Conserved Protein Domain Family
PRKCSH

?
cl06793: PRKCSH Superfamily (this model, PSSM-Id:323615 is obsolete and has been replaced by 414904)
Click on image for an interactive view with Cn3D
Glucosidase II beta subunit-like protein
The sequences found in this family are similar to a region found in the beta-subunit of glucosidase II, which is also known as protein kinase C substrate 80K-H (PRKCSH). The enzyme catalyzes the sequential removal of two alpha-1,3-linked glucose residues in the second step of N-linked oligosaccharide processing. The beta subunit is required for the solubility and stability of the heterodimeric enzyme, and is involved in retaining the enzyme within the endoplasmic reticulum. Mutations in the gene coding for PRKCSH have been found to be involved in the development of autosomal dominant polycystic liver disease (ADPLD), but the precise role the protein has in the pathogenesis of this disease is unknown. This family also includes an ER sensor for misfolded glycoproteins and is therefore likely to be a generic sugar binding domain.
Links
?
Statistics
?
Accession: cl06793
PSSM Id: 323615
Name: PRKCSH
Created: 8-Feb-2008
Updated: 5-Dec-2016
| Disclaimer | Privacy statement | Accessibility |
NCBI Home NCBI Search NCBI SiteMap