产品货号 :  mlR3441

英文名称 :  Phospho-Tuberin (Ser1387)

中文名称 :  磷酸化马铃薯球蛋白(结节性硬化)抗体

别    名 :  Tuberin(phospho S1387); Tuberin (phospho Ser1387); p-Tuberin (Ser1387); Phospho-TSC2 (Ser1387); P-TSC2 (Ser1387);FLJ43106; LAM; OTTHUMP00000158940; OTTHUMP00000198394; OTTHUMP00000198395; TSC2; TSC2_HUMAN; TSC4; Tuberin; Tuberous sclerosis 2; Tuberous sclerosis 2 protein; Tuberous sclerosis 2 protein homolog.  

产品类型 :  磷酸化抗体

研究领域 :  肿瘤  免疫学  转录调节因子  

抗体来源 :  Rabbit

克隆类型 :  Polyclonal

交叉反应  :  Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit,

产品应用 :  WB=1:500-2000 ELISA=1:500-1000 IHC-F=1:400-800 IF=1:100-500 （石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.

分 子 量 :  200kDa

细胞定位 :  细胞浆 细胞膜

性    状 :  Lyophilized or Liquid

浓    度 :  1mg/ml

免 疫 原 :  KLH conjugated Synthesised phosphopeptide derived from human Tuberin around the phosphorylation site of Ser1387:SS(p-S)SP

亚    型 :  IgG

纯化方法 :  affinity purified by Protein A

储 存 液 :  0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

保存条件 :  Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.

PubMed :  PubMed

产品介绍  :  Tuberin, or TSC2 (Tuberous sclerosis complex), is implicated as a tumor suppressor. It may function in vesicular transport, and may also play a role in the regulation of cell growth arrest and in the regulation of transcription mediated by steroid receptors. Interaction between hamartin (TSC1) and tuberin may facilitate vesicular docking. It 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 tuberin lead to constitutive activation of RAP1A in tumors. At least three isoforms of Tuberin exist.

Function:

In complex with TSC1, inhibits the nutrient-mediated or growth factor-stimulated phosphorylation of S6K1 and EIF4EBP1 by negatively regulating mTORC1 signaling. Acts as a GTPase-activating protein (GAP) for the small GTPase RHEB, a direct activator of the protein kinase activity of mTORC1. Implicated as a tumor suppressor. Involved in microtubule-mediated protein transport, but this seems to be due to unregulated mTOR signaling. Stimulates weakly the intrinsic GTPase activity of the Ras-related proteins RAP1A and RAB5 in vitro. Mutations in TSC2 lead to constitutive activation of RAP1A in tumors.

Subunit:

Interacts with TSC1 and HERC1; the interaction with TSC1 stabilizes TSC2 and prevents the interaction with HERC1. May also interact with the adapter molecule RABEP1. The final complex contains TSC2 and RABEP1 linked to RAB5 (Probable). Interacts with HSPA1 and HSPA8. Interacts with DAPK1.

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.

Post-translational modifications:

Phosphorylation at Ser-1387, Ser-1418 or Ser-1420 does not affect interaction with TSC1. Phosphorylation at Ser-939 and Thr-1462 by PKB/AKT1 is induced by growth factor stimulation. Phosphorylation by AMPK activates it and leads to negatively regulates the mTORC1 complex. Phosphorylated at Ser-1798 by RPS6KA1; phosphorylation inhibits TSC2 ability to suppress mTORC1 signaling. Phosphorylated by DAPK1.

DISEASE:

Defects in TSC2 are the cause of tuberous sclerosis type 2 (TSC2) [MIM:613254]. TSC2 is an autosomal dominant multi-system disorder that affects especially the brain, kidneys, heart, and skin. It 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:

Contains 1 Rap-GAP domain.

SWISS:

P49815

Gene ID:

7249

Important Note:

This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

Tuberin 为180kD-220的蛋白产生于结节性硬化（TSC-2）位于染色体16的基因。结节性硬化为常染色体疾病已知为斑痣性错构瘤病其特征为广泛性发展呈良性生长，在许多组织与器官中描述为错构瘤。Tuberin被认为在GTPase活动性蛋白中起作用调节细胞内吞与作为肿瘤抑制子。Tuberin低水平广泛性表达在大多数组织中，而在皮层神经元、小脑浦肯野氏细胞、脊索运动神经元、胰岛B细胞、心肌、肾脏与皮肤的小血管中表达增加。