Mast cell proliferation and alterations in bFGF amount and localization are involved in the response of muscle to dystrophin deficiency in hypertrophic feline dystrophy.

To test the hypothesis that basic fibroblast growth factor and mast cells play a key role in the phenotypic differences between human dystrophinopathies and hypertrophic feline muscular dystrophy, serial sections of dystrophin-deficient, carrier and normal cat muscle biopsy specimens were examined. They were stained immunohistochemically for dystrophin and different markers of differentiation such as desmin, vimentin and utrophin. Basic fibroblast growth factor was increased in the myofibers of dystrophic cats compared to normal controls and carriers. An association of basic fibroblast growth factor with fiber regeneration and necrosis was shown. The amount of mast cells was markedly increased in muscle tissue of dystrophic cats with a clear predominance of tryptase-positive cells present in large amounts in the endomysium. Mast cells, like basic fibroblast growth factor, were concentrated in areas of muscle fiber regeneration and necrosis. Our data concerning basic fibroblast growth factor and mast cells are consistent with a highly abnormal cellular environment in feline dystrophic muscle with very high levels of basic fibroblast growth factor which is likely modulated by mast cells.