Trimming of the first glucose by by mannosyl-oligosaccharide glucosidase

Stable Identifier
R-HSA-532678
Type
Reaction [transition]
Species
Homo sapiens
Compartment
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After the glycosylated precursor is attached to the protein, the outer alpha-1,2-linked glucose is removed by by mannosyl-oligosaccharide glucosidase (MOGS, GCS1 in yeast). This is a mandatory step for the protein folding control and glycan extension, and defects in MOGS are associated with congenital disorder of glycosylation type IIb (CDGIIb) (De Praeter et al. 2000, Völker et al. 2002).

Literature References
PubMed ID Title Journal Year
12145188 Processing of N-linked carbohydrate chains in a patient with glucosidase I deficiency (CDG type IIb)

Völker, C, De Praeter, CM, Hardt, B, Breuer, W, Kalz-Füller, B, Van Coster, RN, Bause, E

Glycobiology 2002
10788335 A novel disorder caused by defective biosynthesis of N-linked oligosaccharides due to glucosidase I deficiency

De Praeter, CM, Gerwig, GJ, Bause, E, Nuytinck, LK, Vliegenthart, JF, Breuer, W, Kamerling, JP, Espeel, MF, Martin, JJ, De Paepe, AM, Chan, NW, Dacremont, GA, Van Coster, RN

Am J Hum Genet 2000
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
mannosyl-oligosaccharide glucosidase activity of MOGS [endoplasmic reticulum membrane]
Physical Entity
Activity
Authored
Reviewed
Created