Episodic hypoxia exacerbates respiratory muscle dysfunction in DMD(mdx) mice.
Many patients with Duchenne muscular dystrophy (DMD) are eventually diagnosed with sleep-disordered breathing (SDB). SDB is associated with reduced ventilation, decreased arterial oxygen tension, and increased respiratory muscle recruitment during sleep, factors that could be especially detrimental to respiratory muscles in DMD. To assess whether SDB impacts dystrophin-deficient respiratory muscle function and fibrosis, diaphragm strength, and collagen content were evaluated in dystrophic mice (Dmd(mdx)) exposed to experimental SDB. Diurnal exposure to episodic hypoxia resulted in a 30% reduction in diaphragm strength without affecting collagen content. Episodic hypoxia secondary to SDB can exacerbate respiratory muscle dysfunction in DMD.
Farkas
Gaspar A
GA
Department of Exercise and Nutrition Sciences, Room 405, Kimball Tower, School of Public Health and Health Professions, University at Buffalo, Buffalo, New York 14214, USA. farkas@buffalo.edu
McCormick
Kathleen M
KM
Gosselin
Luc E
LE
D000818:Animals; D003964:Diaphragm / Q000503:physiopathology*; D006909:Hydroxyproline / Q000378:metabolism; D000860:Hypoxia / Q000473:pathology* / Q000503:physiopathology*; D066298:In Vitro Techniques; D007551:Isotonic Contraction / Q000502:physiology; D008297:Male; D051379:Mice; D018101:Mice, Inbred mdx; D009137:Muscular Dystrophy, Animal / Q000473:pathology* / Q000503:physiopathology*; D010812:Physical Stimulation / Q000379:methods
D006909:Hydroxyproline