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merck millipore,默克密理博,04-427,Anti-TSC2 Antibody, rabbit monoclonal
产品名称：Anti-TSC2 Antibody, rabbit monoclonal
产品型号：04-427
Please note that this product will not be available for sale after March 15, 2015. Please select one of the other antibodies against this target. This Anti-TSC2 Antibody, rabbit is validated for use More>>
merck millipore,默克密理博,04-427,Anti-TSC2 Antibody, rabbit monoclonal
- 产品介绍
重要规格表
品种反应性 主要应用 宿主 格式 抗体类型 R, H, M WB, FC Rb Semi-Purified Monoclonal Antibody 描述 产品目录编号 04-427 品牌系列 Upstate
商名 - Upstate
描述 Anti-TSC2 Antibody, rabbit monoclonal Alternate Names - tuberous sclerosis 2, tuberin
- tuberous sclerosis 2
产品信息 格式 Semi-Purified 应用 应用 Please note that this product will not be available for sale after March 15, 2015. Please select one of the other antibodies against this target. This Anti-TSC2 Antibody, rabbit is validated for use in FC, WB, IH(P) for the detection of TSC2. 主要应用 - Western Blotting
- Flow Cytometry
生物信息 免疫原品种 KLH-conjugated synthetic peptide corresponding to the C-terminal region of human TSC2/Tuberin. 表位 C-terminal domain 宿主 Rabbit 特异性 Recognizes the C-terminal domain of TSC2. 同种型 IgG 品种反应性 RatHumanMouse 抗体类型 Monoclonal Antibody Entrez基因编号 - NM_001077183.1
- NM_021055.1
- NM_021056.1
- NM_000548.3
Entrez基因汇总 Mutations in this gene lead to tuberous sclerosis complex. Its gene product is believed to be a tumor suppressor and is able to stimulate specific GTPases. The protein associates with hamartin in a cytosolic complex, possibly acting as a chaperone for hamartin. Alternative splicing results in multiple transcript variants encoding different isoforms. 基因符号 - Tuberin
- TSC2
- TSC4
- FLJ43106
- tuberin
- LAM
UniProt编号 - P49815
UniProt汇总 FUNCTION: SwissProt: P49815 # Implicated as a tumor suppressor. May have a function in vesicular transport, but may also play a role in the regulation of cell growth arrest and in the regulation of transcription mediated by steroid receptors. Interaction between TSC1 and TSC2 may facilitate vesicular docking. Specifically stimulates the intrinsic GTPase activity of the Ras-related protein RAP1A and RAB5. Suggesting a possible mechanism for its role in regulating cellular growth. Mutations in TSC2 leads to constitutive activation of RAP1A in tumors.
SIZE: 1807 amino acids; 200749 Da
SUBUNIT: Interacts with TSC1. May also interact with the adapter molecule RABEP1. The final complex contains TSC2 and RABEP1 linked to RAB5 (Probable). Interacts with HSPA1 and HSPA8.
SUBCELLULAR LOCATION: Cytoplasm. Membrane; Peripheral membrane protein. Note=At steady state found in association with membranes.
TISSUE SPECIFICITY: Liver, brain, heart, lymphocytes, fibroblasts, biliary epithelium, pancreas, skeletal muscle, kidney, lung and placenta.
PTM: Phosphorylation at Ser-1387, Ser-1418 or Ser-1420 does not affect interaction with TSC1.
DISEASE: SwissProt: P49815 # Defects in TSC2 are the cause of tuberous sclerosis complex (TSC) [MIM:191100]. The molecular basis of TSC is a functional impairment of the tuberin-hamartin 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 TSC2 are a cause of lymphangioleiomyomatosis (LAM) [MIM:606690]. LAM is a progressive and often fatal lung disease characterized by a diffuse proliferation of abnormal smooth muscle cells in the lungs. It affects almost exclusively young women and can occur as an isolated disorder or in association with tuberous sclerosis complex.
SIMILARITY: SwissProt: P49815 ## Contains 1 Rap-GAP domain.产品使用声明 质量保证 Routinely evaluated by immunoblot. 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 2 years at -20°C from date of shipment 包装信息 数量 100 µL
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