其他名称： SAR1B; SARA2; SARB; GTP-binding protein SAR1b; GTP-binding protein B; GTBPB
实测条带： 22kD
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.,subcellular location:Associated with the endoplasmic reticulum and Golgi stacks, in particular in the juxta-nuclear Golgi region.,subunit:Homodimer. Binds PREB. Part of the COPII coat complex. Binds to the cytoplasmic tails of target proteins in the endoplasmic reticulum.,tissue specificity:Expressed in many tissues including small intestine, liver, muscle and brain.,
相关产品： RS0001,RS0002,YM3028,YM3029
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.
其他名称： SAR1B; SARA2; SARB; GTP-binding protein SAR1b; GTP-binding protein B; GTBPB
实测条带： 22kD
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.,subcellular location:Associated with the endoplasmic reticulum and Golgi stacks, in particular in the juxta-nuclear Golgi region.,subunit:Homodimer. Binds PREB. Part of the COPII coat complex. Binds to the cytoplasmic tails of target proteins in the endoplasmic reticulum.,tissue specificity:Expressed in many tissues including small intestine, liver, muscle and brain.,
相关产品： RS0001,RS0002,YM3028,YM3029
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.
在线咨询
湘公网安备