Abstract
The extensive proliferation of connective tissue in muscular dystrophy caused Duchenne1 to term it ‘paralysie myosclerosique’. Surprisingly, there has been little interest in the pathogenesis of this marked fibrosis or of the fat replacement in dystrophic muscle. The fibrosis has generally been considered secondary, with various hypotheses2 to explain the fundamental cause of muscular dystrophy. Several authors have commented on the increased endomysial stroma early in the disease before any apparent muscle degeration3,4 and have suggested that there might be an aberration in the production of connective tissue in muscular dystrophy and that the thickening pericellular reticulum would adversely affect muscle nutrition5,6. lonasescu and his collaborators found an over-production of connective tissue with a concomitant decrease in muscle protein synthesis both by polyribosomes from skeletal muscle and by tissue culture of muscle tissue and skin fibroblasts from patients suffering from Duchenne muscular dystrophy7–9. Thomson et al.10 have observed in tissue culture that dissociated muscle from patients with Duchenne and Becker muscular dystrophy will form unusual clusters of ‘sticky’ cells, which they suggested may reflect an abnormal collagen production. These findings have cast doubts on the interpretation that the extensive connective tissue proliferation characteristic of Duchenne muscular dystrophy, and also seen in limb-girdle, Becker and congenital dystrophies, is simply due to a compensatory replacement of the wasting muscle11 but infers a more primary role for the connective tissue collagen. Only recently has the role of connective tissue in developing muscle come to be appreciated12–14. Moreover, immunofluorescent techniques14–16, now allow investigation of the various types of collagen present in skeletal muscle. We report here on the localisation and change in proportion of collagen types I, III, IV and V in muscle from patients with various forms of neuromuscular disease, and propose a more positive role for collagen.
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Duance, V., Stephens, H., Dunn, M. et al. A role for collagen in the pathogenesis of muscular dystrophy?. Nature 284, 470–472 (1980). https://doi.org/10.1038/284470a0
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DOI: https://doi.org/10.1038/284470a0
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