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PRKCSH is 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.
Gene Name: | protein kinase C substrate 80K-H |
Synonyms: | PRKCSH, 80K-H protein, AGE-binding receptor 2, AGE-R2, Glucosidase II subunit beta, Hepatocystin, Glucosidase II, beta subunit, PCLD, PKCSH, G19P1, Glucosidase 2 subunit beta |
Target Sequences: | NM_002743 NP_002734.2 P14314 |
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