中文名称  磷酸化性硬化症蛋白1抗体品牌 

英文名称  Anti-phospho-TSC1(Ser505)

别    名 TSC1(phospho S505); LAM; TSC1; Tuberous sclerosis 1 protein.

产品属性：

磷酸化性硬化症蛋白1抗体品牌 浓    度  1mg/1ml

规 格  0.1ml/100μg

抗体来源  Rabbit

克隆类型   polyclonal

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

产品类型   一抗 磷酸化抗体  

研究领域     免疫学 染色质和核信号

蛋白分子量  predicted molecular weight: 128kDa 

性    状   Lyophilized or Liquid

免 疫 原  KLH conjugated Synthesised phosphopeptide derived from human TSC1 around the phosphorylation site of Ser505 [FD(p-S)PF]

亚    型  IgG

纯化方法   affinity purified by Protein A

储 存 液   0.01M PBS, pH 7.4 with 10 mg/ml BSA and 0.1% Sodium azide

磷酸化性硬化症蛋白1抗体品牌 产品应用   WB=1:100-500 ELISA=1:500-1000 IP=1:20-100 IHC-P=1:100-500 IHC-F=1:100-500 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.  

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. 

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

产品介绍 Hamartin, or TSC1, is a suspected tumor suppressor implicated in the disease tuberous sclerosis 1. It is a negative regulator of cell division controlling the transition from G0/G1 to S phase, and it seems to act through the phosphatidylinositol 3 kinase/Akt pathway. TSC1 interacts with tuberin m(TSC2), which is thought to be a GAP (GTPase Activating Protein) for the Rap1 and Rab5 small G Proteins. The Hamartin/Tuberin complex has been shown to inhibit mTor. Hamartin has also been shown to interact with ERM (Ezrin-Radixin-Moesin) proteins and with F-actin, suggesting a role for TSC proteins in modulation of cell adhesion and morphology.

Function : In complex with TSC2, inhibits the nutrient-mediated or growth factor-stimulated phosphorylation of S6K1 and EIF4EBP1 by negatively regulating mTORC1 signaling. Seems not to be required for TSC2 GAP activity towards RHEB. Implicated as a tumor suppressor. Involved in microtubule-mediated protein transport, but this seems to be due to unregulated mTOR signaling.

Subunit : Interacts with TSC2, leading to stabilize TSC2. In the absence of TSC2, TSC1 self-aggregates. Interacts with DOCK7. Interacts with FBXW5 and TBC1D7.

Subcellular Location : Cytoplasm. Membrane; Peripheral membrane protein. Note=At steady state found in association with membranes.

Tissue Specificity : Highly expressed in skeletal muscle, followed by heart, brain, placenta, pancreas, lung, liver and kidney. Also expressed in embryonic kidney cells.

Post-translational modifications : Phosphorylation at Ser-505 does not affect interaction with TSC2. Phosphorylated upon DNA damage, probably by ATM or ATR.

DISEASE : Defects in TSC1 are the cause of tuberous sclerosis type 1 (TSC1) [MIM:191100]. It is an autosomal dominant multi-system disorder that affects especially the brain, kidneys, heart, and skin. TS1C 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 TSC1 may be a cause of focal cortical dysplasia of Taylor balloon cell type (FCDBC) [MIM:607341]. FCDBC is a subtype of cortical displasias linked to chronic intractable epilepsy. Cortical dysplasias display a broad spectrum of structural changes, which appear to result from changes in proliferation, migration, differentiation, and apoptosis of neuronal precursors and neurons during cortical development.

性硬化症为常染色体显性遗传，也常见散例。是抑制基因，基因产物分别为Hamartin和tuberin，两者均调节细胞生长。

性硬化症(tuberous sclerosis)又称性脑硬化，Bourneville病。本病可归类于神经皮肤综合征(亦称斑痣性错构瘤病)，是源于外胚层的器官发育异常所致，累及、皮肤和眼，也可累及中胚层，内胚层器官如心、肺、骨，肾和胃肠等。皮脂腺瘤是皮肤神经末梢、增生的结缔组织和组成，视网膜可见、神经节细胞瘤，心、肾、肺、*等也可发生。

而神经胶质增生性硬化广泛发生于大脑皮质、白质、基底节和室管膜下，常伴钙质沉积，可出现一位症及增生等，出现发作及为特征。

实验流程：

全、新、优、品、好四大特点：

磷酸化性硬化症蛋白1抗体品牌 全：公司提供上万种产品，涵盖了生物试剂，elisa试剂盒，标准品，培养基，原装耗材，抗体、培养基、ATCC细胞等，基本上各种科研所需产品在我司都能找到。

新：产品更新速度较快，基本上每周都有新产品出现。

优：产品质量好，投诉比较少。

好：我公司具有优质的技术团队，产品一旦售出，实验过程中遇到困难可提供在线技术咨询。使您使用产品时没有任何的后顾之忧。

技术外包服务:

★磷酸化性硬化症蛋白1抗体品牌 分子生物学：质粒抽提、PCR、Q-PCR、RT-PCR、分子生物学：基因合成、引物合成、基因测序、载体构建等

★蛋白工程：原核、哺乳动物蛋白表达系统等

★病毒包装：腺病毒、慢病毒等

★抗体工程：磁珠分选、病理染色、WB、ELISA、IP、IF、IHC、FACS、Confocal等等

★细胞工程：细胞表型分析(凋亡、增殖、周期、迁移、侵袭、修复、克隆形成)、细胞培养、细胞膜制备、稳定细胞株构建、细胞RNAi技术等等。

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