title: Disodium cromoglycate protects dystrophin-deficient muscle fibers from leakiness.
abstract: In dystrophin-deficient fibers of mdx mice and in Duchenne dystrophy, the lack of dystrophin leads to sarcolemma breakdown and muscle degeneration. We verified that cromolyn, a mast-cell stabilizer agent, stabilized dystrophic muscle fibers using Evans blue dye as a marker of sarcolemma leakiness. Mdx mice (n=8; 14 days of age) received daily intraperitoneal injections of cromolyn (50 mg/kg body weight) for 15 days. Untreated mdx mice (n=8) were injected with saline. Cryostat cross-sections of the sternomastoid, tibialis anterior, and diaphragm muscles were stained with hematoxylin and eosin. Cromolyn dramatically reduced Evans blue dye-positive fibers in all muscles (P<0.05; Student's t-test) and led to a significant increase in the percentage of fibers with peripheral nuclei. This study supports the protective effects of cromolyn in dystrophic muscles and further indicates its action against muscle fiber leakiness in muscles that are differently affected by the lack of dystrophin.
authors.affiliation: Departamento de Anatomia, Instituto de Biologia, Universidade Estadual de Campinas, Campinas, São Paulo 13083-970, Brazil. marques@unicamp.br
authors.forename: Maria Julia
authors.initials: MJ
authors.lastname: Marques
authors.forename: Rafael
authors.initials: R
authors.lastname: Ventura Machado
authors.forename: Elaine
authors.initials: E
authors.lastname: Minatel
authors.forename: Humberto
authors.initials: H
authors.lastname: Santo Neto
mesh_terms: D000818:Animals; D018927:Anti-Asthmatic Agents / Q000494:pharmacology / Q000627:therapeutic use; D002463:Cell Membrane Permeability / Q000187:drug effects / Q000502:physiology; D004205:Cromolyn Sodium / Q000494:pharmacology* / Q000627:therapeutic use; D004195:Disease Models, Animal; D016189:Dystrophin / Q000172:deficiency* / Q000235:genetics; D005260:Female; D007473:Ion Channels / Q000187:drug effects / Q000235:genetics / Q000378:metabolism; D007477:Ions / Q000378:metabolism; D008297:Male; D008407:Mast Cells / Q000187:drug effects / Q000276:immunology; D051379:Mice; D018101:Mice, Inbred mdx; D018485:Muscle Fibers, Skeletal / Q000187:drug effects / Q000378:metabolism / Q000473:pathology; D018482:Muscle, Skeletal / Q000187:drug effects* / Q000378:metabolism* / Q000473:pathology; D009137:Muscular Dystrophy, Animal / Q000188:drug therapy* / Q000378:metabolism / Q000473:pathology; D020388:Muscular Dystrophy, Duchenne / Q000188:drug therapy* / Q000378:metabolism / Q000473:pathology; D012508:Sarcolemma / Q000187:drug effects / Q000235:genetics / Q000378:metabolism; D016896:Treatment Outcome; D014882:Water-Electrolyte Balance / Q000187:drug effects / Q000235:genetics
chemical_list: D018927:Anti-Asthmatic Agents; D016189:Dystrophin; D007473:Ion Channels; D007477:Ions; D004205:Cromolyn Sodium