Boyi, Zara K. Melkoumian, Xiaoyang Wu, Kun-Liang Guan, and Jun-Lin Guan. "Identification of FIP200 interaction with the TSC1–TSC2 complex and its role in regulation of cell size control." Journal of Cell Biology 170, no. 3 (July 25, 2005): 379–89. http://dx.doi.org/10.1083/jcb.200411106.

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FIP200 (focal adhesion kinase [FAK] family interacting protein of 200 kD) is a newly identified protein that binds to the kinase domain of FAK and inhibits its kinase activity and associated cellular functions. Here, we identify an interaction between FIP200 and the TSC1–TSC2 complex through FIP200 binding to TSC1. We found that association of FIP200 with the TSC1–TSC2 complex correlated with its ability to increase cell size and up-regulate S6 kinase phosphorylation but was not involved in the regulation of cell cycle progression. Conversely, knockdown of endogenous FIP200 by RNA interference reduced S6 kinase phosphorylation and cell size, which required TSC1 but was independent of FAK. Furthermore, overexpression of FIP200 reduced TSC1–TSC2 complex formation, although knockdown of endogenous FIP200 by RNA interference did not affect TSC1–TSC2 complex formation. Lastly, we showed that FIP200 is important in nutrient stimulation-induced, but not energy- or serum-induced, S6 kinase activation. Together, these results suggest a cellular function of FIP200 in the regulation of cell size by interaction with the TSC1–TSC2 complex.
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Huang, Jingxiang, and Brendan D. Manning. "A complex interplay between Akt, TSC2 and the two mTOR complexes." Biochemical Society Transactions 37, no. 1 (January 20, 2009): 217–22. http://dx.doi.org/10.1042/bst0370217.

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Akt/PKB (protein kinase B) both regulates and is regulated by the TSC (tuberous sclerosis complex) 1–TSC2 complex. Downstream of PI3K (phosphoinositide 3-kinase), Akt phosphorylates TSC2 directly on multiple sites. Although the molecular mechanism is not well understood, these phosphorylation events relieve the inhibitory effects of the TSC1–TSC2 complex on Rheb and mTORC1 [mTOR (mammalian target of rapamycin) complex] 1, thereby activating mTORC1 in response to growth factors. Through negative-feedback mechanisms, mTORC1 activity inhibits growth factor stimulation of PI3K. This is particularly evident in cells and tumours lacking the TSC1–TSC2 complex, where Akt signalling is severely attenuated due, at least in part, to constitutive activation of mTORC1. An additional level of complexity in the relationship between Akt and the TSC1–TSC2 complex has recently been uncovered. The growth-factor-stimulated kinase activity of mTORC2 [also known as the mTOR–rictor (rapamycin-insensitive companion of mTOR) complex], which normally enhances Akt signalling by phosphorylating its hydrophobic motif (Ser473), was found to be defective in cells lacking the TSC1–TSC2 complex. This effect on mTORC2 can be separated from the inhibitory effects of the TSC1–TSC2 complex on Rheb and mTORC1. The present review discusses our current understanding of the increasingly complex functional interactions between Akt, the TSC1–TSC2 complex and mTOR, which are fundamentally important players in a large variety of human diseases.
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Huang, Jingxiang, Christian C. Dibble, Mika Matsuzaki, and Brendan D. Manning. "The TSC1-TSC2 Complex Is Required for Proper Activation of mTOR Complex 2." Molecular and Cellular Biology 28, no. 12 (April 14, 2008): 4104–15. http://dx.doi.org/10.1128/mcb.00289-08.

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ABSTRACT The mammalian target of rapamycin (mTOR) is a protein kinase that forms two functionally distinct complexes important for nutrient and growth factor signaling. Both complexes phosphorylate a hydrophobic motif on downstream protein kinases, which contributes to the activation of these kinases. mTOR complex 1 (mTORC1) phosphorylates S6K1, while mTORC2 phosphorylates Akt. The TSC1-TSC2 complex is a critical negative regulator of mTORC1. However, how mTORC2 is regulated and whether the TSC1-TSC2 complex is involved are unknown. We find that mTORC2 isolated from a variety of cells lacking a functional TSC1-TSC2 complex is impaired in its kinase activity toward Akt. Importantly, the defect in mTORC2 activity in these cells can be separated from effects on mTORC1 signaling and known feedback mechanisms affecting insulin receptor substrate-1 and phosphatidylinositol 3-kinase. Our data also suggest that the TSC1-TSC2 complex positively regulates mTORC2 in a manner independent of its GTPase-activating protein activity toward Rheb. Finally, we find that the TSC1-TSC2 complex can physically associate with mTORC2 but not mTORC1. These data demonstrate that the TSC1-TSC2 complex inhibits mTORC1 and activates mTORC2, which through different mechanisms promotes Akt activation.
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Bilanges, Benoit, Rhoda Argonza-Barrett, Marina Kolesnichenko, Christina Skinner, Manoj Nair, Michelle Chen, and David Stokoe. "Tuberous Sclerosis Complex Proteins 1 and 2 Control Serum-Dependent Translation in a TOP-Dependent and -Independent Manner." Molecular and Cellular Biology 27, no. 16 (June 11, 2007): 5746–64. http://dx.doi.org/10.1128/mcb.02136-06.

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ABSTRACT The tuberous sclerosis complex (TSC) proteins TSC1 and TSC2 regulate protein translation by inhibiting the serine/threonine kinase mTORC1 (for mammalian target of rapamycin complex 1). However, how TSC1 and TSC2 control overall protein synthesis and the translation of specific mRNAs in response to different mitogenic and nutritional stimuli is largely unknown. We show here that serum withdrawal inhibits mTORC1 signaling, causes disassembly of translation initiation complexes, and causes mRNA redistribution from polysomes to subpolysomes in wild-type mouse embryo fibroblasts (MEFs). In contrast, these responses are defective in Tsc1 −/− or Tsc2 −/− MEFs. Microarray analysis of polysome- and subpolysome-associated mRNAs uncovered specific mRNAs that are translationally regulated by serum, 90% of which are TSC1 and TSC2 dependent. Surprisingly, the mTORC1 inhibitor, rapamycin, abolished mTORC1 activity but only affected ∼40% of the serum-regulated mRNAs. Serum-dependent signaling through mTORC1 and polysome redistribution of global and individual mRNAs were restored upon re-expression of TSC1 and TSC2. Serum-responsive mRNAs that are sensitive to inhibition by rapamycin are highly enriched for terminal oligopyrimidine and for very short 5′ and 3′ untranslated regions. These data demonstrate that the TSC1/TSC2 complex regulates protein translation through mainly mTORC1-dependent mechanisms and implicates a discrete profile of deregulated mRNA translation in tuberous sclerosis pathology.
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Li, Yong, Ken Inoki, and Kun-Liang Guan. "Biochemical and Functional Characterizations of Small GTPase Rheb and TSC2 GAP Activity." Molecular and Cellular Biology 24, no. 18 (September 15, 2004): 7965–75. http://dx.doi.org/10.1128/mcb.24.18.7965-7975.2004.

