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Rabbit Anti-RASA1/BF350 Conjugated antibody (bs-13280R-BF350)
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-13280R-BF350
英文名称 Rabbit Anti-RASA1/BF350 Conjugated antibody
中文名称 BF350标记的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.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
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 (predicted: Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Sheep, )
产品应用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 116kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
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 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
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.
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.

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.
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