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American Journal of Pathology, Vol 101, 657-674, Copyright © 1980 by American Society for Investigative Pathology

REGULAR ARTICLES

The pathology of the feline model of mucopolysaccharidosis VI

ME Haskins, GD Aguirre, PF Jezyk and DF Patterson

Three cats with feline arylsulfatase-B--deficient mucopolysaccharidosis were studied by light and transmission electron microscopy. Membrane- bound cytoplasmic inclusions were present in hepatocytes, bone marrow granulocytes, vascular smooth muscle cells, and fibroblasts in skin, cornea, and cardiac valves. Central nervous system lesions were restricted to mild ventricular dilatation, perithelial cell vacuolation, and, in one animal, cord compression by vertebral exostoses. The lesions in these cats closely resembled those described in human patients with mucopolysaccharidosis VI (Maroteaux-Lamy syndrome).




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Copyright © 1980 by the American Society for Investigative Pathology.