The second glucose (supplied from the donor dolichol-phosphate-glucose) is added to the N-glycan precursor, mediated by ALG8 (Schollen E et al, 2004). Defects in ALG8 are the cause of congenital disorder of glycosylation type 1H (CDG1H) (Schollen E et al, 2004; Sun L et al, 2005).
Thomas, JA, Freeze, HH, Eklund, EA, Sun, L, Van Hove, JL
Reyntjens, R, Hennet, T, Wevers, RA, Smeitink, J, Frank, CG, Matthijs, G, Keldermans, L, Winchester, BG, Grubenmann, CE, Clayton, PT, Schollen, E, Aebi, M
dolichyl-phosphate-glucose-glycolipid alpha-glucosyltransferase activity of ALG8 [endoplasmic reticulum membrane]
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