Increased calcium-activated neutral protease activity in muscles of dystrophic hamsters and mice

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Abstract

A Ca2+-activated neutral protease activity was examined in muscles of normal and dystrophic hamsters and mice. Light grey and golden brown strains of normal and B10 14.6 strain of dystrophic hamsters were used. Normal and dystrophic mice were of the Bar Harbor 129 ReJ strain. Enzyme activity was measured in the post myofibrillar fraction (homogenate) and in the 75,000 × g pellet (particulate fraction) and supernatant using purified myofibrils.

In normal and dystrophic hamsters or mice, the Ca2+-activated neutral protease was most active in the supernatant followed by the homogenate and particulate fractions. As compared to fractions from normal muscle, enzyme activity was significantly elevated in all 3 fractions from dystrophic muscles of hamsters and mice. Both homogenate and supernatant fractions from muscles of normal hamsters had significantly higher enzyme activity than those of normal mice. Enzyme activity was similar in the particulate fraction. Similarly enzyme activity in the 3 fractions from dystrophic hamster and mouse muscles showed no significant difference.

It is suggested that the Ca2+-activated neutral protease may be involved in muscle fibre necrosis in muscular dystrophy.

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    This study was supported by grants from the Medical Research Council and the Muscular Dystrophy Group of Great Britain.

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