Addition of N-acetyl-D-glucosamine to Dolichyl phosphate

Stable Identifier
R-HSA-446191
Type
Reaction [transition]
Species
Homo sapiens
Compartment
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In the first step of N glycan precursor (LLO) synthesis, N acetylglucosamine is added, via an alpha 1,3 linkage, to a molecule of dolichyl phosphate, producing N acetyl D glucosaminyl diphosphodolichol (Eckert et al. 1998). This reaction is catalyzed by DPAGT1 (ALG7 in yeast), mutations in which are associated with CDG disorder type I J (Wu et al. 2003) and with congenital myasthenic syndrome with tubular aggregates type 2 (Belaya et al. 2012). The dolichyl phosphate acts as an anchor for the LLO, so that subsequent sugar addition reactions take place on a sugar anchored in the ER membrane.

Literature References
PubMed ID Title Journal Year
9451016 Cloning and functional expression of the human GlcNAc-1-P transferase, the enzyme for the committed step of the dolichol cycle, by heterologous complementation in Saccharomyces cerevisiae

Eckert, V, Blank, M, Mazhari-Tabrizi, R, Mumberg, D, Funk, M, Schwarz, RT

Glycobiology 1998
22742743 Mutations in DPAGT1 cause a limb-girdle congenital myasthenic syndrome with tubular aggregates

Belaya, K, Finlayson, S, Slater, CR, Cossins, J, Liu, WW, Maxwell, S, McGowan, SJ, Maslau, S, Twigg, SR, Walls, TJ, Pascual Pascual, SI, Palace, J, Beeson, D

Am. J. Hum. Genet. 2012
12872255 Deficiency of UDP-GlcNAc:Dolichol Phosphate N-Acetylglucosamine-1 Phosphate Transferase (DPAGT1) causes a novel congenital disorder of Glycosylation Type Ij

Wu, X, Rush, JS, Karaoglu, D, Krasnewich, D, Lubinsky, MS, Waechter, CJ, Gilmore, R, Freeze, HH

Hum Mutat 2003
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
UDP-N-acetylglucosamine-dolichyl-phosphate N-acetylglucosaminephosphotransferase activity of DPAGT1 [endoplasmic reticulum membrane]
Physical Entity
Activity
Orthologous Events
Authored
Reviewed
Created