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.
Eckert, V, Blank, M, Mazhari-Tabrizi, R, Mumberg, D, Funk, M, Schwarz, RT
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
Wu, X, Rush, JS, Karaoglu, D, Krasnewich, D, Lubinsky, MS, Waechter, CJ, Gilmore, R, Freeze, HH
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