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

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

Stable Identifier

R-HSA-9638097

Type

Reaction [transition]

Species

Homo sapiens

Compartment

Golgi lumen, Golgi membrane

ReviewStatus

5/5

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General

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

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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 α-dystroglycan in cells and tissues Inamori, K, Moore, SA, Bönnemann, 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