Reactome | B4GAT1:GYLTL1B transfers Xyl from UDP-Xyl to GlcA-Xyl-GlcA

B4GAT1:GYLTL1B transfers Xyl from UDP-Xyl to GlcA-Xyl-GlcA

Stable Identifier

R-HSA-5617138

Type

Reaction [transition]

Species

Homo sapiens

Compartment

Golgi lumen, Golgi membrane

ReviewStatus

5/5

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B4GAT1:GYLTL1B transfers Xyl from UDP-Xyl to GlcA-Xyl-GlcA

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Glycosyltransferase-like protein LARGE2 (GYLTL1B; MIM:609709) 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). LARGE2 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