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American Journal of Pathology, Vol 114, 487-495, Copyright © 1984 by American Society for Investigative Pathology

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Morphologic and biochemical studies of canine mucopolysaccharidosis I

RM Shull, RG Helman, E Spellacy, G Constantopoulos, RJ Munger and EF Neufeld

This report presents the necropsy and biochemical findings on the first dog to die with alpha-L-iduronidase deficiency (mucopolysaccharidosis I, MPS I). Gross pathologic features, light- and electron-microscopic findings, and tissue enzyme, glycosaminoglycan (GAG), and sphingolipid levels are compared with the human disease counterpart and the previously described feline model. Results lend further support for the similarities of the canine disease and human MPS I.

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