Trimming of the first glucose by by mannosyl-oligosaccharide glucosidase

Stable Identifier
R-HSA-532678
Type
Reaction
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
10788335 A novel disorder caused by defective biosynthesis of N-linked oligosaccharides due to glucosidase I deficiency

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

Am J Hum Genet 2000
12145188 Processing of N-linked carbohydrate chains in a patient with glucosidase I deficiency (CDG type IIb)

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

Glycobiology 2002
Participants
Participates
Catalyst Activity

Glc3Man9GlcNAc2 oligosaccharide glucosidase activity of MOGS [endoplasmic reticulum membrane]

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