Autosomal dominant inheritance of hereditary canine spinal muscular atrophy

George Henry Sack, L. C. Cork, J. M. Morris, J. W. Griffin, D. L. Price

Research output: Contribution to journalArticle

Abstract

Hereditary canine spinal muscular atrophy (HCSMA) is a motor neuron disease in Brittany spaniels. Three phenotypes are recognized (accelerated, intermediate, and chronic) and are distinguished on the basis of rate of progression and age at onset. Breeding studies within a kindred of more than 125 dogs (Brittany spaniel and beagle-Brittany outcrosses) have established an autosomal dominant inheritance for HCSMA. Pups homozygous for the trait have accelerated disease, whereas heterozygous dogs have intermediate or chronic disease. The reason for the two phenotypes in heterozygous is under study. HCSMA provides a unique opportunity to study the genetic and pathophysiological mechanisms of a motor neuron disease, and findings may have broad relevance to investigations of autosomal dominant degenerative disorders of the central nervous system.

Original languageEnglish (US)
Pages (from-to)369-373
Number of pages5
JournalAnnals of Neurology
Volume15
Issue number4
StatePublished - 1984

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Spinal Muscular Atrophy
Canidae
Motor Neuron Disease
Dogs
Phenotype
Central Nervous System Diseases
Age of Onset
Breeding
Chronic Disease

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Sack, G. H., Cork, L. C., Morris, J. M., Griffin, J. W., & Price, D. L. (1984). Autosomal dominant inheritance of hereditary canine spinal muscular atrophy. Annals of Neurology, 15(4), 369-373.

Autosomal dominant inheritance of hereditary canine spinal muscular atrophy. / Sack, George Henry; Cork, L. C.; Morris, J. M.; Griffin, J. W.; Price, D. L.

In: Annals of Neurology, Vol. 15, No. 4, 1984, p. 369-373.

Research output: Contribution to journalArticle

Sack, GH, Cork, LC, Morris, JM, Griffin, JW & Price, DL 1984, 'Autosomal dominant inheritance of hereditary canine spinal muscular atrophy', Annals of Neurology, vol. 15, no. 4, pp. 369-373.
Sack, George Henry ; Cork, L. C. ; Morris, J. M. ; Griffin, J. W. ; Price, D. L. / Autosomal dominant inheritance of hereditary canine spinal muscular atrophy. In: Annals of Neurology. 1984 ; Vol. 15, No. 4. pp. 369-373.
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