Major alteration of the pathological phenotype in gamma irradiated mdx soleus muscles.
Two thousand rads of gamma irradiation delivered to the lower legs of ten day old normal and x-chromosome linked muscular dystrophy (mdx) mice caused significant inhibition of tibial bone and soleus muscle fiber growth. In the irradiated mdx solei, there was a major loss of muscle fibers, lack of central nucleation, and some endomysial fibrosis. These features were caused by a failure of regeneration of muscle fibers due to impaired proliferative capacity of satellite cells. Gamma irradiation transforms the late pathological phenotype of mdx muscles, so that in one major aspect (muscle fiber loss) they resemble muscles in Duchenne muscular dystrophy. However, extensive endomysial fibrosis which is another characteristic feature of Duchenne muscular dystrophy did not develop. This experimental model could be useful for the functional investigation of possible beneficial effects of therapeutic interventions in mdx dystrophy.
Weller
B
B
Montreal Neurological Institute, McGill University, Quebec, Canada.
Karpati
G
G
Lehnert
S
S
Carpenter
S
S
D000818:Animals; D001846:Bone Development / Q000528:radiation effects; D051379:Mice; D008810:Mice, Inbred C57BL; D024510:Muscle Development; D009132:Muscles / Q000473:pathology* / Q000528:radiation effects; D009137:Muscular Dystrophy, Animal / Q000473:pathology*; D010641:Phenotype