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ABSTRACT Tuberous sclerosis complex (TSC) is a genetic disease caused by a mutation in either the tsc1 or tsc2 tumor suppressor gene. Recent studies have demonstrated that TSC2 displays GAP (GTPase-activating protein) activity specifically towards the small G protein Rheb and inhibits its ability to stimulate the mTOR signaling pathway. Rheb and TSC2 comprise a unique pair of GTPase and GAP, because Rheb has high basal GTP levels and TSC2 does not have the catalytic arginine finger found in Ras-GAP. To investigate the function of TSC2 and Rheb in mTOR signaling, we analyzed the TSC2-stimulated Rheb GTPase activity. We found that Arg15, a residue equivalent to Gly12 in Ras, is important for Rheb to function as a substrate for TSC2 GAP. In addition, we identified asparagine residues essential for TSC2 GAP activity. We demonstrated a novel catalytic mechanism of the TSC2 GAP and Rheb that TSC2 uses a catalytic “asparagine thumb” instead of the arginine finger found in Ras-GAP. Furthermore, we discovered that farnesylation and membrane localization of Rheb is not essential for Rheb to stimulate S6 kinase (S6K) phosphorylation. Analysis of TSC1 binding defective mutants of TSC2 shows that TSC1 is not required for the TSC2 GAP activity but may function as a regulatory component in the TSC1/TSC2 complex. Our data further demonstrate that GAP activity is essential for the cellular function of TSC2 to inhibit S6K phosphorylation.
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Matsumoto, Sanae, Amitabha Bandyopadhyay, David J. Kwiatkowski, Umadas Maitra, and Tomohiro Matsumoto. "Role of the Tsc1-Tsc2 Complex in Signaling and Transport Across the Cell Membrane in the Fission Yeast Schizosaccharomyces pombe." Genetics 161, no. 3 (July 1, 2002): 1053–63. http://dx.doi.org/10.1093/genetics/161.3.1053.

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Abstract Heterozygous inactivation of either human TSC1 or TSC2 causes tuberous sclerosis (TSC), in which development of benign tumors, hamartomas, occurs via a two-hit mechanism. In this study, fission yeast genes homologous to TSC1 and TSC2 were identified, and their protein products were shown to physically interact like the human gene products. Strains lacking tsc1+ or tsc2+ were defective in uptake of nutrients from the environment. An amino acid permease, which is normally positioned on the plasma membrane, aggregated in the cytoplasm or was confined in vacuole-like structures in Δtsc1 and Δtsc2 strains. Deletion of tsc1+ or tsc2+ also caused a defect in conjugation. When a limited number of the cells were mixed, they conjugated poorly. The conjugation efficiency was improved by increased cell density. Δtsc1 cells were not responsive to a mating pheromone, P-factor, suggesting that Tsc1 has an important role in the signal cascade for conjugation. These results indicate that the fission yeast Tsc1-Tsc2 complex plays a role in the regulation of protein trafficking and suggest a similar function for the human proteins. We also show that fission yeast Int6 is involved in a similar process, but functions in an independent genetic pathway.
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Dissertations / Theses on the topic "Tsc1, Tsc2"

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Azzi-Nogueira, Deborah. "Os produtos dos genes Tsc1 e Tsc2 em processos neurodegenerativos." Universidade de São Paulo, 2016. http://www.teses.usp.br/teses/disponiveis/41/41131/tde-09122016-154805/.

