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

Stable Identifier
R-HSA-446189
Type
Reaction
Species
Homo sapiens
Compartment
endoplasmic reticulum lumen
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Addition of a second glucose to the N-glycan precursor by ALG8
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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
Input
Output
Participates
as an event of
Catalyst Activity

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

Physical Entity
ALG8 [endoplasmic reticulum membrane] (Homo sapiens)
Activity
dolichyl-phosphate-glucose-glycolipid alpha-glucosyltransferase activity (GO:0004583)
Orthologous Events
Addition of a second glucose to the N-glycan precursor by ALG8 (Caenorhabditis elegans)
Addition of a second glucose to the N-glycan precursor by ALG8 (Canis familiaris)
Addition of a second glucose to the N-glycan precursor by ALG8 (Danio rerio)
Addition of a second glucose to the N-glycan precursor by ALG8 (Dictyostelium discoideum)
Addition of a second glucose to the N-glycan precursor by ALG8 (Drosophila melanogaster)
Addition of a second glucose to the N-glycan precursor by ALG8 (Gallus gallus)
Addition of a second glucose to the N-glycan precursor by ALG8 (Mus musculus)
Addition of a second glucose to the N-glycan precursor by ALG8 (Rattus norvegicus)
Addition of a second glucose to the N-glycan precursor by ALG8 (Saccharomyces cerevisiae)
Addition of a second glucose to the N-glycan precursor by ALG8 (Schizosaccharomyces pombe)
Addition of a second glucose to the N-glycan precursor by ALG8 (Sus scrofa)
Addition of a second glucose to the N-glycan precursor by ALG8 (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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