Protein Kinase C Substrate 80K-H (PRKCSH)

PRKCSH encodes the beta-subunit of glucosidase II, an N-linked glycan-processing enzyme in the endoplasmic reticulum (ER). This protein is an acidic phospho-protein known to be a substrate for protein kinase C. Mutations in this gene have been associated with the autosomal dominant polycystic liver disease (PCLD). Alternatively spliced transcript variants encoding distinct isoforms have been observed.The PRKCSH cDNA encodes a protein of 527 amino acids. The deduced amino acid sequence showed a highly glutamic acid-rich region.the available sequence of the beta subunit of human glucosidase II predicts a 514-amino acid protein with a putative endoplasmic reticulum retention signal.PRKCSH as hepatocystin and predicted it to be localized to the endoplasmic reticulum.