Rho GTP酶激活蛋白1/血管畸形骨肥大综合征相关蛋白抗体

英文名称    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; GTPase-activating protein; RAS p21 protein activator (GTPase activating protein) 1; Ras p21 protein activator; RASA; RASA1; RASA1_HUMAN; RasGAP; Triphosphatase activating protein.    

Rho GTP酶激活蛋白1/血管畸形骨肥大综合征相关蛋白抗体     

说 明 书    0.1ml  0.2ml      

研究领域    细胞生物  信号转导  G蛋白偶联受体  G蛋白信号      

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.    

分 子 量    116kDa    

细胞定位    细胞浆     

性    状    Lyophilized or Liquid    

浓    度    1mg/1ml    

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    

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.    

Rho GTP酶激活蛋白1/血管畸形骨肥大综合征相关蛋白抗体 产品介绍    background:
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.

Gene ID:
5921

Database links:

Entrez Gene: 5921 Human

Entrez Gene: 218397 Mouse

Entrez Gene: 25676 Rat

Omim: 139150 Human

SwissProt: P20936 Human

SwissProt: P50904 Rat

Unigene: 664080 Human

Unigene: 12223 Rat

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

Rho GTP酶激活蛋白1/血管畸形骨肥大综合征相关蛋白抗体 

hz-0023P GSK-3 Beta(NT) (Glycogen Synthase Kinase-3 Beta)  糖原合酶激酶-3β（抗原）

hz-0341P CKR-L2( G protein-coupled receptor-2 )  G蛋白偶联受体-2(抗原)

hz-0028P GSK-3 Beta, (Glycogen Synthase Kinase-3)  葡萄糖合成激酶-3（抗原）

hz-0136P GTP-CH-1  三磷酸鸟苷环水解酶（抗原）

hz-0344P H5N1-M2(Avian influenza Matrix Protein-2)  H5N1流感病毒M2型（抗原）

hz-0221P HCV-Core  丙型肝炎病毒-C区（多肽）

hz-0218P HCV-NS1  丙型肝炎病毒-NS1（抗原）

hz-0219P HCV-NS3  丙型肝炎病毒-NS3（抗原）

hz-0213P HCV-NS4a  丙型肝炎病毒-NS4a（抗原）

hz-0212P HEV  戊型肝炎病毒（抗原）

hz-0338P HGV(Hepatitis G vivus)  庚型肝炎病毒（抗原）

hz-0287P HisX6  聚组氨酸标签蛋白

hz-0241P HIV gp120  艾滋病病毒（抗原）

hz-0191P HSP-60( Heat shock protein-60)  热休克蛋白-60（抗原）

hz-0126P HSP-70 peptide  热休克蛋白-70（多肽抗原）

hz-1228P alpha Adaptin/AP1  衔接蛋白α抗原

hz-0214P ICAD(Inhibitor of Caspase-Activated DNase )  Caspase 激活的脱氧核糖核酸酶抑制剂（抗原）

hz-0018P IDE  胰岛素降解酶（抗原）

hz-0227P IGF-1 R   人胰岛素样生长因子-I受体

hz-0406P IGFBP-5 ( Insulin-like Growth Factor Binding Protein 5、IBP5)  胰岛素样生长因子结合蛋白-5(抗原)

hz-0056P Isulin  胰岛素（抗原）

hz-0040P APC(Activated protein C)  APC活化蛋白C抗原

hz-2425P Apelin  脂肪炎症因子Apelin抗原

hz-2430P Apelin receptor/APJ receptor   Apelin receptor抗原

hz-1384P APG4B/AUTL1  APG4B细胞自噬相关抗原

hz-0849P apoA1 (Apolipoprotein/APOA1 protein )human  载脂蛋白A1抗原

hz-1354P Apo J/Clusterin- Alpha/ Beta(Apolipoprotein J)  凝聚素α/β抗原

hz-0047P Insulin Receptor-Alpha(ISR-Alpha)  胰岛素受体-α（抗原）

hz-0260P Insulin Receptor-Alpha(ISR-Alpha)  胰岛素受体-α（抗原）

hz-0290P Insulin Receptor- Beta(ISR- Beta)  胰岛素受体-β（抗原）

hz-0172P IRS-1  胰岛素受体底物-1（抗原）

hz-1253P AQP3(aquaporin 3)  水通道蛋白-3抗原

hz-0173P IRS-2   胰岛素受体底物-2（抗原）

hz-1506P AQP1(Aquaporin1)  水通道蛋白-1抗原

hz-0186P IRS-3  胰岛素受体底物-3（抗原）