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O complexo da esclerose tuberosa (TSC) é uma doença genética que pode afetar órgãos específicos de qualquer sistema do organismo humano. Em geral, as lesões surgem pela inativação bialélica de um dos genes supressores tumorais Tuberous Sclerosis Complex 1 (TSC1) ou 2 (TSC2). Por outro lado, nas regiões corticais e subcorticais do cérebro, as lesões decorrentes de falhas de migração neuronal e sua arborização podem ser explicadas pela haploinsuficiência de TSC1 ou TSC2. As lesões do córtex cerebral apresentam-se comumente com epilepsia refratária, a qual, por sua vez, pode se associar a deficiência intelectual e transtornos do comportamento. Estes quadros clínicos podem estar presentes em pacientes com TSC sem lesão anatômica detectável à ressonância nuclear magnética do crânio. As proteínas hamartina ou tuberina, conhecidas também como TSC1 e TSC2, são codificadas respectivamente pelos genes TSC1 e TSC2. Elas agem juntas em um complexo molecular citosólico que inativa a pequena GTPase Rheb, a qual tem ação ativadora da cinase alvo da rapamicina em mamíferos (mTOR), regulando diversos processos celulares, como proliferação, diferenciação, crescimento, migração e metabolismo. Com a hipótese de que a quantidade de TSC1 ou TSC2 no neurônio pode alterar suas funções de forma dependente do estado metabólico, tivemos, neste trabalho, o objetivo geral de caracterizar os padrões de expressão e atividade de TSC1 e TSC2 em dois modelos de neurodegeneração induzida no camundongo adulto e verificar se a redução de quantidade de TSC1 tem efeito sobre a extensão da lesão de neurônios dopaminérgicos em modelo de hemiparkinsonismo. No primeiro modelo empregado, cinco estruturas encefálicas de camundongos submetidos a dieta hiperlipídica mostraram alteração da quantidade de RNAm de Tsc1 e/ou Tsc2 ou sinais de estresse oxidativo. A redução de transcritos de Tsc1 e Tsc2 no córtex cerebral foi dependente de jejum realizado imediatamente antes da eutanásia. No córtex cingulado, houve evidência de estresse oxidativo. O aumento específico de RNAm foi observado no hipocampo (Tsc1 e Tsc2) e no estriado e hipotálamo (Tsc1), embora de forma independente do jejum, sugerindo se tratar de alterações relacionadas à dieta hiperlipídica. No modelo de hemiparkinsonismo, camundongos adultos submetidos a injeção intracerebral de 6-hidroxidopamina apresentaram redução da quantidade total de proteína S6 no lado encefálico tratado quando comparado ao segmento contralateral (p =0,004, r=0,8795; teste de Pearson, IC: 95%), sem alteração de TSC1 ou TSC2. Em análises de imunoperoxidase do encéfalo, descrevemos, de forma independente da lesão, a expressão de TSC1 no estriado, núcleos entopeduncular e arqueado e de TSC2 no tálamo e hipotálamo. Com o objetivo de obter um modelo de camundongo sem expressão pós-natal de Tsc1 em várias regiões encefálicas, de forma independente do tipo celular, realizamos cruzamentos entre uma linhagem de camundongo transgênico em que o gene Tsc1 contém sequências lox nos íntrons 16 e 18 e outra linhagem com Tsc1 tipo-selvagem (WT) em homozigose e o transgene para expressão da recombinase Cre em fusão ao domínio de ligação ao ligante do receptor de estrógeno humano (ESR1) sob o controle de expressão do promotor de ubiquitina C (UBC). Em F1, obtivemos camundongos portadores do transgene UBC-CreESR1 e heterozigotos para Tsc1 (Tsc1WT/Flox). Em F2, entre os animais homozigotos Tsc1Flox/Flox (N = 153) gerados por retrocruzamento, nenhum era portador do transgene (Nesperado = 85; Nobservado = 0; X2 = 348,185; p < 0,0001) É possível que o segmento genômico em que houve inserção do vetor lentiviral que carrega o transgene UBC-CreESR1 esteja ligado ao loco de Tsc1 no cromossomo 2 do camundongo, segregando juntos. O tratamento com 4-hidroxitamoxifeno de animais heterozigotos e portadores do transgene aumentou a quantidade de TSC1 no estriado (p < 0,05) e o cerebelo não apresentou alteração. É possível que mecanismos transcricionais ou traducionais, funcionais no estriado, tenham favorecido o aumento de TSC1 de forma dependente de 4-hidroxitamoxifeno
Tuberous sclerosis complex (TSC) is a genetic disorder that can affect any specific organs. In general, lesions are caused by biallelic inactivation of the tumor suppressor genes Tuberous Sclerosis Complex 1 (TSC1) or 2 (TSC2). On the other hand, in cortical and subcortical brain regions, lesions associated with neuronal migration and arborization failures can be explained by TSC1 or TSC2 haploinsufficiency. Brain cortical lesions commonly cause refractory epilepsy, which, in turn, may be associated with intellectual disabilities and behavioral disorders. These medical conditions may be present in TSC patients without detectable anatomic lesion on magnetic resonance images. TSC1 and TSC2 genes encode hamartin and tuberin, also known as TSC1 and TSC2, respectively. They act together in a cytosolic molecular complex that inactivates small GTPase Rheb, which is a mammalian target of rapamycin (mTOR) activator, regulating diverse cellular processes such as proliferation, differentiation, growth, migration and metabolism. With the hypothesis that the amount of TSC1 or TSC2 in the neuron can change its function depending on the metabolic state, the overall objective of this study was to characterize TSC1 and TSC2 expression patterns and activity in two mice models of induced neurodegeneration; and check whether TSC1 reduction changes dopaminergic neurons damage extent in a hemiparkinsonins model. For the first model, five brain structures from mice fed with high fat diet showed alterations in Tsc1 and/or Tsc2 mRNA, or oxidative stress signals. Reduction of Tsc1 and Tsc2 transcripts in the cerebral cortex was dependent on fasting performed immediately prior to euthanasiaThere was evidence of oxidative stress in the cingulate cortex. Increase in mRNA was observed in the hippocampus (Tsc1 and Tsc2) and striatum and hypothalamus (Tsc1), although independent of the fasting, suggesting that this effect is related to the high fat diet. In hemiparkinsonism model, adult mice subjected to intracerebral injection of 6-hydroxydopamine had decreased levels of S6 in the brain treated side compared to the contralateral segment (p = 0.004, r = 0.8795; Pearson test, CI: 95 %), without alterations in TSC1 nor TSC2. Using imunoperoxidase analysis, we described TSC1 expression in the striatum, entopeduncular and arcuate nuclei, and TSC2 in the thalamus and hypothalamus, independently from the 6-OHDA lesion. To obtain a mouse model without TSC1 postnatal expression in different brain regions, independently of the cell type, we performed crosses between transgenic mouse strain in which the Tsc1 gene contains lox sequences in introns 16 and 18 and strain with Tsc1 wild-type (WT) and the transgene for expression of Cre recombinase fused to the binding domain of the human estrogen receptor (ESR1) ligand, controlled by ubiquitin C (UBC) promoter expression. In F1, we obtained mice carrying the transgene UBC-CreESR1 and heterozygous for Tsc1 (Tsc1WT/flox). In F2, among animals homozygous Tsc1Flox/Flox (N=153) generated by backcrossing, none was carrying the transgene (Nexpected = 85; Nobserved = 0; X2= 348.185, p <0.0001) It is possible that the genomic segment containing the lentiviral vector insertion bearing UBC-CreESR1 transgene is linked to the TSC1 region on mouse chromosome 2, and they segregate together. Treatment with 4-hydroxytamoxifen in animals heterozygous and positive for the transgene showed increased TSC1 in the striatum (p <0.05), while there was no change in the cerebellum. It is possible that transcriptional or translational functional striatum mechanisms favored TSC1 increasing, in a 4-hydroxytamoxifen-dependent manner
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Khatri, Shikha. "FOXO3a Regulates Glycolysis via Transcriptional Control of Tumor Suppressor TSC1." University of Cincinnati / OhioLINK, 2010. http://rave.ohiolink.edu/etdc/view?acc_num=ucin1282570293.

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Marín, Alexandra Belén Saona. "Capacidade proliferativa in vitro de precursores neuro-gliais, telencefálicos e expressão dos genes 1 e 2 do Complexo da Esclerose Tuberosa (TSC1 e TSC2)." Universidade de São Paulo, 2012. http://www.teses.usp.br/teses/disponiveis/41/41131/tde-08032013-105224/.

