B4GAT1:LARGE transfers Xyl from UDP-Xyl to GlcA-Xyl-GlcA

Stable Identifier
Reaction [transition]
Homo sapiens
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Glycosyltransferase-like protein LARGE (MIM:603590) is a bifunctional glycosyltransferase with both xylosyltransferase and beta-1,3-glucuronyltransferase activities involved in the biosynthesis of a phosphorylated O-mannosyl trisaccharide (N-acetylgalactosamine-beta-3-N-acetylglucosamine-beta-4-(phosphate-6-)mannose), a structure present in alpha-dystroglycan (DAG1) which plays a key role in skeletal muscle function and regeneration (Inamori et al. 2012, Inamori et al. 2013, Wells 2013). LARGE belongs to the CAZy glycosyltransferase families GT8 and GT49.

Literature References
PubMed ID Title Journal Year
22223806 Dystroglycan function requires xylosyl- and glucuronyltransferase activities of LARGE

Inamori, K, Yoshida-Moriguchi, T, Anderson, ME, Yu, L, Hara, Y, Campbell, KP

Science 2012
23125099 Xylosyl- and glucuronyltransferase functions of LARGE in ?-dystroglycan modification are conserved in LARGE2

Inamori, K, Yoshida-Moriguchi, T, Zhu, Z, Willer, T, Anderson, ME, Hara, Y, Campbell, KP

Glycobiology 2013
23329833 The o-mannosylation pathway: glycosyltransferases and proteins implicated in congenital muscular dystrophy

Wells, L

J. Biol. Chem. 2013
Catalyst Activity

UDP-xylosyltransferase activity of B4GAT1:LARGE [Golgi membrane]

Orthologous Events
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