title: Aberrant glycosylation of alpha-dystroglycan causes defective binding of laminin in the muscle of chicken muscular dystrophy.
abstract: Dystroglycan is a central component of dystrophin-glycoprotein complex that links extracellular matrix and cytoskeleton in skeletal muscle. Although dystrophic chicken is well established as an animal model of human muscular dystrophy, the pathomechanism leading to muscular degeneration remains unknown. We show here that glycosylation and laminin-binding activity of alpha-dystroglycan (alpha-DG) are defective in dystrophic chicken. Extensive glycan structural analysis reveals that Galbeta1-3GalNAc and GalNAc residues are increased while Siaalpha2-3Gal structure is reduced in alpha-DG of dystrophic chicken. These results implicate aberrant glycosylation of alpha-DG in the pathogenesis of muscular degeneration in this model animal of muscular dystrophy.
authors.affiliation: Department of Neurology and Neuroscience, Teikyo University, Itabashi-ku, Tokyo, Japan. f-saito@med.teikyo-u.ac.jp
authors.forename: Fumiaki
authors.initials: F
authors.lastname: Saito
authors.forename: Martina
authors.initials: M
authors.lastname: Blank
authors.forename: Jörn
authors.initials: J
authors.lastname: Schröder
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Manya
authors.forename: Teruo
authors.initials: T
authors.lastname: Shimizu
authors.forename: Kevin P
authors.initials: KP
authors.lastname: Campbell
authors.forename: Tamao
authors.initials: T
authors.lastname: Endo
authors.forename: Makoto
authors.initials: M
authors.lastname: Mizutani
authors.forename: Stephan
authors.initials: S
authors.lastname: Kröger
authors.forename: Kiichiro
authors.initials: K
authors.lastname: Matsumura
mesh_terms: D000818:Animals; D002645:Chickens; D002846:Chromatography, Affinity; D004195:Disease Models, Animal; D049030:Dystroglycans / Q000737:chemistry / Q000378:metabolism*; D006031:Glycosylation; D007797:Laminin / Q000378:metabolism*; D018482:Muscle, Skeletal / Q000276:immunology / Q000378:metabolism* / Q000473:pathology; D009137:Muscular Dystrophy, Animal / Q000276:immunology / Q000378:metabolism* / Q000473:pathology; D037121:Plant Lectins / Q000378:metabolism; D011485:Protein Binding
chemical_list: D007797:Laminin; D037121:Plant Lectins; D049030:Dystroglycans