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O complexo da esclerose tuberosa (TSC) é um transtorno clínico, com expressividade variável, caracterizado por hamartomas que podem ocorrer em diferentes órgãos. Tem herança autossômica dominante e é devido a mutações em um de dois genes supressores de tumor, TSC1 ou TSC2. Estes codificam para as proteínas hamartina e tuberina, respectivamente, que se associam formando um complexo macromolecular que regula funções como proliferação, diferenciação, crescimento e migração celular. As lesões cerebrais podem ser muito graves em pacientes com TSC e caracterizam-se por nódulos subependimários (SEN), astrocitomas subependimários de células gigantes (SEGA), tuberosidades corticais e heterotopias neuronais, podendo relacionar-se clinicamente à epilepsia refratária à terapia medicamentosa, deficiência intelectual, desordens do comportamento e hidrocefalia. O potencial de crescimento de SEGA até os 21 anos de idade dos pacientes exige acompanhamento periódico por exame de imagem e condutas clínicas ou cirúrgicas, conforme indicação médica. As lesões subependimárias têm sido explicadas por déficits de controle da proliferação, crescimento e diferenciação de precursores neuro-gliais na zona subventricular telencefálica. Embora a capacidade da tuberina em inibir a proliferação celular pela repressão do alvo da rapamicina em mamíferos (mTOR) esteja bem documentada, outros aspectos celulares do desenvolvimento de SEGA ainda não foram examinados. Assim, é importante estabelecer um sistema in vitro para o estudo de células da zona subventricular e testá-lo na análise das proteínas hamartina e tuberina. Neste sentido, o cultivo de neuroesferas em suspensão é muito apropriado. Neste estudo, buscamos relacionar a expressão e distribuição subcelular da hamartina e tuberina à capacidade proliferativa e de diferenciação das células de neuroesferas cultivadas in vitro a partir da dissociação da vesícula telencefálica de embriões de ratos normais. Analisamos a expressão e distribuição subcelular da hamartina e tuberina por imunofluorescência indireta em células entre a primeira e a quarta passagens das neuroesferas, sincronizadas nas fases G1 ou S do ciclo celular e após a reentrada no ciclo celular, através da incorporação de 5-bromo-2\'-desoxiuridina (BrdU) e imunofluorescência com anticorpo anti-BrdU. Em geral, células de neuroesferas apresentaram baixa colocalização entre hamartina e tuberina in vitro. A expressão da tuberina foi elevada em basicamente todas as células das esferas e fases do ciclo celular; ao contrário, a hamartina apresentou-se principalmente nas células da periferia das esferas. A colocalização entre hamartina e tuberina foi observada em células mais periféricas das esferas, sobretudo no citoplasma e, em G1, no núcleo celular. A proteína rheb, que conhecidamente interage diretamente com a tuberina, apresentou distribuição subcelular muito semelhante à desta. Ao carenciamento das células visando à parada do ciclo celular na transição G1/S, tuberina distribuiu-se ao núcleo celular em quase todas as células avaliadas e, de forma menos frequente, a hamartina também. À reentrada no ciclo celular pelo reacréscimo dos fatores de crescimento, avaliaram-se células com incorporação de BrdU ao seu núcleo celular, após 72 e 96 horas. Nestas, tuberina mostrou-se novamente no citoplasma de forma preponderante e hamartina manteve-se citoplasmática, em geral subjacente à membrana plasmática, em níveis mais baixos. Os grupos cujas células reciclaram por 72 ou 96 horas diferiram quanto ao aumento significativo da expressão da hamartina em células proliferativas no último. À diferenciação neuronal, aumentaram-se os níveis de expressão de hamartina observáveis à imunofluorescência indireta, tornando-se equivalentes àqueles da tuberina. Concluímos que as células de neuroesferas cultivadas em suspensão apresentam-se como um sistema apropriado ao estudo da distribuição das proteínas hamartina e tuberina e sua relação com o ciclo celular
The tuberous sclerosis complex (TSC) is a clinical disorder with variable expressivity, characterized by hamartomas that can occur in different organs. It has autosomal dominant inheritance and is due to mutations in one of two tumor suppressor genes, TSC1 or TSC2. These encode for the proteins hamartin and tuberin, respectively, which are associated in a macromolecular complex which functions as a regulator of cell proliferation, differentiation, growth and migration. TSC brain lesions may be severe and are characterized by subependymal nodules (SEN), subependymal giant cell astrocytomas (SEGA), neuronal heterotopias and cortical tubers, and may be clinically related to refractory epilepsy, intellectual disability, behavioral disorders and hydrocephaly. The growth potential of SEGA up to 21 years of age in TSC patients requires regular monitoring by imaging. Clinical and surgical interventions may be medically indicated. Subependymal lesions have been explained by deficient control of proliferation, growth and differentiation of neuro-glial progenitors from the telencephalic subventricular zone. While tuberin ability to inhibit cell proliferation by repressing the mammalian target of rapamycin (mTOR) has been well documented, other cell aspects of SEGA development have not been thoroughly examined. Therefore, it is important to establish conditions for an in vitro system to study the cells from the subventricular zone and to test its suitability for the study of the TSC proteins. In this regard, the neurosphere suspension culture is very appropriate. We evaluated the expression and subcellular distribution of hamartin and tuberin in relation to the proliferation and differentiation capability of neurosphere cells derived in vitro from the dissociation of the telencephalic vesicle of normal E14 rat embryos. These analyses were performed by indirect immunofluorescence in cells from first through fourth passages of neurospheres, synchronized in G1 or S phases of the cell cycle, and after reentry into the cell cycle by the addition of 5-brome-2\'-desoxyuridine (BrdU) and immunolabeling with anti-BrdU antibody. In general, neurosphere cells presented low colocalization between hamartin and tuberin in vitro. Tuberin expression was relatively high in basically all neurosphere cells and cell cycle phases, whereas hamartin distributed mainly to cells from the periphery of the spheres. In these cells, hamartin and tuberin colocalization was evident mostly in the cytoplasm and, in G1, also in the cell nucleus. Rheb, which is known to interact directly with tuberin, had subcellular distribution very similar to tuberin. Cell starvation indicating cell cycle arrest at G1/S redistributed tuberin to the cell nucleus in virtually all cells examined, what was accompanied by nuclear location of hamartin in a small subset of cells. When cells were allowed to reenter cell cycle by adding growth factors, we evaluated BrdU-labeled nuclei 72 and 96 hours later. In the two groups, tuberin was shown to move back to the cytoplasm as well as hamartin, which apparently maintained its lower expression levels distribution underneath the plasma membrane. Group of cells that recycled for 96 hours had significantly more expression of hamartin than those cells that cycled for only 72 hours. After neuronal differentiation, hamartin expression levels observed by immunofluorescence were similar to those of tuberin. We conclude that neurosphere cells cultured in suspension showed to be an appropriate cell system to study hamartin and tuberin distribution in respect to the cell cycle
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Schmid, Maria [Verfasser], and Joachim-Ulrich [Akademischer Betreuer] Walther. "Genotyp-Phänotyp-Korrelation beim Tuberöse-Sklerose-Komplex : Auswertung der Mutationsanalyse von TSC1 und TSC2 aus der Diagnostik von TSC-Patienten und Vergleich unterschiedlicher Techniken / Maria Schmid ; Betreuer: Joachim-Ulrich Walther." München : Universitätsbibliothek der Ludwig-Maximilians-Universität, 2017. http://d-nb.info/1127527851/34.

