Muscular Dystrophy in a Dog Resembling Human Becker Muscular Dystrophy

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...

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Bibliographic Details
Published in:Journal of comparative pathology 2014-05, Vol.150 (4), p.429-433
Main Authors: Baroncelli, A.B., Abellonio, F., Pagano, T.B., Esposito, I., Peirone, B., Papparella, S., Paciello, O.
Format: Article
Language:English
Subjects:
Animals
dog
Dog Diseases - metabolism
Dog Diseases - pathology
Dogs
dystrophin
Dystrophin - metabolism
Male
Muscle, Skeletal - metabolism
Muscle, Skeletal - pathology
muscular dystrophy
Muscular Dystrophy, Animal - metabolism
Muscular Dystrophy, Animal - pathology
Muscular Dystrophy, Duchenne - metabolism
Muscular Dystrophy, Duchenne - pathology
myopathy
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Summary: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.
ISSN:0021-9975
1532-3129
DOI:10.1016/j.jcpa.2013.12.006