title: Dystrophin and dystrophin-related protein (utrophin) distribution in normal and dystrophin-deficient skeletal muscles.
abstract: The respective localizations of dystrophin and dystrophin-related protein (DRP or utrophin) along the sarcolemmal membrane and at the neuromuscular junctions (NMJs) in normal and dystrophin-deficient skeletal muscles, were determined using confocal laser microscopy. The analysis was prompted by the recent availability of a new anti-utrophin mAb [Bewick et al. NeuroReport 1992; 3:857-860] and different mAbs that react with dystrphin or both dystrophin and utrophin. In dystrophin-deficient muscles, utrophin was expressed and detectable over large subcellular areas normally occupied by dystrophin along the sarcolemmal membranes and at the NMJs. Utrophin was expressed in a non-uniform, discontinuous way on the sarcolemmal membrane in dystrophin-deficient skeletal muscles, similar to dystrophin in normal muscle fibres. The respective distributions of both related muscle proteins and their positions relative to the alpha-bungarotoxin acetylcholine (ACh) receptor marker were determined. Double-staining experiments and superimposition of the confocal images showed that utrophin was more closely associated with ACh receptors than dystrophin at the NMJs in normal muscles. Utrophin distribution consequently differed from that of dystrophin.
authors.affiliation: Institut National de la Santé et de la Recherche Médicale, INSERM U300, Faculté de Pharmacie, Montpellier, France.
authors.forename: F
authors.initials: F
authors.lastname: Pons
authors.forename: L V
authors.initials: LV
authors.lastname: Nicholson
authors.forename: A
authors.initials: A
authors.lastname: Robert
authors.forename: T
authors.initials: T
authors.lastname: Voit
authors.forename: J J
authors.initials: JJ
authors.lastname: Leger
mesh_terms: D000293:Adolescent; D000818:Animals; D003598:Cytoskeletal Proteins / Q000032:analysis* / Q000378:metabolism; D016189:Dystrophin / Q000032:analysis* / Q000172:deficiency / Q000378:metabolism; D005260:Female; D005455:Fluorescent Antibody Technique; D006580:Genetic Carrier Screening; D006801:Humans; D008565:Membrane Proteins; D051379:Mice; D008818:Mice, Neurologic Mutants; D008875:Middle Aged; D009132:Muscles / Q000166:cytology / Q000378:metabolism* / Q000473:pathology; D009136:Muscular Dystrophies / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D009137:Muscular Dystrophy, Animal / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D051381:Rats; D012016:Reference Values; D049411:Utrophin
chemical_list: D003598:Cytoskeletal Proteins; D016189:Dystrophin; D008565:Membrane Proteins; C483600:Utrn protein, mouse; D049411:Utrophin