Collagen types in neuromuscular diseases

Abstract

The striking proliferation of connective tissue characteristic of the muscular dystrophies can be attributed predominantly to an increase in endomysial and perimysial type III collagen. Carriers of muscular dystrophy occasionally revealed a slight increase in anti-type III collagen fluorescence, but no abnormalities in collagen disposition were observed in foetuses "at risk" for DMD. In contrast, the proportion of collagen types in neurogenic atrophies appeared normal although anti-type IV and V staining, which delineated the basement membrane, was very intense around atrophied fibres, as was also the case in small fibres in myopathic diseases. The detection of staining with anti-type III, IV and V collagens in splits which are sometimes observed in hypertrophied fibres in the muscular dystrophies supports the suggestion that abnormalities in collagen production, perhaps involving a defective modulation of myoblast-fibroblast expression, may be involved in the pathogenesis of these diseases.