The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Protein concentration is above or equal to 0.05 mg/ml.
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Preparation and Storage
Stability and Storage
Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.
pH: 8.00 Constituents: 0.31% Glutathione, 0.79% Tris HCl
AGE-binding receptor 2
Glucosidase 2 subunit beta
Glucosidase II beta subunit
Glucosidase II subunit beta
Protein kinase C substrate 60.1 kDa protein heavy chain
Protein kinase C substrate 80 Kda protein
Protein kinase C substrate 80K-H
Protein kinase C substrate, 80 Kda protein
Regulatory subunit of glucosidase II.
Glycan metabolism; N-glycan metabolism.
Involvement in 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.