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Type: Journal article
Title: Pathology of mucopolysaccharidosis IIIA in huntaway dogs
Author: Jolly, R.
Johnstone, A.
Norman, E.
Hopwood, J.
Walkley, S.
Citation: Veterinary Pathology, 2007; 44(5):569-578
Publisher: Amer Coll Vet Pathologist
Issue Date: 2007
ISSN: 0300-9858
Statement of
R. D. Jolly, A. C. Johnstone, E. J. Norman, J. J. Hopwood and S. U. Walkley
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.
Keywords: Liver; Kidney; Cerebellum; Cerebral Cortex; Animals; Dogs; Mucopolysaccharidosis III; Dog Diseases; Microscopy, Electron, Transmission
Description: Copyright © 2007 by the American College of Veterinary Pathologists.
RMID: 0020073224
DOI: 10.1354/vp.44-5-569
Published version:
Appears in Collections:Paediatrics publications

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