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品种反应性 主要应用 宿主 格式 抗体类型 R, H WB M Ascites Monoclonal Antibody 描述 产品目录编号 05-1113 Replaces 04-426 描述 Anti-Harmartin (TSC1) Antibody Alternate Names - tumor suppressor
- Tuberous sclerosis 1 protein
- tuberous sclerosis 1
背景信息 Harmartin is the gene product of tuberous sclerosis-1 (TSC1). It is thought to have a tumor suppression function and also augments the expression of the TSC2 product tuberin by inhibiting its ubiquitination. 产品信息 格式 Ascites 控制 - NIH/3T3 cell lysate
演示 Ascites fluid. Liquid. Contains no preservative. 应用 应用 This Anti-Harmartin (TSC1) Antibody is validated for use in WB, IH(P) for the detection of Harmartin (TSC1). 主要应用 - Western Blotting
应用说明 Immunohistochemistry Analysis: A 1:100 dilution of this antibody detected Harmartin in human skin tissue, melanocytes. 生物信息 免疫原品种 Recombinant protein from human Harmartin 宿主 Mouse 特异性 This antibody recognizes Harmartin 同种型 IgG1 品种反应性 RatHuman Species Reactivity Note Human, rat 抗体类型 Monoclonal Antibody Entrez基因编号 - Q92574
基因符号 - hamartin
- KIAA0243
- LAM
- MGC86987
- OTTHUMP00000022439
- TSC
纯化方法 Unpurified ascites fluid UniProt编号 - Q92574
UniProt汇总 FUNCTION: In complex with TSC2, inhibits the nutrient-mediated or growth factor-stimulated phosphorylation of S6K1 and EIF4EBP1 by negatively regulating mTORC1 signaling. Seems not to be required for TSC2 GAP activity towards RHEB. Implicated as a tumor suppressor. Involved in microtubule-mediated protein transport, but this seems to be due to unregulated mTOR signaling.
SUBUNIT STRUCTURE: Interacts with TSC2, leading to stabilize TSC2. In the absence of TSC2, TSC1 self-aggregates. Interacts with DOCK7. SUBCELLULAR LOCATION: Cytoplasm. Membrane; Peripheral membrane protein. Note: At steady state found in association with membranes.
TISSUE SPECIFICITY: Highly expressed in skeletal muscle, followed by heart, brain, placenta, pancreas, lung, liver and kidney. Also expressed in embryonic kidney cells.
DOMAIN: The C-terminal putative coiled-coil domain is necessary for interaction with TSC2.
PTM: Phosphorylation at Ser-505 does not affect interaction with TSC2. Phosphorylated upon DNA damage, probably by ATM or ATR.
INVOLVEMENT IN DISEASE: Defects in TSC1 are the cause of tuberous sclerosis complex (TSC) [MIM:191100]. The molecular basis of TSC is a functional impairment of the hamartin-tuberin complex. TSC is an autosomal dominant multi-system disorder that affects especially the brain, kidneys, heart, and skin. TSC is characterized by hamartomas (benign overgrowths predominantly of a cell or tissue type that occurs normally in the organ) and hamartias (developmental abnormalities of tissue combination). Clinical symptoms can range from benign hypopigmented macules of the skin to profound mental retardation with intractable seizures to premature death from a variety of disease-associated causes.
Defects in TSC1 may be a cause of focal cortical dysplasia of Taylor balloon cell type (FCDBC) [MIM:607341]. FCDBC is a subtype of cortical displasias linked to chronic intractable epilepsy. Cortical dysplasias display a broad spectrum of structural changes, which appear to result from changes in proliferation, migration, differentiation, and apoptosis of neuronal precursors and neurons during cortical development.产品使用声明 质量保证 Evaluated by Western Blot in NIH/3T3 cell lysate.
Western Blot Analysis: 1:500 dilution of this antibody detected Harmartin in 10 µg of NIH/3T3 cell lysate.使用声明 - Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
储存和货运信息 存储条件 Maintain at -20°C in undiluted aliquots for up to 12 months after date of receipt. Avoid repeated freeze/thaw cycles. 包装信息 数量 100 µL
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