Addition of a second glucose to the N-glycan precursor by ALG8

Stable Identifier
R-HSA-446189
Type
Reaction
Species
Homo sapiens
Compartment
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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).

Literature References
PubMed ID Title Journal Year
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
15235028 Clinical and molecular features of three patients with congenital disorders of glycosylation type Ih (CDG-Ih) (ALG8 deficiency)

Reyntjens, R, Hennet, T, Wevers, RA, Smeitink, J, Frank, CG, Matthijs, G, Keldermans, L, Winchester, BG, Grubenmann, CE, Clayton, PT, Schollen, E, Aebi, M

J Med Genet 2004
Participants
Participates
Catalyst Activity

dolichyl-phosphate-glucose-glycolipid alpha-glucosyltransferase activity of ALG8 [endoplasmic reticulum membrane]

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