Propagation in the transverse tubular system and voltage dependence of calcium release in normal and mdx mouse muscle fibres

J Physiol. 2005 Nov 1;568(Pt 3):867-80. doi: 10.1113/jphysiol.2005.089318. Epub 2005 Aug 25.

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.

Publication types

  • Comparative Study
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Action Potentials / physiology
  • Animals
  • Calcium / metabolism*
  • Calcium Channels / physiology*
  • Calcium Signaling / physiology
  • Membrane Potentials / physiology
  • Mice
  • Mice, Inbred C57BL
  • Mice, Inbred mdx
  • Muscle Fibers, Skeletal / physiology*
  • Muscle, Skeletal / physiology*
  • Muscular Dystrophy, Duchenne / physiopathology*
  • Neural Conduction / physiology*
  • Sarcoplasmic Reticulum / physiology*

Substances

  • Calcium Channels
  • Calcium