Electrical properties of diaphragm and EDL muscles during the life of dystrophic mice.

Abstract

The membrane electrical properties of diaphragm and extensor digitorum longus (EDL) muscle fibers of dystrophic mdx and control mice from 4 wk to 14-19 mo of age were recorded with the intracellular microelectrode technique. Up to 8 wk of age, the diaphragm and EDL muscles did not differ between the two strains. From 8 up to 20 wk, the mdx diaphragm fibers showed a higher membrane resistance (Rm), which was due to significantly lower values of resting chloride conductance (GCl) and an overexcitability with respect to age-matched controls. Oppositely, the mdx EDL muscle fibers had significantly lower Rm and higher GCl values than age-related controls at 8, 10, and 13 wk, along with a decreased membrane excitability. These differences were no longer detectable at 20 wk. The diaphragm and EDL muscles from 14- to 19-mo-old controls showed a decrease of GCl and an increase of potassium conductance with respect to adult animals. In aged mdx animals, these changes were very dramatic in diaphragm fibers, whereas no differences, with respect to adults, were found in the EDL muscle. Thus GCl is an index of the dystrophic condition of mdx muscles. In the degenerating diaphragm, the impairment of GCl can account for some of the pathological features of the muscle. In the EDL muscle, the changes of GCl can follow the high regenerative potential of the hindlimb muscles of the mdx phenotype.