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Schmid, Maria Anna Katharina [Verfasser], and Joachim-Ulrich [Akademischer Betreuer] Walther. "Genotyp-Phänotyp-Korrelation beim Tuberöse-Sklerose-Komplex : Auswertung der Mutationsanalyse von TSC1 und TSC2 aus der Diagnostik von TSC-Patienten und Vergleich unterschiedlicher Techniken / Maria Schmid ; Betreuer: Joachim-Ulrich Walther." München : Universitätsbibliothek der Ludwig-Maximilians-Universität, 2017. http://d-nb.info/1127527851/34.

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Alzhrani, Jasser Ali S. "Na+/K+ Pump and Cl--coupled Na+ and K+ co-transporters in Mouse Embryonic Fibroblasts lacking the Tuberous Sclerosis Complex TSC1 and TSC2 genes." Wright State University / OhioLINK, 2015. http://rave.ohiolink.edu/etdc/view?acc_num=wright1440683830.

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Zügge, Karin Louise. "Molecular genetic investigation of the variability of the GTPase activating protein- (GAP-) related domain of the tuberous sclerosis-2 (TSC2) gene in TSC patients and healthy subjects." [S.l.] : [s.n.], 2004. http://deposit.ddb.de/cgi-bin/dokserv?idn=972115366.

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Wilson, Catherine. "Molecular analysis of a Tsc1-deficient mouse." Thesis, Cardiff University, 2006. http://orca.cf.ac.uk/54274/.

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Tuberous sclerosis complex (TSC) is an autosomal dominant disorder caused by mutation in either the TSC1 or TSC2 genes and characterised by the development of benign hamartomatous growths in multiple organ systems. We have inactivated Tsd in the mouse germ line by gene targeting in ES cells and confirmed that the mutant allele (Tsc1) has a recessive embryonic lethal phenotype. Tsc1+, mice developed macroscopically visible renal lesions as early as 3-6 months. Renal lesions progressed from cysts through cystadenomas to solid carcinomas. Eighty percent of Tsc1+/ mice on a Balb/c background exhibited solid renal cell carcinomas (RCC) by 15-18 months and in 41%, RCCs were _5mm, resulting in grossly deformed kidneys. Some RCCs had a sarcomatoid morphology of spindle cells in whorled patterns and metastasised to the lungs. This new murine model of hamartin deficiency exhibits a more severe phenotype than existing models. A Bloom's deficient mouse model (Blmm3/m3) has been shown to induce colorectal tumourigenesis when crossed with Apc+/M,n mice. Here, we investigate whether the Blmm3/m3 genotype could induce tumourigenesis in extra-colonic tissues in Tsc1+/ mice that are predisposed to renal cystadenomas and carcinomas. Tsc1+/ B mm3,m3 mice had significantly more macroscopic and microscopic renal lesions at 3-6 months compared to Tsc1+' Blm+/m3 mice. Tsc1+, Blrrf3*"3 mice tumours showed significantly increased levels of somatic LOH of the wild type Tsd (Tsrf *) allele, as compared to those from Tsc1+, Blm+/+ mice. This work demonstrates the utility of the Blmm3/m3 mice for inducing renal tumourigenesis and the high levels ( 87%) of LOH in the resultant tumours will help facilitate mapping of loci involved in tumour progression. TSC1 and TSC2 are generally considered to act as tumour suppressors that fulfil Knudson's '2-hit hypothesis'. Here, we identified somatic Tsd mutations (2nd hits) in -80% of CAs and RCCs, but only 31.6% of cysts from Tsc1+/- mice, raising the possibility that haploinsufficiency for Tsd plays a role in cyst formation. Consistent with this proposal, many cysts showed little or no staining for phosphorylated mTOR and phosphorylated S6 ribosomal protein, whereas >90% of CAs and RCCs showed strong staining for both markers. We also sought somatic mutations in renal lesions from Tsc1+/ Blm'A mice that have a high frequency of somatic LOH, thereby facilitating the detection of 2nd hits. We also found significantly less somatic mutations in cysts, as compared to CAs and RCCs from these mice. Our data indicate that although activation of the mTOR pathway is an important step in Tsc-associated renal tumourigenesis, it may not be the key initiating event in this process.
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Hien, Annie. "Regulation of Translation and Synaptic Plasticity by TSC2." eScholarship@UMMS, 2020. https://escholarship.umassmed.edu/gsbs_diss/1097.

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Mutations in TSC2 cause the disorder tuberous sclerosis (TSC), which has a high incidence of autism and intellectual disability. TSC2 regulates mRNA translation required for group 1 metabotropic glutamate receptor-dependent synaptic long-term depression (mGluR-LTD), but the identity of mRNAs responsive to mGluR-LTD signaling in the normal and TSC brain is largely unknown. We generated Tsc2+/- mice to model TSC autism and performed ribosome profiling to identify differentially expressed genes following mGluR-LTD in the normal and Tsc2+/- hippocampus. Ribosome profiling reveals that in Tsc2+/-mice, RNA-binding targets of Fragile X Mental Retardation Protein (FMRP) are increased. In wild-type hippocampus, induction of mGluR-LTD caused rapid changes in the steady state levels of hundreds of mRNAs, many of which are FMRP targets. Moreover, mGluR-LTD signaling failed to promote phosphorylation of eukaryotic elongation factor 2 (eEF2) in Tsc2+/- mice, and chemically mimicking phospho-eEF2 with low cycloheximide enhances mGluR-LTD in the Tsc2+/- brain. These results suggest a molecular basis for bidirectional regulation of synaptic plasticity by TSC2 and FMRP. Furthermore, deficient mGluR-regulated translation elongation contributes to impaired synaptic plasticity in Tsc2+/- mice.
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Virdi, Simerjot Kaur. "Roles of the TSN1 and TSC2 Genes in Conferring Susceptibility of Durum Wheat to Tan Spot and Septoria Nodorum Blotch." Thesis, North Dakota State University, 2015. https://hdl.handle.net/10365/27628.

