Target：Glucosidase IIβ

Fields：Protein processing in endoplasmic reticulum

Gene Name：PRKCSH

Protein Name：Glucosidase 2 subunit beta

Human Gene Id：5589

Human Swiss Prot No：P14314

Mouse Gene Id：19089

Mouse Swiss Prot No：O08795

Immunogen：The antiserum was produced against synthesized peptide derived from human GLU2B. AA range:81-130

Specificity：Glucosidase IIβ Polyclonal Antibody detects endogenous levels of Glucosidase IIβ protein.

Formulation：Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

Source：Polyclonal, Rabbit,IgG

Dilution：WB 1:500 - 1:2000. IF 1:200 - 1:1000. ELISA: 1:10000. Not yet tested in other applications.

Purification：The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Concentration：1 mg/ml

Storage Stability：-15°C to -25°C/1 year(Do not lower than -25°C)

Other Name：PRKCSH;G19P1;Glucosidase 2 subunit beta;80K-H protein;Glucosidase II subunit beta;Protein kinase C substrate 60.1 kDa protein heavy chain;PKCSH

Observed Band(KD)：59kD

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],

Function：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.,

Subcellular Location：Endoplasmic reticulum .

Expression： Lung,Lymphocyte,Platelet,