ALG3 transfers Man to N-glycan precursor (GlcNAc)2 (Man)5 (PP-Dol)1

Stable Identifier
R-HSA-446188
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Synonyms
Addition of the sixth mannose to the N-glycan precursor by ALG3
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The sixth mannose is added to the N-glycan precursor. This reaction occurs in the ER lumen and uses a different mannose donor (dolichyl-phosphate-mannose) than the previous steps. It has been proposed that ALG3, along with all the mannosyl- and glucosyltransferases in the N-glycan biosynthesis pathway that use dolichyl-phosphate-mannose or dolichyl-phosphate-glucose as donor, derive from duplications of a common ancestral enzyme (Oriol et al. 2002). Defects in ALG3 are associated with Congenital Disorder of Glycosylation 1D (CDG1D) (Sun et al. 2005).

Literature References
PubMed ID Title Journal Year
15840742 Congenital disorder of glycosylation id presenting with hyperinsulinemic hypoglycemia and islet cell hyperplasia

Sun, L, Eklund, EA, Chung, WK, Wang, C, Cohen, J, Freeze, HH

J Clin Endocrinol Metab 2005
12200473 Common origin and evolution of glycosyltransferases using Dol-P-monosaccharides as donor substrate

Oriol, R, Martinez-Duncker, I, Chantret, I, Mollicone, R, Codogno, P

Mol Biol Evol 2002
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
alpha-1,3-mannosyltransferase activity of ALG3 [endoplasmic reticulum membrane]
Physical Entity
Activity
This event is regulated
Orthologous Events
Authored
Reviewed
Created