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Regeneration‐blocked mdx muscle: in vivo model for testing treatments
Author(s) -
Quinlan John G.,
Cambier Denise,
Lyden Sean,
Dalvi Arif,
Upputuri Ram K.,
Gartside Peter,
Michaels Scott E.,
Denman David
Publication year - 1997
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/(sici)1097-4598(199708)20:8<1016::aid-mus12>3.0.co;2-t
Subject(s) - duchenne muscular dystrophy , wasting , regeneration (biology) , muscular dystrophy , medicine , dystrophy , in vivo , mdx mouse , anatomy , skeletal muscle , pathology , biology , endocrinology , dystrophin , microbiology and biotechnology
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. © 1997 John Wiley & Sons, Inc. Muscle Nerve 20: 1016–1023, 1997