FITC标记的马铃薯球蛋白(结节性硬化)抗体上海沪震实业有限公司

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产品名称: FITC标记的马铃薯球蛋白(结节性硬化)抗体
英文名称: Anti-Tuberin/FITC
抗体货号: HZ-3586R-FITC
产品规格: 100ul
级    别: 分析纯, , 分析纯,
产品产地: 中国/上海
品牌商标: HZbscience
价    格: 2980元
抗原: Rabbit
抗原来源: Rabbit
抗体来源: Rabbit
适用物种: 
FITCconjugate:FITC
应用范围: IF=1:50-200
更新时间: 2018/9/13 12:18:00
详细资料:  实验方法技术资料
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使用范围:仅限科研使用,不能应用于临床
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上海沪震实业有限公司
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产品详细描述

 Rabbit Anti-Tuberin/FITC Conjugated antibody

FITC标记的马铃薯球蛋白(结节性硬化)抗体

 

英文名称 Anti-Tuberin/FITC
中文名称 FITC标记的马铃薯球蛋白(结节性硬化)抗体
别    名 TSC2; FLJ43106; LAM; OTTHUMP00000158940; OTTHUMP00000198394; OTTHUMP00000198395; TSC2; TSC2_HUMAN; TSC4; Tuberin; Tuberous sclerosis 2; Tuberous sclerosis 2 protein; Tuberous sclerosis 2 protein homolog.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 肿瘤  细胞生物  免疫学  细胞表面分子  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, 
产品应用 IF=1:50-200  
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 200kDa
细胞定位 细胞膜 
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Tuberin
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.
产品介绍 background:
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.

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 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
   

Tuberin,或TSC2(结节性硬化症),被牵连作为肿瘤抑制剂。它可能在囊泡运输中起作用,也可能在调节细胞生长停滞和调节类固醇受体介导的转录中发挥作用。HAMARTIN(TSC1)与结核杆菌之间的相互作用可促进囊泡对接。它特异性地刺激RAS相关蛋白RAP1A和Rab5的内在GTP酶活性,提示其可能参与调控细胞生长的机制。结节突变导致RAP1A在肿瘤中的组成性激活。Tuberin至少存在三种亚型。

生物在线声明:以上所展示的信息由企业自行提供,内容的真实性、准确性和合法性由发布企业负责。生物在线对此不承担任何保证责任。

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