Xylosyl- and glucuronyltransferase functions of LARGE in α-dystroglycan modification are conserved in LARGE2

Glycobiology. 2013 Mar;23(3):295-302. doi: 10.1093/glycob/cws152. Epub 2012 Nov 2.

Abstract

LARGE-dependent modification enables α-dystroglycan (α-DG) to bind to its extracellular matrix ligands. Mutations in the LARGE gene and several others involved in O-mannosyl glycan synthesis have been identified in congenital and limb-girdle muscular dystrophies that are characterized by perturbed glycosylation and reduced ligand-binding affinity of α-DG. LARGE is a bifunctional glycosyltransferase that alternately transfers xylose and glucuronic acid, thereby generating the heteropolysaccharides on α-DG that confer its ligand binding. Although the LARGE paralog LARGE2 (also referred to as GYLTL1B) has likewise been shown to enhance the functional modification of α-DG in cultured cells, its enzymatic activities have not been identified. Here, we report that LARGE2 is also a bifunctional glycosyltransferase and compare its properties with those of LARGE. By means of a high-performance liquid chromatography-based enzymatic assay, we demonstrate that like LARGE, LARGE2 has xylosyltransferase (Xyl-T) and glucuronyltransferase (GlcA-T) activities, as well as polymerizing activity. Notably, however, the pH optima of the Xyl-T and GlcA-T of LARGE2 are distinct from one another and also from those of LARGE. Our results suggest that LARGE and LARGE2 catalyze the same glycosylation reactions for the functional modification of α-DG, but that they have different biochemical properties.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • CHO Cells
  • Catalytic Domain
  • Cricetinae
  • Cricetulus
  • Dystroglycans / metabolism*
  • Glucuronic Acid / metabolism
  • Glycosyltransferases / chemistry
  • Glycosyltransferases / metabolism*
  • Hydrogen-Ion Concentration
  • Kinetics
  • Mice
  • N-Acetylglucosaminyltransferases / chemistry
  • N-Acetylglucosaminyltransferases / metabolism*
  • Protein Multimerization
  • Xylose / metabolism

Substances

  • Dystroglycans
  • Glucuronic Acid
  • Xylose
  • Glycosyltransferases
  • Large1 protein, mouse
  • Large2 protein, mouse
  • N-Acetylglucosaminyltransferases