免疫原
The antiserum was produced against synthesized peptide derived from human SAR1B. AA range:111-160
特异性
Sar1B Polyclonal Antibody detects endogenous levels of Sar1B protein.
来源
Polyclonal, Rabbit,IgG
组成(Formulation)
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
稀释比例
WB: 1/500 - 1/2000. IHC: 1/100 - 1/300. ELISA: 1/20000.. IF 1:50-200
纯化工艺(Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
背景(Background)
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],
功能
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.,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.,similarity:Belongs to the small GTPase superfamily.,similarity:Belongs to the small GTPase superfamily. SAR1 family
蛋白名称
GTP-binding protein SAR1b
其他名称
SAR1B; SARA2; SARB; GTP-binding protein SAR1b; GTP-binding protein B; GTBPB
Fields
>>Protein processing in endoplasmic reticulum;>>Legionellosis
细胞定位
Endoplasmic reticulum membrane ; Peripheral membrane protein . Golgi apparatus, Golgi stack membrane ; Peripheral membrane protein . Associated with the endoplasmic reticulum and Golgi stacks, in particular in the juxta-nuclear Golgi region. .
组织表达
Expressed in many tissues including small intestine, liver, muscle and brain.