Hereditary canine spinal muscular atrophy

Linda Collins Cork, John W. Griffin, John F. Munnell, Michael D. Lorenz, Robert John Adams, Donald L. Price

Research output: Contribution to journalArticle

Abstract

Hereditary canine spinal muscular atrophy is a newly recognized motor neuron disease occurring in Brittany Spaniels. The clinical manifestations, pattern of inheritance, electrodiagnostic findings, and muscle biopsies have features in common with human spinal muscular atrophy. Neuropathological examination discloses some loss of motor neurons in the spinal cord and brainstem. Many of the surviving motor neurons have neurofibrillary swellings in proximal axons, an abnormality similar to that which occurs early in the course of human amyotrophic lateral sclerosis. These axonal swellings are filled with maloriented skeins of neurofilaments. Since the proteins comprising neurofilaments are carried by slow axonal transport, their accumulation within axons suggest that the swellings may result from impaired slow transport, a hypothesis that can be tested in affected Brittany Spaniels. Hereditary canine spinal muscular atrophy is a new genetic, clinical, and pathological entity, and, at present, it appears to be the best currently available animal model of motor neuron disease.

Original languageEnglish (US)
Pages (from-to)209-221
Number of pages13
JournalJournal of Neuropathology and Experimental Neurology
Volume38
Issue number3
StatePublished - 1979

Fingerprint

Spinal Muscular Atrophy
Canidae
Motor Neuron Disease
Motor Neurons
Axons
Neurofilament Proteins
Inheritance Patterns
Axonal Transport
Intermediate Filaments
Amyotrophic Lateral Sclerosis
Brain Stem
Spinal Cord
Animal Models
Biopsy
Muscles

ASJC Scopus subject areas

  • Clinical Neurology
  • Pathology and Forensic Medicine
  • Cellular and Molecular Neuroscience
  • Neurology
  • Neuroscience(all)

Cite this

Cork, L. C., Griffin, J. W., Munnell, J. F., Lorenz, M. D., Adams, R. J., & Price, D. L. (1979). Hereditary canine spinal muscular atrophy. Journal of Neuropathology and Experimental Neurology, 38(3), 209-221.

Hereditary canine spinal muscular atrophy. / Cork, Linda Collins; Griffin, John W.; Munnell, John F.; Lorenz, Michael D.; Adams, Robert John; Price, Donald L.

In: Journal of Neuropathology and Experimental Neurology, Vol. 38, No. 3, 1979, p. 209-221.

Research output: Contribution to journalArticle

Cork, LC, Griffin, JW, Munnell, JF, Lorenz, MD, Adams, RJ & Price, DL 1979, 'Hereditary canine spinal muscular atrophy', Journal of Neuropathology and Experimental Neurology, vol. 38, no. 3, pp. 209-221.
Cork LC, Griffin JW, Munnell JF, Lorenz MD, Adams RJ, Price DL. Hereditary canine spinal muscular atrophy. Journal of Neuropathology and Experimental Neurology. 1979;38(3):209-221.
Cork, Linda Collins ; Griffin, John W. ; Munnell, John F. ; Lorenz, Michael D. ; Adams, Robert John ; Price, Donald L. / Hereditary canine spinal muscular atrophy. In: Journal of Neuropathology and Experimental Neurology. 1979 ; Vol. 38, No. 3. pp. 209-221.
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