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

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

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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
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