title: Characteristics of skeletal muscle in mdx mutant mice.
abstract: We review the extensive research conducted on the mdx mouse since 1987, when demonstration of the absence of dystrophin in mdx muscle led to X-chromosome-linked muscular dystrophy (mdx) being considered as a homolog of Duchenne muscular dystrophy. Certain results are contradictory. We consider most aspects of mdx skeletal muscle: (i) the distribution and roles of dystrophin, utrophin, and associated proteins; (ii) morphological characteristics of the skeletal muscle and hypotheses put forward to explain the regeneration characteristic of the mdx mouse; (iii) special features of the diaphragm; (iv) changes in basic fibroblast growth factor, ion flux, innervation, cytoskeleton, adhesive proteins, mastocytes, and metabolism; and (v) different lines of therapeutic research.
authors.affiliation: Laboratoire de Neurobiologie Cellulaire et Moléculaire, CNRS UPR 9040, Gif sur Yvette, France.
authors.forename: S
authors.initials: S
authors.lastname: De la Porte
authors.forename: S
authors.initials: S
authors.lastname: Morin
authors.forename: J
authors.initials: J
authors.lastname: Koenig
mesh_terms: D000818:Animals; D002415:Cats; D003598:Cytoskeletal Proteins / Q000737:chemistry; D004285:Dogs; D016189:Dystrophin / Q000737:chemistry; D008565:Membrane Proteins / Q000737:chemistry; D051379:Mice; D018101:Mice, Inbred mdx; D018482:Muscle, Skeletal / Q000473:pathology / Q000503:physiopathology*; D009137:Muscular Dystrophy, Animal / Q000503:physiopathology* / Q000628:therapy; D013045:Species Specificity; D049411:Utrophin
chemical_list: D003598:Cytoskeletal Proteins; D016189:Dystrophin; D008565:Membrane Proteins; C483600:Utrn protein, mouse; D049411:Utrophin