Xylosyl- and glucuronyltransferase LARGE1

Definition

Bifunctional glycosyltransferase with both alpha-1,3-xylosyltransferase and beta-1,3-glucuronyltransferase activities involved in the maturation of alpha-dystroglycan (DAG1) by glycosylation leading to DAG1 binding to laminin G-like domain-containing extracellular proteins with high affinity. Elongates the glucuronyl-beta-1,4-xylose-beta disaccharide primer structure initiated by B4GAT1 by adding repeating units [-3-Xylose-alpha-1,3-GlcA-beta-1-] to produce a heteropolysaccharide. Requires the phosphorylation of core M3 (O-mannosyl trisaccharide) by POMK to elongate the glucuronyl-beta-1,4-xylose-beta disaccharide primer. Plays a key role in skeletal muscle function and regeneration (By similarity).

References:

[1]. Ameya S Walimbe, et al. POMK regulates dystroglycan function via LARGE1-mediated elongation of matriglycan. Elife. 2020 Sep 25;9:e61388. [Content Brief]

[2]. Kei-ichiro Inamori, et al. Dystroglycan function requires xylosyl- and glucuronyltransferase activities of LARGE. Science. 2012 Jan 6;335(6064):93-6. [Content Brief]

[3]. Jeremy L Praissman, et al. B4GAT1 is the priming enzyme for the LARGE-dependent functional glycosylation of α-dystroglycan. Elife. 2014 Oct 3;3:e03943. [Content Brief]

[4]. Katsuya Fujimura, et al. LARGE2 facilitates the maturation of alpha-dystroglycan more effectively than LARGE. Biochem Biophys Res Commun. 2005 Apr 15;329(3):1162-71. [Content Brief]

[5]. Tobias Willer, et al. The glucuronyltransferase B4GAT1 is required for initiation of LARGE-mediated α-dystroglycan functional glycosylation. Elife. 2014 Oct 3;3:e03941. [Content Brief]

[6]. Kei-ichiro Inamori, et al. Xylosyl- and glucuronyltransferase functions of LARGE in α-dystroglycan modification are conserved in LARGE2. Glycobiology. 2013 Mar;23(3):295-302. [Content Brief]

[7]. Martin Brockington, et al. Localization and functional analysis of the LARGE family of glycosyltransferases: significance for muscular dystrophy. Hum Mol Genet. 2005 Mar 1;14(5):657-65. [Content Brief]

[8]. Kei-ichiro Inamori, et al. Endogenous glucuronyltransferase activity of LARGE or LARGE2 required for functional modification of α-dystroglycan in cells and tissues. J Biol Chem. 2014 Oct 10;289(41):28138-48. [Content Brief]

[9]. Yuji Hara, et al. Like-acetylglucosaminyltransferase (LARGE)-dependent modification of dystroglycan at Thr-317/319 is required for laminin binding and arenavirus infection. Proc Natl Acad Sci U S A. 2011 Oct 18;108(42):17426-31. [Content Brief]

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