PRKCSH
PRKCSH (PRKCSH beta subunit of glucosidase II, also known as glucosidase 2 subunit beta, 80K-H, VASAP-60 and AGE-R2) is the regulatory subunit of glucosidase II, an N-linked glycan-processing enzyme of the endoplasmic reticulum. Mutations in the PRKCSH gene are associated with autosomal dominant polycystic liver disease. Browse Assay Genie's current range of PRKCSH research reagents below, or read on for the biology behind the target.
PRKCSH is the regulatory subunit of glucosidase II. The enzyme cleaves, in sequence, the two innermost alpha-1,3-linked glucose residues from the Glc(2)Man(9)GlcNAc(2) oligosaccharide precursor carried by immature glycoproteins. This trimming is a step in N-linked glycan processing within the endoplasmic reticulum. PRKCSH is also required for efficient biogenesis of PKD1/polycystin-1 and for its trafficking to the plasma membrane of the primary cilia, which links glycoprotein processing to ciliary biology.
The PRKCSH gene encodes the beta subunit of glucosidase II, an acidic phosphoprotein that is a known substrate for protein kinase C. HGNC places it in the EF-hand domain containing and MRH domain containing gene groups. Alternative splicing of the gene produces multiple transcript variants. The mouse ortholog is Prkcsh, which allows the biology to be studied in murine systems.
The protein has gone by several names, reflecting its different lines of discovery: protein kinase C substrate 80K-H, VASAP-60, AGE-R2, GIIB, GIIbeta and GluIIbeta. The symbols PCLD and PLD1, together with the previous name polycystic liver disease, reflect the association of mutations in this gene with autosomal dominant polycystic liver disease. Researchers studying PRKCSH, whether through a PRKCSH antibody, a PRKCSH ELISA kit or recombinant PRKCSH protein, are typically interested in glycoprotein quality control, polycystin biogenesis or hepatic cystogenesis.
PRKCSH regulates glucosidase II, which removes glucose residues from the Glc(2)Man(9)GlcNAc(2) precursor of immature glycoproteins in the endoplasmic reticulum.
Mutations in the PRKCSH gene are associated with autosomal dominant polycystic liver disease, making it a central gene in studies of hepatic cyst formation.
PRKCSH is required for efficient PKD1/polycystin-1 biogenesis and its delivery to the plasma membrane of primary cilia.
As an acidic phosphoprotein and protein kinase C substrate, PRKCSH offers a model for how phosphorylation relates to endoplasmic reticulum enzyme regulation.
PRKCSH is the regulatory beta subunit of glucosidase II. The enzyme sequentially removes the two innermost alpha-1,3-linked glucose residues from the Glc(2)Man(9)GlcNAc(2) precursor of immature glycoproteins. PRKCSH is also required for efficient PKD1/polycystin-1 biogenesis and trafficking to the primary cilia plasma membrane.
Aliases include glucosidase 2 subunit beta, 80K-H, VASAP-60, AGE-R2, GIIB, GIIbeta, GluIIbeta, PKCSH, G19P1, PCLD and PLD1. Previous names are protein kinase C substrate 80K-H and polycystic liver disease. Alternative splicing gives multiple transcript variants.
Mutations in the PRKCSH gene are associated with autosomal dominant polycystic liver disease. The mouse ortholog is called Prkcsh. Check the datasheet of any product for its validated reactivity before planning cross-species experiments.
Glucosidase II partners, polycystin proteins and other polycystic disease genes with products at Assay Genie.
Gene and protein annotations from HGNC, NCBI Gene and UniProt P14314; product specifications from Assay Genie datasheets.