产品货号 :  mlR19392

英文名称 :  SAR1B

中文名称 :  GTP结合蛋白SAR1B抗体

别    名 :  ANDD; CMRD; GTBPB; GTP binding protein SAR1b; GTP binding protein Sara; GTP-binding protein B; GTP-binding protein SAR1B; SAR1 gene homolog B (S. cerevisiae); SAR1 homolog B; SAR1a gene homolog 2; SAR1B; SAR1B_HUMAN; SARA2; SARB.  

研究领域 :  细胞生物  信号转导  结合蛋白  G蛋白信号  

抗体来源 :  Rabbit

克隆类型 :  Polyclonal

交叉反应 :  Human, Mouse, Rat, Pig, Cow,

产品应用 :  ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 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 :  22kDa

细胞定位 :  细胞浆

性    状 :  Lyophilized or Liquid

浓    度 :  1mg/ml

免 疫 原 :  KLH conjugated synthetic peptide derived from human SAR1B:51-150/198

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.

PubMed :  PubMed

产品介绍 :   The protein encoded by this gene is a small GTPase that acts as a homodimer. The encoded protein is activated by the guanine nucleotide exchange factor PREB and is involved in protein transport from the endoplasmic reticulum to the Golgi. This protein is part of the COPII coat complex. Defects in this gene are a cause of chylomicron retention disease (CMRD), also known as Anderson disease (ANDD). Two transcript variants encoding the same protein have been found for this gene. [provided by RefSeq, Mar 2010]

Function:

Involved in transport from the endoplasmic reticulum to the Golgi apparatus. Activated by the guanine nucleotide exchange factor PREB. Involved in the selection of the protein cargo and the assembly of the COPII coat complex.

Subunit:

Homodimer. Binds PREB. Part of the COPII coat complex. Binds to the cytoplasmic tails of target proteins in the endoplasmic reticulum (By similarity).

Subcellular Location:

Endoplasmic reticulum membrane. Golgi apparatus > Golgi stack membrane. Associated with the endoplasmic reticulum and Golgi stacks, in particular in the juxta-nuclear Golgi region.

Tissue Specificity:

Expressed in many tissues including small intestine, liver, muscle and brain.

DISEASE:

Defects in SAR1B are the cause of chylomicron retention disease (CMRD) [MIM:246700]; also known as Anderson disease (ANDD). CMRD is an autosomal recessive disorder of severe fat malabsorption associated with failure to thrive in infancy. The condition is characterized by deficiency of fat-soluble vitamins, low blood cholesterol levels, and a selective absence of chylomicrons from blood. Affected individuals accumulate chylomicron-like particles in membrane-bound compartments of enterocytes, which contain large cytosolic lipid droplets.

Similarity:

Belongs to the small GTPase superfamily. SAR1 family.

SWISS:

Q9Y6B6

Gene ID:

51128

Important Note:

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