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Tan spot is an important disease caused by the necrotrophic fungus Pyrenophora triticirepentis. Two common necrotrophic effectors produced by this fungus are Ptr ToxA and Ptr ToxB, which recognize host sensitivity genes Tsn1 and Tsc2, respectively. In this research, a tetraploid recombinant inbred line population was evaluated for reaction to the Ptr ToxA and Ptr ToxB-producing isolates 86-124 (race 2) and DW5 (race 5). The results indicated that a compatible Tsc2-Ptr ToxB interaction accounted for 26% of the disease variation, which states that this interaction plays a significant role in the development of tan spot. On the contrary, the Tsn1-Ptr ToxA interaction was not associated with tan spot caused by 86-124. However, evaluation of a ToxA-producing isolate of Parastagonospora nodorum, indicated that the Tsn1- ToxA interaction accounted for 38% of the variation. Therefore, the Tsn1-ToxA interaction played a significant role in the development of septoria nodorum blotch, but not tan spot.
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Books on the topic "Tsc1, Tsc2"

1

Chrismon, Randolph L. The TSCA compliance handbook. New York, N.Y: Executive Enterprises Publications Co., 1989.

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Griffin, Ginger L. The TSCA compliance handbook. New York, N.Y: Executive Enterprises, 1994.

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Woodyard, John P. PCB management under TSCA. New York, N.Y: Executive Enterprises Publications Co., 1989.

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The TSCA compliance handbook. 3rd ed. New York: J. Wiley & Sons, 1996.

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Alfonso, Armada. TSC: Diario da noite. Vigo: Edicións Xerais de Galicia, 2009.

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TSC: Diario da noite. Vigo: Edicións Xerais de Galicia, 2009.

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Tume ya Utumishi wa Walimu (Tanzania). Ijue Tume ya Utumishi wa Walimu (TSC). 2nd ed. Dar es Salaam: Jamhuri ya Muungano wa Tanzania, Tume ya Utumishi wa Walimu, 2002.

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1950-, Thunder James M., ed. Federal chemical regulation: TSCA, EPCRA and the Pollution Prevention Act. Washington, D.C: Bureau of National Affairs, 1997.

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Fritz, Donald J. Nusa Tenggara Timur transport system consultancy project (TSCP): Final report. [Jakarta]: Direktorat Tata Kota dan Tata Daerah, Direktorat Jenderal Cipta Karya, Departemen Pekerjaan Umum, 1989.

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Wagner, Travis. The complete guide to the hazardous waste regulations: RCRA, TSCA, HMTA, OSHA, and Superfund. 3rd ed. New York: J. Wiley, 1999.

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Book chapters on the topic "Tsc1, Tsc2"

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Kajino, K., and O. Hino. "TSC1 and TSC2 Gene Mutations in Human Kidney Tumors." In Contributions to Nephrology, 45–50. Basel: KARGER, 1999. http://dx.doi.org/10.1159/000059974.

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Fischer, R. X., and W. H. Baur. "TSC." In Zeolite-Type Crystal Structures and their Chemistry. Framework Type Codes STO to ZON, 132–38. Berlin, Heidelberg: Springer Berlin Heidelberg, 2013. http://dx.doi.org/10.1007/978-3-642-32372-0_11.

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Raj, Satish R., S. R. Wayne Chen, Robert S. Sheldon, Arti N. Shah, Bharat K. Kantharia, Ulrich Salzer, Bodo Grimbacher, et al. "TSC." In Encyclopedia of Molecular Mechanisms of Disease, 2122. Berlin, Heidelberg: Springer Berlin Heidelberg, 2009. http://dx.doi.org/10.1007/978-3-540-29676-8_7534.

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Gooch, Jan W. "TSC." In Encyclopedic Dictionary of Polymers, 772. New York, NY: Springer New York, 2011. http://dx.doi.org/10.1007/978-1-4419-6247-8_12187.

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Harris, P. C. "The TSC2/PKD1 Contiguous Gene Syndrome." In Hereditary Kidney Diseases, 76–82. Basel: KARGER, 1997. http://dx.doi.org/10.1159/000059872.

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Campbell, Daniel, Corey Ray-Subramanian, Winifred Schultz-Krohn, Kristen M. Powers, Renee Watling, Christoph U. Correll, Stephanie Bendiske, et al. "TSC Tuberous Sclerosis." In Encyclopedia of Autism Spectrum Disorders, 3190. New York, NY: Springer New York, 2013. http://dx.doi.org/10.1007/978-1-4419-1698-3_101486.

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Donnelly, Shawn. "The TSCG and EMU reform." In Power Politics, Banking Union and EMU, 52–82. Abingdon, Oxon ; New York, NY : Routledge, 2018. | Series: Routledge/UACES contemporary European studies ; 41: Routledge, 2018. http://dx.doi.org/10.4324/9780203702130-3.

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Giassi, Lisa J., and John L. Gainer. "TSC and Hemorrhagic Shock." In Advances in Experimental Medicine and Biology, 55–60. Boston, MA: Springer US, 2003. http://dx.doi.org/10.1007/978-1-4757-6125-2_9.

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Jellinek, Steven D. "Information needs for TSCA and FIFRA." In Chemical Information, 79–91. Berlin, Heidelberg: Springer Berlin Heidelberg, 1989. http://dx.doi.org/10.1007/978-3-642-75165-3_10.

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Arand, M., E. Harwig, M. Schultheiß, and L. Kinzl. "Verlaufsbeurteilung nach operativer Therapie der traumatischen Spondylolisthesis C2 (TSC2)." In Hefte zur Zeitschrift „Der Unfallchirurg“, 218–19. Berlin, Heidelberg: Springer Berlin Heidelberg, 1997. http://dx.doi.org/10.1007/978-3-642-60913-8_83.

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Conference papers on the topic "Tsc1, Tsc2"

1

Goncharova, Elena A., Dmitry A. Goncharov, and Vera P. Krymskaya. "TSC1 And TSC2 Differentially Modulate Actin Cytoskeleton And Motility." In American Thoracic Society 2010 International Conference, May 14-19, 2010 • New Orleans. American Thoracic Society, 2010. http://dx.doi.org/10.1164/ajrccm-conference.2010.181.1_meetingabstracts.a2092.

