title: Propagation in the transverse tubular system and voltage dependence of calcium release in normal and mdx mouse muscle fibres.
abstract: Using a two-microelectrode voltage clamp technique, we investigated possible mechanisms underlying the impaired excitation-contraction coupling in skeletal muscle fibres of the mdx mouse, a model of the human disease Duchenne muscular dystrophy. We evaluated the role of the transverse tubular system (T-system) by using the potentiometric indicator di-8 ANEPPS, and that of the sarcoplasmic reticulum (SR) Ca2+ release by measuring Ca2+ transients with a low affinity indicator in the presence of high EGTA concentrations under voltage clamp conditions. We observed minimal differences in the T-system structure and the T-system electrical propagation was not different between normal and mdx mice. Whereas the maximum Ca2+ release elicited by voltage pulses was reduced by approximately 67% in mdx fibres, in agreement with previous results obtained using AP stimulation, the voltage dependence of SR Ca2+ release was identical to that seen in normal fibres. Taken together, our data suggest that the intrinsic ability of the sarcoplasmic reticulum to release Ca2+ may be altered in the mdx mouse.
authors.affiliation: Department of Physiology, UCLA School of Medicine, Los Angeles, CA 90095, USA.
authors.forename: Christopher E
authors.initials: CE
authors.lastname: Woods
authors.forename: David
authors.initials: D
authors.lastname: Novo
authors.forename: Marino
authors.initials: M
authors.lastname: DiFranco
authors.forename: Joana
authors.initials: J
authors.lastname: Capote
authors.forename: Julio L
authors.initials: JL
authors.lastname: Vergara
mesh_terms: D000200:Action Potentials / Q000502:physiology; D000818:Animals; D002118:Calcium / Q000378:metabolism*; D015220:Calcium Channels / Q000502:physiology*; D020013:Calcium Signaling / Q000502:physiology; D008564:Membrane Potentials / Q000502:physiology; D051379:Mice; D008810:Mice, Inbred C57BL; D018101:Mice, Inbred mdx; D018485:Muscle Fibers, Skeletal / Q000502:physiology*; D018482:Muscle, Skeletal / Q000502:physiology*; D020388:Muscular Dystrophy, Duchenne / Q000503:physiopathology*; D009431:Neural Conduction / Q000502:physiology*; D012519:Sarcoplasmic Reticulum / Q000502:physiology*
chemical_list: D015220:Calcium Channels; D002118:Calcium