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.
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
Funk, M, Mumberg, D, Blank, M, Schwarz, RT, Mazhari-Tabrizi, R, Eckert, V
Rush, JS, Wu, X, Freeze, HH, Lubinsky, MS, Karaoglu, D, Waechter, CJ, Gilmore, R, Krasnewich, D
UDP-N-acetylglucosamine-dolichyl-phosphate N-acetylglucosaminephosphotransferase activity of DPAGT1 [endoplasmic reticulum membrane]
© 2023 Reactome
