Bifunctional glycosyltransferase with both xylosyltransferase and beta-1,3-glucuronyltransferase activities involved in the biosynthesis of the phosphorylated O-mannosyl trisaccharide (N-acetylgalactosamine-beta-3-N-acetylglucosamine-beta-4-(phosphate-6-)mannose), a carbohydrate structure present in alpha-dystroglycan (DAG1)
Involvement in disease
Muscular dystrophy-dystroglycanopathy congenital with mental retardation B6 (MDDGB6); Muscular dystrophy-dystroglycanopathy congenital with brain and eye anomalies A6 (MDDGA6)
Golgi apparatus membrane, Single-pass type II membrane protein
Glycosyltransferase 49 family; Glycosyltransferase 8 family
Ubiquitous. Highest expression in heart, brain and skeletal muscle.