Glucosidase IIβ Rabbit Polyclonal Antibody

Description

EnkiLife offers the Glucosidase IIβ Rabbit Polyclonal Antibody. This antibody reacts with Human,Mouse species and is suitable for WB,ICC/IF,ELISA application. Alternative Names of Immunogen: PRKCSH; G19P1; Glucosidase 2 subunit beta; 80K-H protein; Glucosidase II subunit beta; Protein kinase C substrate 60.1 kDa protein heavy chain; PKCSH. The product boasts remarkable high specificity, outstanding reproducibility, and exceptional affinity, making it a reliable and superior choice for your needs. Dilution Ratio: WB 1:500-1:2000,ICC/IF 1:200-1:1000,ELISA 1:5000-1:20000 Background: This gene encodes the beta-subunit of glucosidase II, an N-linked glycan-processing enzyme in the endoplasmic reticulum. The encoded protein is an acidic phosphoprotein known to be a substrate for protein kinase C. Mutations in this gene have been associated with the autosomal dominant polycystic liver disease. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jan 2014],disease:Defects in PRKCSH are a cause of polycystic liver disease (PCLD) [MIM:174050]. PCLD is an autosomal dominant disorder and is characterized by the presence of multiple liver cysts of biliary epithelial origin. PCLD is a distinct clinical and genetic entity that can occur independently from autosomal dominant polycystic kidney disease (ADPKD) [MIM:173900], which in a considerable but uncertain proportion of cases is associated with hepatic cysts.,function:Regulatory subunit of glucosidase II.,pathway:Glycan metabolism; N-glycan metabolism.,similarity:Contains 1 PRKCSH domain.,similarity:Contains 2 EF-hand domains.,subunit:Heterodimer of a catalytic alpha subunit (GANAB) and a beta subunit (PRKCSH). Binds glycosylated PTPRC.,