磷酸化Rho GTP酶激活蛋白1/骨肥大綜合征相關(guān)蛋白抗體說明書 英文名稱  Anti-phospho-RASA1 (Tyr460)

中文名稱  磷酸化Rho GTP酶激活蛋白1/骨肥大綜合征相關(guān)蛋白抗體說明書 

別    名 GAP (phospho Y460); p-GAP (phospho Y460); Ras GAP; CM AVM; CMAVM; DKFZp434N071; GAP; GTPase activating protein; GTPase-activating protein; OTTHUMP00000222390; OTTHUMP00000222391; OTTHUMP00000222392; OTTHUMP00000222393; p120GAP; p120RASGAP; PKWS; Ras GTPase-activating protein 1; RAS p21 protein activator (GTPase activating protein) 1; Ras p21 protein activator; RASA; RASA1; RASA1_HUMAN; RasGAP; Triphosphatase activating protein.

產(chǎn)品屬性：

濃    度  1mg/1ml

規(guī) 格  0.1ml/100μg

抗體來源  Rabbit

克隆類型   polyclonal

交叉反應(yīng)   Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Sheep

產(chǎn)品類型   一抗 磷酸化抗體  

研究領(lǐng)域     細(xì)胞生物 信號(hào)轉(zhuǎn)導(dǎo) G蛋白偶聯(lián)受體 G蛋白信號(hào)

蛋白分子量  predicted molecular weight: 116kDa 

性    狀   Lyophilized or Liquid

免 疫 原  KLH conjugated synthesised phosphopeptide derived from human GAP around the phosphorylation site of Tyr460

亞    型  IgG

純化方法   affinity purified by Protein A

儲(chǔ) 存 液   Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4

磷酸化Rho GTP酶激活蛋白1/骨肥大綜合征相關(guān)蛋白抗體說明書 產(chǎn)品應(yīng)用   WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=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.  

保存條件  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. 

產(chǎn)品介紹 The mammalian c-H-, c-K- and N-Ras proto-oncogenes encode ubiquitously expressed proteins (1,2). p21Ras can exist in either a physiologically quiescent GDP-binding state or a GTP-binding signal-emitting state (3). Oncogenic p21Ras proteins are trapped in the excited signal-emitting state because the mechanism normally employed to delimit their excitation period, hydrolysis of their bound GTP to GDP, is impaired as a result of specific mutations (3). Interaction of p21Ras with GTPase activating protein (GAP) can increase hydrolysis of p21Ras-bound GTP by as much as 1000-fold (4,5). The product of the neurofibromatosis type 1 gene (NF1) has also been shown to exhibit p21Ras GAP activity (6,7), and proteins that stimulate the GTPase activity of three other low molecular weight GTPases, including Rho, Rab 3A and Rap 1, have also been described (8,9).

Function : Inhibitory regulator of the Ras-cyclic AMP pathway. Stimulates the GTPase of normal but not oncogenic Ras p21.

Subunit : Interacts with SQSTM1. Interacts with SPSB1; the interaction does not promote degradation. Interacts with CAV2 (tyrosine phosphorylated form). Directly interacts with NCK1. Interacts with PDGFRB (tyrosine phosphorylated). Interacts (via SH2 domain) with the 'Tyr-9' phosphorylated form of PDPK1.

Subcellular Location : Cytoplasm.

Tissue Specificity : In placental villi, detected only in the trophoblast layer (cytotrophoblast and syncytiotrophoblast). Not detected in stromal, endothelial or Hofbauer cells (at protein level).

DISEASE : Note=Mutations in the SH2 domain of RASA seem to be oncogenic and cause basal cell carcinomas.

Defects in RASA1 are the cause of capillary malformation-arteriovenous malformation (CMAVM) [MIM:608354]. CMAVM is a disorder characterized by atypical capillary malformations that are multiple, small, round to oval in shape and pinkish red in color. These capillary malformations are associated with either arteriovenous malformation, arteriovenous fistula, or Parkes Weber syndrome.

Defects in RASA1 are a cause of Parkes Weber syndrome (PKWS) [MIM:608355]. PKWS is a disorder characterized by a cutaneous flush with underlying multiple micro-arteriovenous fistulas, in association with soft tissue and skeletal hypertrophy of the affected limb.

Similarity : Contains 1 C2 domain.

Contains 1 PH domain.

Contains 1 Ras-GAP domain.

Contains 2 SH2 domains.

Contains 1 SH3 domain.

Database links : UniProtKB/Swiss-Prot: P20936.1

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