Function | 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. |
Protein Name | Xylosyl- And Glucuronyltransferase Large1Acetylglucosaminyltransferase-Like 1aGlycosyltransferase-Like ProteinLarge Xylosyl- And Glucuronyltransferase 1 Includes - Alpha-1 -3-Xylosyltransferase Large1 - Beta-1 -3-Glucuronyltransferase Large1 |
Database Links | Reactome: R-HSA-5083627Reactome: R-HSA-5173105 |
Cellular Localisation | Golgi Apparatus MembraneSingle-Pass Type Ii Membrane Protein |
Alternative Antibody Names | Anti-Xylosyl- And Glucuronyltransferase Large1 antibodyAnti-Acetylglucosaminyltransferase-Like 1a antibodyAnti-Glycosyltransferase-Like Protein antibodyAnti-Large Xylosyl- And Glucuronyltransferase 1 Includes - Alpha-1 -3-Xylosyltransferase Large1 - Beta-1 -3-Glucuronyltransferase Large1 antibodyAnti-LARGE1 antibodyAnti-KIAA0609 antibodyAnti-LARGE antibody |
Information sourced from Uniprot.org