An ultrastructural study of the vascular and muscular basement membrane in Duchenne-type dystrophy.
The ultrastructural distribution of basement membrane around the capillaries as well as muscle cells of boys with Duchenne-type muscular dystrophy was determined. In dystrophic muscles, a diffuse thickness of vascular as well as muscular basement membrane was observed. Lamina densa lost its regular ribbon-like appearance and was split into several thin layers. After staining with tannic acid (TA), a densely stained meshwork was present on the muscle cell surface as well as in the extra-cellular space. Hyaluronidase treatment removed TA-stained deposits, indicating that hyaluronic acid is a major component. Enzyme resistant structures, presumably fibrous long-spacing collagen fibrils, have been found in dystrophic muscles. Based on the results of the study, faulty structure of the basement membrane in dystrophic muscle is suggested.
Department of Neurology, Medical School, Warszawa, Poland.
A
A
Fidziańska
Z
Z
Glinka
M
M
Walski
D000293:Adolescent; D001485:Basement Membrane / Q000648:ultrastructure; D002196:Capillaries / Q000648:ultrastructure; D002648:Child; D006801:Humans; D008297:Male; D009132:Muscles / Q000098:blood supply* / Q000648:ultrastructure; D009136:Muscular Dystrophies / Q000473:pathology*