Regeneration-blocked mdx muscle: in vivo model for testing treatments.
We have refined the mdx mouse as a clinical model for Duchenne dystrophy. Our power estimates, primary measures, regular sacrifice intervals, and quality checks constitute a high-speed, low-cost system for preclinically testing therapies designed to slow muscle destruction in Duchenne dystrophy. Irradiated (18 Gy) and contralateral shielded anterior tibial muscles were studied in 21-day-old mdx and normal mice at the time of irradiation and 4, 8, 12, 16, and 20 weeks thereafter. Regeneration-blocked mdx (irradiated) muscle expressed muscular dystrophy as progressive wasting after a brief (4 week) period of growth. Regeneration-blocked normal muscle showed stunted growth but neither progressive wasting nor microscopic pathological changes.
Quinlan
J G
JG
Department of Neurology, University of Cincinnati, Ohio 45237-0525, USA.
Cambier
D
D
Lyden
S
S
Dalvi
A
A
Upputuri
R K
RK
Gartside
P
P
Michaels
S E
SE
Denman
D
D
D000818:Animals; D001284:Atrophy; D004195:Disease Models, Animal; D016189:Dystrophin / Q000172:deficiency / Q000235:genetics; D015870:Gene Expression / Q000502:physiology; D006614:Hindlimb; D051379:Mice; D008810:Mice, Inbred C57BL; D018101:Mice, Inbred mdx; D018485:Muscle Fibers, Skeletal / Q000473:pathology / Q000502:physiology* / Q000528:radiation effects; D018482:Muscle, Skeletal / Q000473:pathology / Q000502:physiology* / Q000528:radiation effects; D009136:Muscular Dystrophies / Q000628:therapy; D009137:Muscular Dystrophy, Animal / Q000235:genetics / Q000473:pathology / Q000503:physiopathology*; D010641:Phenotype; D012038:Regeneration / Q000528:radiation effects*
D016189:Dystrophin