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American Journal of Pathology, Vol 112, 27-36, Copyright © 1983 by American Society for Investigative Pathology

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The pathology of the feline model of mucopolysaccharidosis I

ME Haskins, GD Aguirre, PF Jezyk, RJ Desnick and DF Patterson

Five cats with feline alpha-L-iduronidase-deficient mucopolysaccharidosis were studied. Membrane-bound cytoplasmic inclusions were present in central nervous system neurons, hepatocytes, chondrocytes, vascular and splenic smooth muscle cells, bone marrow leukocytes, and fibroblasts of the skin, eye, and cardiac valves. The lesions in these cats closely resemble those described in human patients with mucopolysaccharidosis I H (Hurler syndrome).




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Copyright © 1983 by the American Society for Investigative Pathology.