GM2 gangliosidosis in a Japanese Spaniel

J. F. Cummings, P. A. Wood, Steven U. Walkley, A. de Lahunta, M. E. DeForest

Research output: Contribution to journalArticle

44 Citations (Scopus)

Abstract

A storage disease in a 2-year-old Japanese Spaniel resembled a GM2 gangliosidosis previously identified in a now extinct line of German Shorthaired Pointers. Despite a later appearance of signs in the Japanese Spaniel, the distribution, staining, and ultrastructure of the stored material were similar in the two breeds. Golgi studies of cerebral cortical neurons revealed the formation of spiny and aspiny enlargements at the axon hillock region (meganeurites) and the growth of secondary neurites from this region. As in the German Shorthaired Pointer model, there was massive storage of GM2 ganglioside as well as a seemingly paradoxical increase in total β-hexosaminidase activity measured in vitro.

Original languageEnglish (US)
Pages (from-to)247-253
Number of pages7
JournalActa Neuropathologica
Volume67
Issue number3-4
DOIs
StatePublished - Sep 1985

Fingerprint

GM2 Gangliosidosis
G(M2) Ganglioside
Hexosaminidases
Neurites
Staining and Labeling
Neurons
Growth
Axon Initial Segment
In Vitro Techniques

Keywords

  • Dog
  • GM Gangliosidosis
  • Meganeurites

ASJC Scopus subject areas

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

Cite this

Cummings, J. F., Wood, P. A., Walkley, S. U., de Lahunta, A., & DeForest, M. E. (1985). GM2 gangliosidosis in a Japanese Spaniel. Acta Neuropathologica, 67(3-4), 247-253. https://doi.org/10.1007/BF00687809

GM2 gangliosidosis in a Japanese Spaniel. / Cummings, J. F.; Wood, P. A.; Walkley, Steven U.; de Lahunta, A.; DeForest, M. E.

In: Acta Neuropathologica, Vol. 67, No. 3-4, 09.1985, p. 247-253.

Research output: Contribution to journalArticle

Cummings, JF, Wood, PA, Walkley, SU, de Lahunta, A & DeForest, ME 1985, 'GM2 gangliosidosis in a Japanese Spaniel', Acta Neuropathologica, vol. 67, no. 3-4, pp. 247-253. https://doi.org/10.1007/BF00687809
Cummings JF, Wood PA, Walkley SU, de Lahunta A, DeForest ME. GM2 gangliosidosis in a Japanese Spaniel. Acta Neuropathologica. 1985 Sep;67(3-4):247-253. https://doi.org/10.1007/BF00687809
Cummings, J. F. ; Wood, P. A. ; Walkley, Steven U. ; de Lahunta, A. ; DeForest, M. E. / GM2 gangliosidosis in a Japanese Spaniel. In: Acta Neuropathologica. 1985 ; Vol. 67, No. 3-4. pp. 247-253.
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