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mannosyl-oligosaccharide glucosidase; processing A-glucosidase I (MOGS, GCS1)
Function:
1) leaves the distal alpha 1,2-linked glucose residue from the Glc(3)Man(9)GlcNAc(2) oligosaccharide precursor
2) exohydrolysis of the non-reducing terminal glucose residues in the mannosyl-oligosaccharide Glc(3)Man(9)GlcNAc(2)
3) N-linked oligosaccharide processing, 1st step
4) 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
General
glucosidase
glycoprotein
membrane protein
Properties
SIZE: entity length = 837 aa
MW = 92 kD
COMPARTMENT: endoplasmic reticulum
MOTIF: transmembrane domain {39-59}
N-glycosylation site {N657}
Database Correlations
OMIM correlations
MORBIDMAP 601336
UniProt Q13724
Pfam PF03200
Entrez Gene 7841
Kegg hsa:7841
ENZYME 3.2.1.106
References
- UniProt :accession Q13724
- GeneReviews
http://www.ncbi.nlm.nih.gov/sites/genetests/lab/gene/GCS1