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Goncharova, Elena A., Dmitry A. Goncharov, Irene Khavin, Okio Hino, and Vera P. Krymskaya. "Pulmonary Lymphangioleiomyomatosis (LAM): TSC1/TSC2 Modulates E-Cadherin Localization Through Small GTPase Rac1." In American Thoracic Society 2010 International Conference, May 14-19, 2010 • New Orleans. American Thoracic Society, 2010. http://dx.doi.org/10.1164/ajrccm-conference.2010.181.1_meetingabstracts.a2091.

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Palma, Norma A., Juliann Chmielecki, Garrett Frampton, Siraj Ali, Maren Levin, Jeffrey S. Ross, Deborah Morosini, et al. "Abstract P2-03-06: FoundationOne profiling of TSC1 and TSC2-mutated advanced breast cancers." In Thirty-Seventh Annual CTRC-AACR San Antonio Breast Cancer Symposium; December 9-13, 2014; San Antonio, TX. American Association for Cancer Research, 2015. http://dx.doi.org/10.1158/1538-7445.sabcs14-p2-03-06.

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Kang, Ju-Yeon, Youn-Young Jang, Nam-Su Huh, Ki-Seok Kim, and Woo-Yeon Cho. "Limit Strains of X70 Pipes With a Semi-Elliptical Crack Based on Initiation and Ductile Tearing Criteria." In ASME 2018 Pressure Vessels and Piping Conference. American Society of Mechanical Engineers, 2018. http://dx.doi.org/10.1115/pvp2018-84641.

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Crack-tip opening displacement (CTOD) and J-integral have been used for elastic-plastic fracture parameters as a crack driving force (CDF) and crack resistance curve to evaluate tensile strain capacity (TSC) of cracked pipelines based on strain-based design (SBD). The TSC can be determined by using two kinds of failure criteria. One is based on the limit state corresponding to an onset of stable crack growth and the other is tangency approach which determines an onset of unstable crack growth by comparing crack driving force and resistance curve. For this reason, the accurate calculation of crack driving force and crack resistance curve is highly required to determine TSC. In the present study, the TSCs for X70 pipelines with a circumferential semi-elliptical surface crack were estimated based on both crack initiation and ductile tearing criteria using crack driving force diagram (CDFD) method. The CDF curves of cracked pipelines were calculated through the detailed elastic-plastic finite element (FE) analyses. Crack resistance curves were obtained from experimental data of single edged notch tension (SENT) specimens. Both the CDF and crack resistance curves were represented using CTOD and J-integral, respectively. As for loading conditions, axial strain and internal pressure were considered. The TSCs based on CTOD were compared with those based on J-integral to investigate the effect of choice of the fracture parameters on TSC. From the FE results, the TSCs based on ductile tearing allowed higher TSCs than those based on crack initiation. Although there were some differences between the TSCs using CTOD and J-integral, the effect of choice of fracture parameter on TSC with internal pressure was not significant.
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Moon, Ji-Hee, Nam-Su Huh, and Ki-Seok Kim. "Investigation Into Applications of Local Failure Criterion for X70 Pipeline With Corrosion Defect." In ASME 2018 Pressure Vessels and Piping Conference. American Society of Mechanical Engineers, 2018. http://dx.doi.org/10.1115/pvp2018-84566.

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In this paper, the local failure criterion using stress modified critical strain method based on annex B of API 579 is applied to evaluate the ductile failure of API X70 pipelines with a volumetric corrosion defect. Ductile failure is quantified in terms of strain, representing the tensile strain capacity (TSC) which is commonly used in strain-based assessment for fitness-for-service of pipelines installed in frozen area where large-scale ground movement can arise due to earthquakes, freezing and thawing. Based on the local failure criterion suggested for API X70 steel material, the TSCs of the corroded pipelines are evaluated by using the detailed finite element (FE) analyses. The effects of internal pressure and defect size (such as longitudinal length, circumferential width and depth in the direction of thickness) on TSC of pipelines subjected to axial displacement are systematically investigated. In addition, TSCs based on local failure criterion are compared with those based on net-section limit load. The TSCs from the present FE analyses for various defect geometries and internal pressure can be used to predict ductile failure of corroded pipelines and to build the framework for a strain-based assessment for in-service pipelines.
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Lafont, J., J. H. Catherine, M. Lejeune, U. Ordioni, R. Lan, and F. Campana. "Manifestations buccales de la sclérose tubéreuse de Bourneville." In 66ème Congrès de la SFCO. Les Ulis, France: EDP Sciences, 2020. http://dx.doi.org/10.1051/sfco/20206603014.

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L’objectif de ce travail est de faire le point sur les manifestations buccales de la sclérose tubéreuse de Bourneville (STB) à travers le cas d’un jeune patient. Un jeune homme de 15 ans était adressée pour la mise en place de minivis orthodontique afin de fermer des espaces d’agénésies de 35 et 45. L’interrogatoire retrouvait une STB dont les manifestations épileptiques étaient traitées par de la lamotrigine 75mg/j et de la carbamazépine LP 200mg/j. L’examen clinique exo-buccal retrouvait des macules hypochromiques sur le membre inférieur droit, des angiofibromes faciaux et une malformation vasculaire jugale gauche. L’examen endo-buccal retrouvait de multiples lésions buccales sur les papilles interdentaires pouvant évoquer des fibromes ou des hamartomes. Une biopsie était réalisée et retrouvait un revêtement malpighien, discrètement hyperplasique et sans atypie cellulaire. Les faisceaux collagènes du conjonctif étaient mêlés à de nombreux fibroblastes aux noyaux réguliers, sans mitose visible. Les cellules inflammatoires, essentiellement mononuclées, étaient dispersées mais tendaient à se regrouper autour de vaisseaux nombreux et hyperplasiques. L’examen concluait à un fibrome. Aucun traitement buccal n’était proposé devant l’absence de symptôme et de demande esthétique. La STB est une maladie génétique autosomique dominante avec une incidence de 1/10 000. Elle est liée à une mutation du gène TSC1 sur le chromosome 9 ou du gène TSC2 sur le chromosome 16 qui perturbe la sécrétion d’une protéine régulant la voie mTOR. C’est une maladie multisystème avec une expression clinique variable. Les principaux symptômes sont l’épilepsie, le retard mental et la présence d’adénomes sébacés, mais la maladie est associée à un polymorphisme clinique rendant le diagnostic difficile. La conférence de consensus de 2012 a ainsi défini des critères diagnostiques majeurs (lésions cutanées, oculaires, cérébrales, cardiaques, pulmonaires, rénales,..) et mineurs dont deux sont bucco-dentaires. Le diagnostic est retenu devant deux critères majeurs ou un critères majeur et deux critères mineurs. Les signes oraux sont la présence de trois ou plus puits d’émail et deux ou plus fibromes gingivaux. Les fibromes gingivaux atteindraient 50 à 70% des patients. La région antérieure maxillaire semble la plus touchée. L’exérèse est indiquée en cas de gêne esthétique ou de saignements associés. Actuellement, les inhibiteurs de mTOR représentent une option thérapeutique proposée dans la prise en charge des patients atteints de STB. La STB est une pathologie rare. La présence de lésions buccales fait partie des critères diagnostiques.
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Liu, Ming, Yong-Yi Wang, David Horsley, and Steve Nanney. "Multi-Tier Tensile Strain Models for Strain-Based Design: Part 3 — Model Evaluation Against Experimental Data." In 2012 9th International Pipeline Conference. American Society of Mechanical Engineers, 2012. http://dx.doi.org/10.1115/ipc2012-90660.

