[Degeneration and regeneration of striated skeletal muscle fibers in Duchenne muscular dystrophy].
Like all other muscular dystrophies, Duchenne muscular dystrophy is characterized by the coexistence of degenerative lesions of the muscle fibers and of regenerative changes. The present study has been carried out in order to precise the degree of regeneration at different stages of the disease, by analyzing the expression of several markers of cell proliferation and of muscular differentiation. In the two affected foetuses of our series, the m. quadriceps is histologically normal, except for the absent expression of immunoreactive dystrophin. The quadriceps from the eight children of our series (20 months-16 years) all present clear dystrophic changes. Muscle regeneration is characterized by activation of the satellite cells, by their multiplication followed by their fusion giving birth to regenerative fibers. By studying the expression of muscular markers (vimentin, desmin, isoforms of the myosin heavy chains), it has been possible to define more precisely the degree of maturation and of differentiation of these regenerative fibers. Our results suggest that an abortive regeneration of the muscle fibers in Duchenne muscular dystrophy can explain, at least partly, the progressive evolution of this disease.
Brasseur
G
G
Laboratoire d'Histologie Embryologie Cytogénétique, Faculté de Médecine Cochin Port-Royal, Paris.
Onolfo
J P
JP
Copin
H
H
Leperchey
F
F
Barbet
J P
JP
D002648:Child; D002675:Child, Preschool; D005315:Fetal Diseases / Q000503:physiopathology*; D005865:Gestational Age; D006801:Humans; D007150:Immunohistochemistry; D018485:Muscle Fibers, Skeletal / Q000502:physiology*; D018482:Muscle, Skeletal / Q000502:physiology*; D009136:Muscular Dystrophies / Q000503:physiopathology*; D012038:Regeneration / Q000502:physiology*