Reaction: Addition of a second glucose to the N-glycan precursor by ALG8
- in pathway: Biosynthesis of the N-glycan precursor (dolichol lipid-linked oligosaccharide, LLO) and transfer to a nascent protein
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).
Reaction - small molecule participants:
DOLP [integral component of lumenal side of endoplasmic reticulum membrane]
(Glc)2 (GlcNAc)2 (Man)9 (PP-Dol)1 [integral component of lumenal side of endoplasmic reticulum membrane]
(Glc)1 (GlcNAc)2 (Man)9 (PP-Dol)1 [integral component of lumenal side of endoplasmic reticulum membrane]
DbGP [integral component of lumenal side of endoplasmic reticulum membrane]
Reactome.org reaction link: R-HSA-446189
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Reaction input - small molecules:
alpha-Gal-(1->3)-alpha-Glc-(1->3)-alpha-Man-(1->2)-alpha-Man-(1->2)-alpha-Man-(1->3)-[alpha-Man-(1->2)-alpha-Man-(1->3)-[alpha-Man-(1->2)-alpha-Man-(1->6)]-alpha-Man-(1->6)]-beta-Man-(1->4)-beta-GlcNAc-(1->4)-alpha-GlcNAc(PP-Dol)
dolichyl beta-D-glucosyl phosphate
Reaction output - small molecules:
dolichyl phosphate(2-)
glycan G10599
Reactome.org link: R-HSA-446189