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This is the third paper in a three-paper series related to the development of tensile strain models. The fundamental basis [1] and formulation [2] of the models are presented in two companion papers. This paper covers the evaluation of the models against large-scale experimental data which include a total of 24 full-scale pipe tests with and without internal pressure [3,4] and 30 curved wide plate (CWP) tests [5,6]. The 24 full-scale pipe specimens are nominally X65 grade (12.75″ OD and 12.7-mm wall thickness) and made by two manufacturers. The actual yield strength of the two pipes differs by approximately 14 ksi. The girth welds are made with three welding procedures, creating three weld strength levels. The full-scale test program are designed to evaluate the effects of internal pressure, weld strength mismatch, pipe strength, pipe Y/T ratio, flaw location, flaw size, and toughness. The 30 CWP specimens are from 36″ OD and 19.1 mm wall thickness X100 pipes. The girth welds are made with two welding procedures, creating two slightly different weld strength mismatch levels. The CWP test specimens expand the range of material grade and wall thickness for the model evaluation. The model evaluation demonstrates that the overall correlations between the experimental test data and model predations are similar when the model predictions are made with Level 2 and 3 procedures and various toughness options. The Level 2 procedure with Charpy energy option and Level 3b provide the best overall one-to-one correlation between the test data and model prediction. The Level 3b shows greater scatter than Level 2 with the Charpy energy option. The most significant contributor to the TSC variations and the difference between the measured and predicted TSCs is the strength variation in the pipes. A small variation in the strength can lead to a large variation of the measured remote strain even when the flaw behavior is essentially the same. For the 24 full-scale pipe tests, a strength variation of 1 ksi in the pipes would explain the large variations of the measured TSC in comparison to the model predictions. The TSC models produce consistent results that capture the overall trend of the test data.
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8

Hahm, Oliver, Cédric Adjih, Emmanuel Baccelli, Thomas C. Schmidt, and Matthias Wählisch. "ICN over TSCH." In ICN'16: 3rd International Conference on Information-Centric Networking. New York, NY, USA: ACM, 2016. http://dx.doi.org/10.1145/2984356.2985226.

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9

van der Lee, Tim, Antonio Liotta, and Georgios Exarchakos. "TSCH schedules assessment." In 2017 IEEE 14th International Conference on Networking, Sensing and Control (ICNSC). IEEE, 2017. http://dx.doi.org/10.1109/icnsc.2017.8000175.

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10

Zorbas, Dimitrios, Panayiotis Kotzanikolaou, and Christos Douligeris. "R-TSCH: Proactive Jamming Attack Protection for IEEE 802.15.4-TSCH Networks." In 2018 IEEE Symposium on Computers and Communications (ISCC). IEEE, 2018. http://dx.doi.org/10.1109/iscc.2018.8538705.

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Reports on the topic "Tsc1, Tsc2"

1

Nellist, Mark, Marianne Hoogeveen-Westerveld, and Dicky Halley. Biochemical Characterisation of TSC1 and TSC2 Variants Identifiedd in Patients with Tuberous sclerosis Complex. Fort Belvoir, VA: Defense Technical Information Center, July 2008. http://dx.doi.org/10.21236/ada622174.

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2

Nellist, Mark, Marianne Hoogeveen-Westerveld, and Dicky Halley. Biochemical Characterisation of TSC1 and TSC2 Variants Identified in Patients with Tuberous Sclerosis Complex. Fort Belvoir, VA: Defense Technical Information Center, July 2010. http://dx.doi.org/10.21236/ada624706.

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3

Guan, Kun-Liang. Regulation of TS1/TSC2 Stability and Rheb GTP Level by Herc1. Fort Belvoir, VA: Defense Technical Information Center, January 2006. http://dx.doi.org/10.21236/ada470087.

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4

Ess, Kevin. Neural Development in tsc2-Deficient Zebrafish. Fort Belvoir, VA: Defense Technical Information Center, October 2011. http://dx.doi.org/10.21236/ada590191.

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5

Walker, Cheryl. TSC2 Happloinsufficiency Leads to a Mutator Phenotype. Fort Belvoir, VA: Defense Technical Information Center, November 2007. http://dx.doi.org/10.21236/ada481229.

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Smith, L. L. Technical evaluation of the TSCA Ambient Air Monitoring Program. [Toxic Substances Control Act (TSCA)]. Office of Scientific and Technical Information (OSTI), June 1992. http://dx.doi.org/10.2172/5122074.

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D. L. Layton. Toxic Substances Control Act (TSCA) Polychlorinate. Office of Scientific and Technical Information (OSTI), June 2013. http://dx.doi.org/10.2172/1084682.

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Wang, Lizhong. Synergistic Action of FOXP3 and TSC1 Pathways During Tumor Progression. Fort Belvoir, VA: Defense Technical Information Center, October 2015. http://dx.doi.org/10.21236/ada625959.

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Zervas, Mark. Temporal Loss of Tsc1: Neural Development and Brain Disease in Tuberous Sclerosis. Fort Belvoir, VA: Defense Technical Information Center, June 2014. http://dx.doi.org/10.21236/ada609442.

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Zervas, Mark. Temporal Loss of Tsc1: Neural Development and Brain Disease in Tuberous Sclerosis. Fort Belvoir, VA: Defense Technical Information Center, June 2013. http://dx.doi.org/10.21236/ada584730.

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