Pathology of mucopolysaccharidosis IIIA in huntaway dogs

Robert D. Jolly, A. C. Johnstone, E. J. Norman, J. J. Hopwood, S. U. Walkley

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17 Citations (Scopus)

Abstract

Dogs with mucopolysaccharidosis (MPS) IIIA were bred within an experimental colony. As part of characterizing them as a model for testing therapeutic strategies for the analogous disease of children, a pathologic study was undertaken. By histology, there were variably stained storage cytosomes within neurons, including many that stained for gangliosides. On ultrastructure examination, these cytosomes contained either moderately dense granular material, tentatively interpreted as precipitated glycosaminoglycan; a variety of multilaminar bodies, interpreted as being associated with secondary accumulation of gangliosides; or a mixture of both types. In the liver, storage vesicles also contained excess glycogen as a secondary storage product. In various tissues, there were large foamy macrophages. In the brain, many of these were in juxtaposition with neurons, and, on ultrastructure examination, they contained storage cytosomes similar to those in neurons. However, the neuron in association with such a macrophage frequently showed little such material.

LanguageEnglish
Pages569-578
Number of pages10
JournalVeterinary Pathology
Volume44
Issue number5
DOIs
Publication statusPublished - 1 Sep 2007

Keywords

  • Dog diseases
  • Gangliosides
  • Heparan sulfate proteoglycans
  • Histopathology
  • Macrophages
  • Mucopolysaccharidosis IIIA
  • Nervous system diseases

ASJC Scopus subject areas

  • veterinary(all)

Cite this

Jolly, R. D., Johnstone, A. C., Norman, E. J., Hopwood, J. J., & Walkley, S. U. (2007). Pathology of mucopolysaccharidosis IIIA in huntaway dogs. Veterinary Pathology, 44(5), 569-578. https://doi.org/10.1354/vp.44-5-569