The first glucose is added to the N-glycan precursor, mediated by ALG6. Defects in ALG6 are associated with CDG-Ic disorder (Imbach T et al, 1999; Sun L et al, 2005). The donor is a dolichol-phosphate-glucose (synthesized by ALG5).
Hennet, T, Wevers, RA, Burda, P, Berger, EG, Imbach, T, Aebi, M, Kuhnert, P
Thomas, JA, Freeze, HH, Eklund, EA, Sun, L, Van Hove, JL
dolichyl-phosphate-glucose-glycolipid alpha-glucosyltransferase activity of ALG6 [endoplasmic reticulum membrane]
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