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

Stable Identifier
R-HSA-446191
Type
Reaction [transition]
Species
Homo sapiens
Compartment
endoplasmic reticulum membrane, cytosol
ReviewStatus
5/5
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Addition of N-acetyl-D-glucosamine to Dolichyl phosphate
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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

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
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
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
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Catalyst Activity

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

Physical Entity
DPAGT1 [endoplasmic reticulum membrane] (Homo sapiens)
Activity
UDP-N-acetylglucosamine-dolichyl-phosphate N-acetylglucosaminephosphotransferase activity (GO:0003975)
Orthologous Events
Addition of N-acetyl-D-glucosamine to Dolichyl phosphate (Bos taurus)
Addition of N-acetyl-D-glucosamine to Dolichyl phosphate (Caenorhabditis elegans)
Addition of N-acetyl-D-glucosamine to Dolichyl phosphate (Canis familiaris)
Addition of N-acetyl-D-glucosamine to Dolichyl phosphate (Danio rerio)
Addition of N-acetyl-D-glucosamine to Dolichyl phosphate (Dictyostelium discoideum)
Addition of N-acetyl-D-glucosamine to Dolichyl phosphate (Drosophila melanogaster)
Addition of N-acetyl-D-glucosamine to Dolichyl phosphate (Gallus gallus)
Addition of N-acetyl-D-glucosamine to Dolichyl phosphate (Mus musculus)
Addition of N-acetyl-D-glucosamine to Dolichyl phosphate (Plasmodium falciparum)
Addition of N-acetyl-D-glucosamine to Dolichyl phosphate (Rattus norvegicus)
Addition of N-acetyl-D-glucosamine to Dolichyl phosphate (Saccharomyces cerevisiae)
Addition of N-acetyl-D-glucosamine to Dolichyl phosphate (Schizosaccharomyces pombe)
Addition of N-acetyl-D-glucosamine to Dolichyl phosphate (Sus scrofa)
Addition of N-acetyl-D-glucosamine to Dolichyl phosphate (Xenopus tropicalis)
Authored
Dall'Olio, GM  (2009-11-10)
Reviewed
Gagneux, P  (2010-04-16)
Created
Jassal, B  (2009-11-10)
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