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