Canine X-linked muscular dystrophy as an animal model of Duchenne muscular dystrophy: A review

Authors

  • Dr. Beth A. Valentine,

    Corresponding author
    1. Department of Pathology, New York State College of Veterinary Medicine, Cornell University, Ithaca, New York
    • Department of Pathology, New York State College of Veterinary Medicine, Cornell University, Ithaca, NY 14853-6401
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  • Nena J. Winand,

    1. Department of Pathology, New York State College of Veterinary Medicine, Cornell University, Ithaca, New York
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  • Deepti Pradhan,

    1. Department of Pathology, New York State College of Veterinary Medicine, Cornell University, Ithaca, New York
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  • N. Sydney Moise,

    1. Department of Clinical Sciences, New York State College of Veterinary Medicine, Cornell University, Ithaca, New York
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  • Alexander de Lahunta,

    1. Department of Anatomy, New York State College of Veterinary Medicine, Cornell University, Ithaca, New York
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  • Joe N. Kornegay,

    1. Department of Companion Animal and Special Species Medicine, College of Veterinary Medicine, North Carolina State University, Raleigh, North Carolina
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  • Barry J. Cooper

    1. Department of Pathology, New York State College of Veterinary Medicine, Cornell University, Ithaca, New York
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Abstract

Canine X-linked muscular dystrophy is a spontaneously occurring, progressive, degenerative myopathy of dogs that is clinically and pathologically similar to Duchenne muscular dystrophy in man. The molecular basis for the disease has been shown to be a lack of dystrophin, the protein product of the Duchenne muscular dystrophy gene. Breeding colonies of dystrophic dogs have been established. This report reviews the findings of genetic, clinical, pathologic, molecular biologic, and immunocytochemical studies of the canine model, and compares the features of the canine disease to those of Duchenne dystrophy in man.

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