Characteristics of skeletal muscle in mdx mutant mice.
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.
De la Porte
S
S
Laboratoire de Neurobiologie Cellulaire et Moléculaire, CNRS UPR 9040, Gif sur Yvette, France.
Morin
S
S
Koenig
J
J
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
D003598:Cytoskeletal Proteins; D016189:Dystrophin; D008565:Membrane Proteins; C483600:Utrn protein, mouse; D049411:Utrophin