Rho GTP酶激活蛋白1/血管畸形骨肥大综合征相关蛋白抗体

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英文名称  Anti-RASA1

中文名称  Rho GTP酶激活蛋白1/血管畸形骨肥大综合征相关蛋白抗体

别    名  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.  

浓    度  1mg/1ml

规 格  0.2ml/200μg

Rho GTP酶激活蛋白1/血管畸形骨肥大综合征相关蛋白抗体概述：

B淋巴细胞在抗原的刺激下，能够分化、增殖形成具有针对这种抗原分泌特异性抗体的能力。B细胞的这种能力和量是有限的，不可能持续分化增殖下去，因此产生免疫球蛋白的能力也是极其微小的。将这种B细胞与非分泌型的骨髓瘤细胞融合形成杂交瘤细胞，再进一步克隆化，这种克隆化的杂交瘤细胞是既具有瘤细胞的无限分裂的能力，又具有产生特异性抗体的B淋巴细胞的能力。将这种克隆化的杂交瘤细胞进行培养或注入小鼠腹水内即可获得大量的高效、单一的特异性抗体。这种技术即称为单克隆抗体技术。

抗体来源  Rabbit  

克隆类型  polyclonal

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

产品类型  一抗    

研究领域  细胞生物 信号转导 G蛋白偶联受体 G蛋白信号  

蛋白分子量  predicted molecular weight: 116kDa

性    状  Lyophilized or Liquid

免 疫 原  KLH conjugated synthetic peptide derived from human Ras GTPase-activating protein 1

亚    型  IgG

纯化方法  affinity purified by Protein A

储 存 液  Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4

产品应用   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 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.

Rho GTP酶激活蛋白1/血管畸形骨肥大综合征相关蛋白抗体Contains 2 SH2 domains.

Contains 1 SH3 domain.

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