title: Muscular dystrophy in a dog resembling human becker muscular dystrophy.
abstract: 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.
authors: Department of Animal Pathology, College of Veterinary Medicine, University of Turin, Napoli, Italy; Clinica Albese per Animali da Compagnia, Alba, Napoli, Italy.
authors: A B
authors: AB
authors: Baroncelli
authors: Clinica Albese per Animali da Compagnia, Alba, Napoli, Italy.
authors: F
authors: F
authors: Abellonio
authors: Department of Veterinary Medicine and Animal Production, Laboratory of Comparative Neuromuscular Diseases, University of Naples Federico II, Napoli, Italy. Electronic address: teresabruna@hotmail.it.
authors: T B
authors: TB
authors: Pagano
authors: Department of Veterinary Medicine and Animal Production, Laboratory of Comparative Neuromuscular Diseases, University of Naples Federico II, Napoli, Italy.
authors: I
authors: I
authors: Esposito
authors: Department of Animal Pathology, College of Veterinary Medicine, University of Turin, Napoli, Italy.
authors: B
authors: B
authors: Peirone
authors: Department of Veterinary Medicine and Animal Production, Laboratory of Comparative Neuromuscular Diseases, University of Naples Federico II, Napoli, Italy.
authors: S
authors: S
authors: Papparella
authors: Department of Veterinary Medicine and Animal Production, Laboratory of Comparative Neuromuscular Diseases, University of Naples Federico II, Napoli, Italy.
authors: O
authors: O
authors: Paciello
mesh_terms: D000818:Animals; D004283:Dog Diseases / Q000378:metabolism / Q000473:pathology*; D004285:Dogs; D016189:Dystrophin / Q000378:metabolism; D008297:Male; D018482:Muscle, Skeletal / Q000378:metabolism / Q000473:pathology*; D009137:Muscular Dystrophy, Animal / Q000378:metabolism / Q000473:pathology*; D020388:Muscular Dystrophy, Duchenne / Q000378:metabolism / Q000473:pathology
chemical_list: D016189:Dystrophin
keywords: dog; dystrophin; muscular dystrophy; myopathy