Muscular Dystrophy in a Dog Resembling Human Becker Muscular Dystrophy

Autor: Serenella Papparella, I. Esposito, A.B. Baroncelli, B. Peirone, Orlando Paciello, F. Abellonio, Teresa Bruna Pagano
Přispěvatelé: Baroncelli, Ab, Abellonio, F, Pagano, Tb, Esposito, I, Peirone, B, Papparella, Serenella, Paciello, Orlando
Rok vydání: 2014
Předmět:
Zdroj: Journal of Comparative Pathology. 150:429-433
ISSN: 0021-9975
Popis: A 3-year-old, male Labrador retriever dog was presented with clinical signs of progressive exercise intolerance, bilateral elbow extension, rigidity of the forelimbs, hindlimb flexion and kyphosis. Microscopical examination of muscle tissue showed marked variability in myofibre size, replacement of muscle with mature adipose tissue and degeneration/regeneration of muscle fibres, consistent with muscular dystrophy. Immunohistochemical examination for dystrophin showed markedly reduced labelling with monoclonal antibodies specific for the rod domain and the carboxy-terminal of dystrophin, while expression of β-sarcoglycan, γ-sarcoglycan and β-dystroglycan was normal. Immunoblotting revealed a truncated dystrophin protein of approximately 135 kDa. These findings supported a diagnosis of congenital canine muscular dystrophy resembling Becker muscular dystrophy in man.
Databáze: OpenAIRE