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Publication : The mdx mouse skeletal muscle myopathy: I. A histological, morphometric and biochemical investigation.

First Author  Coulton GR Year  1988
Journal  Neuropathol Appl Neurobiol Volume  14
Issue  1 Pages  53-70
PubMed ID  2967442 Mgi Jnum  J:152524
Mgi Id  MGI:4359086 Doi  10.1111/j.1365-2990.1988.tb00866.x
Citation  Coulton GR, et al. (1988) The mdx mouse skeletal muscle myopathy: I. A histological, morphometric and biochemical investigation. Neuropathol Appl Neurobiol 14(1):53-70
abstractText  Skeletal muscle has been examined in a colony of the mdx strain of myopathic mice. Sixty-five mice from 22 to 303 days of age, showed extensive and recurrent areas of necrosis and regeneration of muscle fibres, often accompanied by active cellular infiltration. Morphometry of the soleus muscle revealed an abnormal proportion of small and large muscle fibres; over half of the muscle fibres contained 'central' (non-peripheral) nuclei. No histochemical muscle fibre-type grouping was detected. Serum activities of muscle-derived enzymes were greatly elevated in all animals and probably reflect enzyme leakage from damaged muscle fibres. Histological evidence of a cardiomyopathy was found in 13 mice. The mdx myopathy thus shows features seen in Duchenne muscular dystrophy. Mdx differs from Duchenne dystrophy principally in that it exhibits a greater degree of compensatory muscle regeneration and an absence of fibro-fatty replacement of muscle fibres.
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