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Vet Pathol 44:569-578 (2007)
© 2007 American College of Veterinary Pathologists
Animal Model Article

Pathology of Mucopolysaccharidosis IIIA in Huntaway Dogs

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

Institute of Veterinary, Animal and Biomedical Sciences, Massey University, Palmerston North, New Zealand (RDJ, ACJ, EJN), Dominic P. Purpura, Department of Neuroscience, Rose F. Kennedy Center, Albert Einstein College of Medicine, Bronx, NY (SUW), Lysosomal Disease Research Unit, Department of Genetic Medicine, Children, Youth and Women's Health Service, North Adelaide, SA, Australia (JFH)

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.

Key words: Mucopolysaccharidosis IIIA; heparan sulfate proteoglycans; dog diseases; macrophages; gangliosides; histopathology; nervous system diseases.

Request reprints from Request reprints from Robert D Jolly, Institute of Veterinary, Animal and Biomedical Sciences, Massey University, Private Bag 11 222, Palmerston North, 4442, New Zealand. E-mail: R.D.Jolly{at}massey.ac.nz






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