Addition of the first mannose to the N-glycan precursor by ALG1

Stable Identifier
Reaction [transition]
Homo sapiens
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A mannose is added to the N-glycan precursor via a beta-1,4 linkage. The reaction is catalyzed by ALG1 (Takahashi T et al, 2000). Defects in ALG1 lead to congenital disorder of glycosylation type 1K (CDG1K) (Schwarz et al. 2004; Kranz et al. 2004; Grubenmann et al. 2004).

Literature References
PubMed ID Title Journal Year
14973782 Congenital disorder of glycosylation type Ik (CDG-Ik): a defect of mannosyltransferase I

Kranz, C, Denecke, J, Lehle, L, Sohlbach, K, Jeske, S, Meinhardt, F, Rossi, R, Gudowius, S, Marquardt, T

Am J Hum Genet 2004
14709599 Deficiency of the first mannosylation step in the N-glycosylation pathway causes congenital disorder of glycosylation type Ik

Grubenmann, CE, Frank, CG, Hülsmeier, AJ, Schollen, E, Matthijs, G, Mayatepek, E, Berger, EG, Aebi, M, Hennet, T

Hum Mol Genet 2004
14973778 Deficiency of GDP-Man:GlcNAc2-PP-dolichol mannosyltransferase causes congenital disorder of glycosylation type Ik

Schwarz, M, Thiel, C, Lübbehusen, J, Dorland, B, de Koning, T, von Figura, K, Lehle, L, Körner, C

Am J Hum Genet 2004
10704531 Cloning of the human cDNA which can complement the defect of the yeast mannosyltransferase I-deficient mutant alg 1

Takahashi, T, Honda, R, Nishikawa, Y

Glycobiology 2000
Participant Of
Catalyst Activity
Catalyst Activity
chitobiosyldiphosphodolichol beta-mannosyltransferase activity of ALG1 [endoplasmic reticulum membrane]
Physical Entity
Orthologous Events
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