mannosyl-oligosaccharide glucosidase; processing A-glucosidase I (MOGS, GCS1)

Function

• leaves the distal alpha 1,2-linked glucose residue from the Glc(3)Man(9)GlcNAc(2) oligosaccharide precursor
• exohydrolysis of the non-reducing terminal glucose residues in the mannosyl-oligosaccharide Glc(3)Man(9)GlcNAc(2)
• N-linked oligosaccharide processing, 1st step
• glycosylation of immmunoglobulins prolongs their 1/2 life

Structure

belongs to the glycosyl hydrolase 63 family

Compartment

• endoplasmic reticulum membrane
• single-pass type 2 membrane protein

Pathology

• defects in GCS1 result in
  • congenital disorder of glycosylation 2B
    • defects apparently impair viral entry into cells & viral replication as well as shorten 1/2 life of immunoglobulins

More general terms

• glucosidase
• glycoprotein
• membrane protein

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=7841
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:7841
• OMIM: https://mirror.omim.org/entry/601336
• OMIM: https://mirror.omim.org/entry/606056
• UniProt: http://www.uniprot.org/uniprot/Q13724.html