Canine X-linked muscular dystrophy: An animal model of Duchenne muscular dystrophy: Clinical studies

摘要: The progression of clinical disease and serum creatine kinase (CK) levels in canine X-linked muscular dystrophy (CXMD) was studied 7 dogs from birth to 12-14 months 18 at varying intervals 8 weeks. One affected male age 3.5 6 years, all pups were descendants this dog. A lethal neonatal form recognized some pups. In the more typical form, signs stunting, weakness gait abnormalities evident by 6-9 weeks progressive, leading marked muscle atrophy, fibrosis contractures months. Serum CK markedly elevated, such that could be identified 1 week. values increased until 6-8 weeks, then plateaued approx. 100 times normal. Affected females beagle-cross less severely than large breed-cross dogs. 2 adult with cardiac insufficiency had decreased 5-15 These studies show CXMD Duchenne have striking phenotypic as well genotypic similarities. addition, these suggest smaller same genetic defect results a severe disease.