After the lipid-linked oligosaccharide (LLO) precursor is attached to the protein, the outer alpha-1,2-linked glucose is removed by by mannosyl-oligosaccharide glucosidase (MOGS). This is a mandatory step for protein folding control and glycan extension. Defects in MOGS are associated with congenital disorder of glycosylation type IIb (CDGIIb), a multisystem disorder caused by a defect in glycoprotein biosynthesis and characterised by under-glycosylated serum glycoproteins (De Praeter et al. 2000, Voelker et al. 2002). Type II CDGs refer to defects in the trimming and processing of protein-bound glycans.
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
Hardt, B, Kalz-Füller, B, Bause, E, De Praeter, CM, Breuer, W, Van Coster, RN, Völker, C
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