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

Stable Identifier
R-HSA-446191
Type
Reaction
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
22742743 Mutations in DPAGT1 cause a limb-girdle congenital myasthenic syndrome with tubular aggregates

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

Am. J. Hum. Genet. 2012
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

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

Glycobiology 1998
12872255 Deficiency of UDP-GlcNAc:Dolichol Phosphate N-Acetylglucosamine-1 Phosphate Transferase (DPAGT1) causes a novel congenital disorder of Glycosylation Type Ij

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

Hum Mutat 2003
Participants
Participates
Catalyst Activity

UDP-N-acetylglucosamine-dolichyl-phosphate N-acetylglucosaminephosphotransferase activity of DPAGT1 [endoplasmic reticulum membrane]

Orthologous Events
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