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

Stable Identifier
R-HSA-446202
Type
Reaction [transition]
Species
Homo sapiens
Compartment
endoplasmic reticulum lumen
ReviewStatus
5/5
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Addition of the first glucose to the N-glycan precursor by ALG6
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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

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

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

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

Am J Med Genet A 2005
Participants
Input
Output
Participates
as an event of
Catalyst Activity

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

Physical Entity
ALG6 [endoplasmic reticulum membrane] (Homo sapiens)
Activity
dolichyl-phosphate-glucose-glycolipid alpha-glucosyltransferase activity (GO:0004583)
Orthologous Events
Addition of the first glucose to the N-glycan precursor by ALG6 (Bos taurus)
Addition of the first glucose to the N-glycan precursor by ALG6 (Caenorhabditis elegans)
Addition of the first glucose to the N-glycan precursor by ALG6 (Canis familiaris)
Addition of the first glucose to the N-glycan precursor by ALG6 (Dictyostelium discoideum)
Addition of the first glucose to the N-glycan precursor by ALG6 (Drosophila melanogaster)
Addition of the first glucose to the N-glycan precursor by ALG6 (Gallus gallus)
Addition of the first glucose to the N-glycan precursor by ALG6 (Mus musculus)
Addition of the first glucose to the N-glycan precursor by ALG6 (Rattus norvegicus)
Addition of the first glucose to the N-glycan precursor by ALG6 (Saccharomyces cerevisiae)
Addition of the first glucose to the N-glycan precursor by ALG6 (Schizosaccharomyces pombe)
Addition of the first glucose to the N-glycan precursor by ALG6 (Sus scrofa)
Addition of the first glucose to the N-glycan precursor by ALG6 (Xenopus tropicalis)
Authored
Dall'Olio, GM  (2009-11-10)
Reviewed
Gagneux, P  (2010-04-16)
Created
Jassal, B  (2009-11-10)
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