Contents

Search

glucosidase 2 subunit beta; glucosidase II subunit beta; protein kinase C substrate 60.1 kD protein heavy chain; PKCSH; 80K-H protein (PRKCSH, G19P1)

Function: - regulatory subunit of glucosidase 2 - glycan metabolism; N-glycan metabolism - binds glycosylated PTPRC (putative) Structure: - heterodimer of a catalytic alpha subunit (GANAB) & a beta subunit (PRKCSH) - contains 2 EF-hand domains - contains 1 PRKCSH domain Compartment: endoplasmic reticulum (putative) Pathology: - defects in PRKCSH are a cause of polycystic liver disease

Interactions

molecular events

General

enzyme regulatory subunit phosphoprotein

Properties

SIZE: entity length = 528 aa MW = 59 kD COMPARTMENT: endoplasmic reticulum MOTIF: signal sequence {1-14} N-glycosylation site {N72} Ser phosphorylation site {S89} EF hand SITE: 209-244 MOTIF: Ca+2-binding site SITE: 213-236 EF hand SITE: 245-290 glutamate-rich region {313-336} MOTIF: glutamate residue (SEVERAL) Ser phosphorylation site {S383} Ser phosphorylation site {S390} PRKCSH {413-468} MOTIF: Ser phosphorylation site {S434} N-glycosylation site {N476} peptide motif {525-528}

Database Correlations

OMIM correlations MORBIDMAP 177060 UniProt P14314 Pfam PF07915 Entrez Gene 5589 Kegg hsa:5589

References

  1. UniProt :accession P14314
  2. GeneReviews https://www.genecards.org/cgi-bin/carddisp.pl?gene=PRKCSH
  3. Ross CA, McInnis MG, Margolis RL, Li SH. Genes with triplet repeats: candidate mediators of neuropsychiatric disorders. Trends Neurosci. 1993 Jul;16(7):254-60. Review. PMID: 7689767

Component-of

glucosidase 2