产品详情
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
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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)