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磷酸化馬鈴薯球蛋白(結(jié)節(jié)性硬化)抗體
  • 產(chǎn)品貨號(hào)：
    BN40923R
  • 中文名稱：
    磷酸化馬鈴薯球蛋白(結(jié)節(jié)性硬化)抗體
  • 英文名稱：
    Rabbit anti-Phospho-Tuberin (Thr1462) Polyclonal antibody
  • 品牌：
    Biorigin

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  • BN40923R-100ul

    100ul

    ￥2470.00

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

產(chǎn)品描述

英文名稱Phospho-Tuberin (Thr1462)
中文名稱磷酸化馬鈴薯球蛋白(結(jié)節(jié)性硬化)抗體
別    名Tuberin(phospho T1462); Tuberin (phospho Thr1462); p-Tuberin (Thr1462); FLJ43106; LAM; OTTHUMP00000158940; OTTHUMP00000198394; OTTHUMP00000198395; TSC2; TSC2_HUMAN; TSC4; Tuberin; Tuberous sclerosis 2; Tuberous sclerosis 2 protein; Tuberous sclerosis 2 protein homolog.  
產(chǎn)品類型磷酸化抗體 
研究領(lǐng)域腫瘤  細(xì)胞生物  免疫學(xué)  
抗體來源Rabbit
克隆類型Polyclonal
交叉反應(yīng)Rat,  (predicted: Human, Mouse, Chicken, Dog, Pig, Cow, Horse, Rabbit, )
產(chǎn)品應(yīng)用ELISA=1:5000-10000 IHC-P=1:100-500 （石蠟切片需做抗原修復(fù)）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量200kDa
細(xì)胞定位細(xì)胞漿 細(xì)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 Thr1462:GY(p-T)IS 
亞    型IgG
純化方法affinity purified by Protein A
儲(chǔ) 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(chǎn)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.

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(chǎn)生于結(jié)節(jié)性硬化（TSC-2）位于染色體16的基因。結(jié)節(jié)性硬化為常染色體疾病已知為斑痣性錯(cuò)構(gòu)瘤病其特征為廣泛性發(fā)展呈良性生長，在許多組織與器官中描述為錯(cuò)構(gòu)瘤。Tuberin被認(rèn)為在GTPase活動(dòng)性蛋白中起作用調(diào)節(jié)細(xì)胞內(nèi)吞與作為腫瘤抑制子。Tuberin低水平廣泛性表達(dá)在大多數(shù)組織中，而在皮層神經(jīng)元、小腦浦肯野氏細(xì)胞、脊索運(yùn)動(dòng)神經(jīng)元、胰島B細(xì)胞、心肌、腎臟與皮膚的小血管中表達(dá)增加。