2. Academic Validation
  3. Dystroglycan function requires xylosyl- and glucuronyltransferase activities of LARGE

Dystroglycan function requires xylosyl- and glucuronyltransferase activities of LARGE

  • Science. 2012 Jan 6;335(6064):93-6. doi: 10.1126/science.1214115.
Kei-ichiro Inamori 1,  Takako Yoshida-Moriguchi,  Yuji Hara,  Mary E Anderson,  Liping Yu,  Kevin P Campbell
Affiliations

Affiliation

  • 1 Howard Hughes Medical Institute, Department of Molecular Physiology and Biophysics, University of Iowa Roy J. and Lucille A. Carver College of Medicine, 4283 Carver Biomedical Research Building, 285 Newton Road, Iowa City, IA 52242-1101, USA.
PMID: 22223806 DOI: 10.1126/science.1214115
Abstract

Posttranslational modification of alpha-dystroglycan (α-DG) by the like-acetylglucosaminyltransferase (LARGE) is required for it to function as an extracellular matrix (ECM) receptor. Mutations in the LARGE gene have been identified in congenital muscular dystrophy patients with brain abnormalities. However, the precise function of LARGE remains unclear. Here we found that LARGE could act as a bifunctional Glycosyltransferase, with both xylosyltransferase and glucuronyltransferase activities, which produced repeating units of [-3-xylose-α1,3-glucuronic acid-β1-]. This modification allowed α-DG to bind laminin-G domain-containing ECM ligands.

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