title: Mdx myotubes have normal excitability but show reduced contraction-relaxation dynamics.
abstract: The pathogenesis of Duchenne muscular dystrophy (DMD), characterised by lack of the cytoskeletal protein dystrophin, is not completely understood. An early event in the degenerative process of DMD muscle could be a rise in cytosolic calcium concentration. In order to investigate whether this leads to alterations of contractile behaviour, we studied the excitability and contractile properties of cultured myotubes from control (C57BL/10) and mdx mice, an animal model for DMD. The myotubes were stimulated electrically and their motion was recorded photometrically. No significant differences were found between control and mdx myotubes with respect to the following parameters: chronaxy and rheobase (0.33 +/- 0.03 ms and 23 +/- 4 V vs. 0.39 +/- 0.07 ms and 22 +/- 2 V for C57 and mdx myotubes, respectively), tetanisation frequency (a similar distribution pattern was found between 5 and 30 Hz), fatigue during tetanus (found in 35% of both types of myotubes) and post-tetanic contracture. In contrast, contraction and relaxation times were longer (P < 0.005) in mdx (36 +/- 2 and 142 +/- 13 ms, respectively) than in control myotubes (26 +/- 1 and 85 +/- 9 ms, respectively). Together with our earlier findings, these results suggest a decreased capacity for calcium removal in mdx cells leading, in particular, to alterations of muscle relaxation.
authors.affiliation: Group of Pharmacology, School of Pharmacy, University of Lausanne, Switzerland.
authors.forename: V
authors.initials: V
authors.lastname: Nicolas-Metral
authors.forename: E
authors.initials: E
authors.lastname: Raddatz
authors.forename: P
authors.initials: P
authors.lastname: Kucera
authors.forename: U T
authors.initials: UT
authors.lastname: Ruegg
mesh_terms: D000818:Animals; D000831:Animals, Newborn; D002118:Calcium / Q000378:metabolism*; D016189:Dystrophin / Q000172:deficiency / Q000235:genetics / Q000502:physiology*; D004558:Electric Stimulation; D006801:Humans; D008297:Male; D051379:Mice; D008810:Mice, Inbred C57BL; D018101:Mice, Inbred mdx; D009119:Muscle Contraction; D018763:Muscle Fatigue; D009126:Muscle Relaxation; D009137:Muscular Dystrophy, Animal / Q000235:genetics / Q000503:physiopathology*; D020388:Muscular Dystrophy, Duchenne / Q000503:physiopathology*; D009924:Organ Culture Techniques
chemical_list: D016189:Dystrophin; D002118:Calcium