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

Stable Identifier
Reaction [transition]
Homo sapiens
Locations in the PathwayBrowser
SVG |   | PPTX  | SBGN
Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout

Glycosyltransferase-like protein LARGE is a bifunctional glycosyltransferases with both xylosyltransferase and beta-1,3-glucuronyltransferase activities which can form polymeric Xyl-GlcA repeats. LARGE is involved in the physiological function of alpha-dystroglycan (DAG1) which plays a key role in skeletal muscle function and regeneration (Inamori et al. 2013, Wells 2013, Inamori et al. 2014).

Literature References
PubMed ID Title Journal Year
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
25138275 Endogenous glucuronyltransferase activity of LARGE or LARGE2 required for functional modification of ╬▒-dystroglycan in cells and tissues

Inamori, K, Moore, SA, B├Ânnemann, CG, Clarke, NF, Willer, T, Guicheney, P, Anderson, ME, Hara, Y, Campbell, KP, Venzke, D

J. Biol. Chem. 2014
23329833 The o-mannosylation pathway: glycosyltransferases and proteins implicated in congenital muscular dystrophy

Wells, L

J. Biol. Chem. 2013
Catalyst Activity

glucuronosyltransferase activity of B4GAT1:LARGE [Golgi membrane]

Orthologous Events
Cite Us!