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RASA1 Rabbit pAb (bs-13280R)  
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50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
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产品编号 bs-13280R
英文名称 RASA1 Rabbit pAb
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.  
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 Rat (predicted: Human,Mouse,Pig,Sheep,Cow,Chicken,Dog,Horse)
产品应用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 116 kDa
检测分子量
细胞定位 细胞浆 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Ras GTPase-activating protein 1: 451-550/1047 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
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.
Contains 2 SH2 domains.
Contains 1 SH3 domain.

SWISS:
P20936

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



产品图片
Paraformaldehyde-fixed, paraffin embedded (rat brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Anti
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