Addition of a second mannose to the N-glycan precursor by ALG2

Stable Identifier
R-HSA-446208
Type
Reaction [transition]
Species
Homo sapiens
Compartment
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A second mannose is added to the N-glycan precursor via an alpha-1,3 linkage. The reaction is catalyzed by the mannosyltransferase ALG2. This is a bifunctional enzyme with both alpha 1,3- and alpha 1,6-mannosyltransferase activities. In humans, only the alpha 1,3 activity used in this reaction has been elucidated (Thiel et al. 2003). Defects in ALG2 are the cause of ALG2-CDG (CDG-1i; MIM:607906) (Thiel et al. 2003).

Literature References
PubMed ID Title Journal Year
12684507 A new type of congenital disorders of glycosylation (CDG-Ii) provides new insights into the early steps of dolichol-linked oligosaccharide biosynthesis

Thiel, C, Schwarz, M, Peng, J, Grzmil, M, Hasilik, M, Braulke, T, Kohlsch√ľtter, A, von Figura, K, Lehle, L, K√∂rner, C

J Biol Chem 2003
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
alpha-1,3-mannosyltransferase activity of ALG2 [endoplasmic reticulum membrane]
Physical Entity
Activity
Orthologous Events
Authored
Reviewed
Created