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

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

Stable Identifier

R-HSA-446189

Type

Reaction [transition]

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
15235028	Clinical and molecular features of three patients with congenital disorders of glycosylation type Ih (CDG-Ih) (ALG8 deficiency) Schollen, E, Frank, CG, Keldermans, L, Reyntjens, R, Grubenmann, CE, Clayton, PT, Winchester, BG, Smeitink, J, Wevers, RA, Aebi, M, Hennet, T, Matthijs, G	J Med Genet	2004
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