Major alteration of the pathological phenotype in gamma irradiated mdx soleus muscles
- Montreal Neurological Institute, McGill University, Quebec (Canada)
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.
- OSTI ID:
- 5448383
- Journal Information:
- Journal of Neuropathology and Experimental Neurology; (United States), Vol. 50:4; ISSN 0022-3069
- Country of Publication:
- United States
- Language:
- English
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MUSCLES
ANIMAL GROWTH
NERVOUS SYSTEM DISEASES
RADIOTHERAPY
TIBIA
BIOLOGICAL RADIATION EFFECTS
GAMMA RADIATION
INHIBITION
MICE
PHENOTYPE
X CHROMOSOME
ANIMALS
BIOLOGICAL EFFECTS
BODY
CHROMOSOMES
DISEASES
ELECTROMAGNETIC RADIATION
GROWTH
HETEROCHROMOSOMES
IONIZING RADIATIONS
MAMMALS
MEDICINE
NUCLEAR MEDICINE
ORGANS
RADIATION EFFECTS
RADIATIONS
RADIOLOGY
RODENTS
SKELETON
THERAPY
VERTEBRATES
560152* - Radiation Effects on Animals- Animals