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Vet Pathol 38:281-290 (2001)
© 2001 American College of Veterinary Pathologists

GM1-gangliosidosis in Alaskan Huskies: Clinical and Pathologic Findings

G. Müller, S. Alldinger, A. Moritz, A. Zurbriggen, N. Kirchhof, A. Sewell and W. Baumgärtner

Institut für Veterinär-Pathologie (GM, SA, NK, WB) and Medizinische und Gerichtliche Veterinärklinik I (AM), Justus-Liebig-Universität Giessen, Giessen, Germany; Institute for Animal Neurology, Bern, Switzerland (AZ); and Zentrum für Kinderheilkunde, Kliniken der Johann-Wolfgang-Goethe-Universität, Frankfurt, Germany (AS)

Three Alaskan Huskies, two females and one male, were diagnosed with GM1-gangliosidosis. Clinically, diseased animals exhibited proportional dwarfism and developed progressive neurologic impairment with signs of cerebellar dysfunction at the age of 5–7 months. Skeletal lesions characterized by retarded enchondral ossification of vertebral epiphyses were revealed by radiographs of the male dog at 5.5 months of age. Histologic examination of the central nervous system (CNS) revealed that most neurons were enlarged with a foamy to granular cytoplasm due to tightly packed vacuoles that displaced the Nissl substance. Vacuoles in paraffin-embedded sections stained positively with Luxol fast blue and Grocott's method, and in frozen sections vacuoles were periodic acid–Schiff positive. Foamy vacuolation also occurred within neurons of the autonomic ganglia. Extracerebral cells such as macrophages and peripheral lymphocytes also displayed foamy cytoplasm and vacuolation. In the CNS of diseased animals, a mild demyelination and axonal degeneration was accompanied by a significant astrogliosis (P < 0.05) in the gray matter as compared with age- and sex-matched control dogs. There was also a significant loss (P < 0.05) of oligodendrocytes in the gray and white matter of affected animals as compared with controls. Ultrastructurally, the neuronal storage material consisted of numerous circular to concentric whorls of lamellated membranes or stacks of membranes in parallel arrays. GM1-gangliosidosis in Alaskan Huskies resembles ß-galactosidase deficiency in other canine breeds, and these CNS disorders may be a consequence of neuronal storage and disturbed myelin processing.

Key words: Alaskan Huskies; CNS; dogs; GM1-gangliosidosis; immunohistochemistry; in situ hybridization; lysosomal storage disease.

Request reprints from Prof. Dr. W. Baumgärtner, Institut für Veterinär-Pathologie, Justus-Liebig-Universität Giessen, Frankfurter Strasse 96, D-35392 Giessen (Germany). Email: wolfgang.baumgaertner{at}vetmed.uni-giessen.de.




This article has been cited by other articles:


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 GeneticsHome page
R. Kreutzer, T. Leeb, G. Muller, A. Moritz, and W. Baumgartner
A Duplication in the Canine {beta}-Galactosidase Gene GLB1 Causes Exon Skipping and GM1-Gangliosidosis in Alaskan Huskies
Genetics, August 1, 2005; 170(4): 1857 - 1861.
[Abstract] [Full Text] [PDF]


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