Addition of the first glucose to the N-glycan precursor by ALG6

Stable Identifier
R-HSA-446202
Type
Reaction
Species
Homo sapiens
Compartment
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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).

Literature References
PubMed ID Title Journal Year
10359825 A mutation in the human ortholog of the Saccharomyces cerevisiae ALG6 gene causes carbohydrate-deficient glycoprotein syndrome type-Ic

Hennet, T, Wevers, RA, Burda, P, Berger, EG, Imbach, T, Aebi, M, Kuhnert, P

Proc Natl Acad Sci U S A 1999
16007612 Clinical and molecular characterization of the first adult congenital disorder of glycosylation (CDG) type Ic patient

Thomas, JA, Freeze, HH, Eklund, EA, Sun, L, Van Hove, JL

Am J Med Genet A 2005
Participants
Participates
Catalyst Activity

dolichyl-phosphate-glucose-glycolipid alpha-glucosyltransferase activity of ALG6 [endoplasmic reticulum membrane]

Orthologous Events
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