B. Lucasheron et al., MDX MOUSE SKELETAL-MUSCLE - COULD A MITOCHONDRIAL FACTOR BE RESPONSIBLE FOR THE ABSENCE OF PROGRESSIVE NECROSIS, Neuroscience letters, 169(1-2), 1994, pp. 97-100
We compared the myotoxic effect of chlorpromazine on mitochondria of g
astrocnemius muscle in X-related muscular dystrophy (mdx) and control
mice relative to changes in calmitine and calcium concentrations befor
e and 3 and 6 days after a single injection of the drug. The results i
ndicate that mdx mouse mitochondria are less sensitive to the myotoxic
effect of chlorpromazine; calmitine and calcium binding were only sli
ghtly reduced compared to controls. Our observations indicate that the
calmitine structure could differ in mdx and control mice with respect
to calcium binding structures, and that the presence of calmitine in
the mitochondria of mdx mouse skeletal muscle could explain why muscle
degeneration does not occur in these animals. However, the muscles of
patients with Duchenne muscular dystrophy (DMD) are lacking in calmit
ine and are subject to extensive progressive degeneration.