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tuberous sclerosis/proline

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Tuberous sclerosis: aberrant metabolism of ornithine, proline and glutamate in cultured fibroblasts.

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To investigate aberrant metabolism of proline (Pro) and its precursors in tuberous sclerosis (TS), 6, 7 and 5 strains of control, TS (normal skin) and TS (tumor) fibroblasts, respectively, were cultured in Eagle's MEM containing dialyzed fetal bovine serum with or without 0.1 mM ornithine (Orn).

Tuberous sclerosis: aberrant proline sensitivity of skin fibroblasts.

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Cultured skin fibroblasts from patients with tuberous sclerosis were analyzed as to their sensitivities to proline, glutamic acid and arginine, and their analogues by means of a cell growth study. A difference was observed between tuberous sclerosis and normal cells when proline was added. Skin

Tuberous sclerosis: proline and hydroxyproline contents in serum.

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The serum levels of proline and hydroxyproline were determined by high-performance liquid chromatography using the post-labeled method with o-phthalaldehyde and sodium hypochlorite in 30 patients of tuberous sclerosis (TS) and compared with those in 32 pathological control subjects of similar age.

Somatic mutation of the tuberous sclerosis (Tsc2) tumor suppressor gene in chemically induced rat renal carcinoma cell.

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OBJECTIVE von Hippel-Lindau (VHL) gene mutations are detected in noninherited, sporadic human renal cell carcinomas (RCs) at a high frequency. We recently identified a germline mutation in the rat homologue of the human tuberous sclerosis (TSC2) predisposing RC gene in the Eker rat model, and in

The proline-rich Akt substrate of 40 kDa (PRAS40) is a physiological substrate of mammalian target of rapamycin complex 1.

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The proline-rich Akt substrate of 40 kilodaltons (PRAS40) was identified as a raptor-binding protein that is phosphorylated directly by mammalian target of rapamycin (mTOR) complex 1 (mTORC1) but not mTORC2 in vitro, predominantly at PRAS40 (Ser(183)). The binding of S6K1 and 4E-BP1 to raptor

Chemical analysis of an angiofibroma from a patient with tuberous sclerosis.

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Amino-acid analyses on the acid hydrolysates of an angiofibroma and skin established that the former tissue contained less collagen than skin based on the reduced content of hydroxyproline, glycine, proline and alanine in the tumour. From lysosomal enzyme measurements it became evident that the

Taking aim at Alzheimer's disease through the mammalian target of rapamycin.

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A significant portion of the world's population suffers from sporadic Alzheimer's disease (AD) with available present therapies limited to symptomatic care that does not alter disease progression. Over the next decade, advancing age of the global population will dramatically increase the incidence

Tuberin and PRAS40 are anti-apoptotic gatekeepers during early human amniotic fluid stem-cell differentiation.

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Embryoid bodies (EBs) are three-dimensional multicellular aggregates allowing the in vitro investigation of stem-cell differentiation processes mimicking early embryogenesis. Human amniotic fluid stem (AFS) cells harbor high proliferation potential, do not raise the ethical issues of embryonic stem

Stimulation of muscle protein synthesis by prolonged parenteral infusion of leucine is dependent on amino acid availability in neonatal pigs.

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The postprandial rise in amino acids, particularly leucine, stimulates muscle protein synthesis in neonates. Previously, we showed that a 1-h infusion of leucine increased protein synthesis, but this response was not sustained for 2 h unless the leucine-induced decrease in amino acids was prevented.

IGF-1 stimulates protein synthesis by enhanced signaling through mTORC1 in bovine mammary epithelial cells.

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Using the MAC-T cell line as a model, the effects of insulin-like growth factor (IGF)-1 on the regulation of protein synthesis through the mammalian target of rapamycin complex 1 (mTORC1) signaling in bovine mammary epithelial cells were evaluated. Global rates of protein synthesis increased by 47%

Research resource: identification of novel growth hormone-regulated phosphorylation sites by quantitative phosphoproteomics.

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GH and GH receptors are expressed throughout life, and GH elicits a diverse range of responses, including growth and altered metabolism. It is therefore important to understand the full spectrum of GH signaling pathways and cellular responses. We applied mass spectrometry-based phosphoproteomics

Knockdown of PRAS40 inhibits insulin action via proteasome-mediated degradation of IRS1 in primary human skeletal muscle cells.

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OBJECTIVE The proline-rich Akt substrate of 40 kDa (PRAS40) is a component of the mammalian target of rapamycin complex 1 (mTORC1) and among the most prominent Akt substrates in skeletal muscle. Yet the cellular functions of PRAS40 are incompletely defined. This study assessed the function of PRAS40

PTEN inhibitor VO-OHpic suppresses TSC2-/- MEFs proliferation by excessively inhibiting autophagy via the PTEN/PRAS40 pathway.

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Tuberous sclerosis complex (TSC) is a relatively rare autosomal dominant disease which involves multiple organs, including the brain, kidney, lung, skin and heart. Renal angiomyolipomas (RAML) are the main causes of mortality in patients with TSC. The preferred treatment for RAML is the use of mTOR

Identification of novel immune phenotypes for allergic and nonallergic childhood asthma.

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BACKGROUND Childhood asthma is classified into allergic asthma (AA) and nonallergic asthma (NA), yet both are treated identically, with only partial success. OBJECTIVE We sought to identify novel immune phenotypes for childhood AA and NA. METHODS The Clinical Asthma Research Association cohort study

Intermittent bolus feeding has a greater stimulatory effect on protein synthesis in skeletal muscle than continuous feeding in neonatal pigs.

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Orogastric tube feeding, using either continuous or intermittent bolus delivery, is common in infants for whom normal feeding is contraindicated. To compare the impact of different feeding strategies on muscle protein synthesis, after withholding food overnight, neonatal pigs received a complete
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