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Hereditary Canine Spinal Muscular Atrophy: An Animal Model of Motor Neuron Disease

Published online by Cambridge University Press:  18 September 2015

Linda C. Cork
Linda C. Cork*
Affiliation:
Division of Comparative Medicine, The Johns Hopkins University School of Medicine, Baltimore
*
Division of Comparative Medicine, John Hopkins University School of Medicine, Baltimore, Maryland, U.S.A. 21205
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Abstract:

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Motor neuron diseases selectively produce degeneration and death of motor neurons; the pathogenesis of these disorders and the specificity for this population of neurons are unknown. Hereditary Canine Spinal Muscular Atrophy produces a lower motor neuron disease which is clinically and pathologically similar to human motor neuron disease: motor neurons dysfunction and degenerate. The canine model provides an opportunity to investigate early stages of disease when there are viable motor neurons still present and might be responsive to a variety of therapeutic interventions. The canine disease, like the human disease, is inherited as an autosomal dominant. The extensive canine pedigree of more than 200 characterized individuals permits genetic analysis using syntenic linkage techniques which may identify a marker for the canine trait and provide insights into homologous regions for study in human kindreds.

Type
Research Article
Information
Canadian Journal of Neurological Sciences , Volume 18 , Issue S3 , August 1991 , pp. 432 - 434
Copyright
Copyright © Canadian Neurological Sciences Federation 1991

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