Total Glucosidase IIβ Cell-Based Colorimetric ELISA Kit

  • Catalog No.:KA3542C
  • Applications:ELISA
  • Reactivity:Human;Mouse
    • Gene Name:
    • PRKCSH
    • Human Gene Id:
    • 5589
    • Human Swiss Prot No:
    • P14314
    • Mouse Swiss Prot No:
    • O08795
    • Storage Stability:
    • 2-8°C/6 months
    • Other Name:
    • Glucosidase 2 subunit beta (80K-H protein) (Glucosidase II subunit beta) (Protein kinase C substrate 60.1 kDa protein heavy chain) (PKCSH)
    • Detection Method:
    • Colorimetric
    • Background:
    • 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.,
    • Function:
    • intracellular signaling cascade, protein kinase cascade,
    • Subcellular Location:
    • Endoplasmic reticulum .