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    磷酸化結節性硬化蛋白抗體
    • 產品貨號:
      BN41147R
    • 中文名稱:
      磷酸化結節性硬化蛋白抗體
    • 英文名稱:
      Rabbit anti-Phospho-Tuberin (Ser1418) Polyclonal antibody
    • 品牌:
      Biorigin
    • 貨號

      產品規格

      售價

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    • BN41147R-100ul

      100ul

      ¥2470.00

      交叉反應:Human,Mouse,Rat(predicted:Chicken,Dog,Pig,Cow,Horse) 推薦應用:IHC-P,IHC-F,IF,Flow-Cyt,ELISA

    產品描述

    英文名稱Phospho-Tuberin (Ser1418)
    中文名稱磷酸化結節性硬化蛋白抗體
    別    名TSC2(phospho S1418); Tuberin (Phospho Ser1418); Tuberin (Phospho S1418); Phospho-TSC2 (Ser1418); P-Tuberin/TSC2 (Ser1418); P-TSC2 (Ser1418); FLJ43106; LAM; TSC2; TSC2_HUMAN; TSC4; Tuberin; Tuberous sclerosis 2 protein.  
    產品類型磷酸化抗體 
    研究領域腫瘤  免疫學  神經生物學  
    抗體來源Rabbit
    克隆類型Polyclonal
    交叉反應Mouse, Rat,  (predicted: Human, Chicken, Dog, Pig, Cow, Horse, )
    產品應用ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 (石蠟切片需做抗原修復)
    not yet tested in other applications.
    optimal dilutions/concentrations should be determined by the end user.
    分 子 量200kDa
    細胞定位細胞漿 細胞膜 
    性    狀Liquid
    濃    度1mg/ml
    免 疫 原KLH conjugated Synthesised phosphopeptide derived from human Tuberin around the phosphorylation site of Ser1418:AR(p-S)QS 
    亞    型IgG
    純化方法affinity purified by Protein A
    儲 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
    保存條件Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
    PubMedPubMed
    產品介紹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

    Database links:

    Entrez Gene: 7249 Human

    Entrez Gene: 22084 Mouse

    Entrez Gene: 24855 Rat

    Omim: 191092 Human

    SwissProt: P49815 Human

    SwissProt: Q61037 Mouse

    SwissProt: P49816 Rat

    Unigene: 90303 Human

    Unigene: 30435 Mouse

    Unigene: 5875 Rat



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

    Tuberin 為180kD-220的蛋白產生于結節性硬化(TSC-2)位于染色體16的基因。結節性硬化為常染色體疾病已知為斑痣性錯構瘤病其特征為廣泛性發展呈良性生長,在許多組織與器官中描述為錯構瘤。Tuberin被認為在GTPase活動性蛋白中起作用調節細胞內吞與作為腫瘤抑制子。Tuberin低水平廣泛性表達在大多數組織中,而在皮層神經元、小腦浦肯野氏細胞、脊索運動神經元、胰島B細胞、心肌、腎臟與皮膚的小血管中表達增加。


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