Animal models for lysosomal storage diseases: A new case of feline mucopolysaccharidosis VI

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Abstract
Summary: Two long‐haired Siamese cats are reported with clinical manifestations of human mucopolysaccharidosis VI (Maroteaux‐Lamy disease): facial dysmorphia, dysostosis multiplex, paralysis. Urine of the two affected animals contained a high concentration of glycosaminoglycans, as detected by the dimethylmethylene blue test. Qualitative analysis, performed by thin‐layer chromatography of the cetylpyridinium chloride‐precipitable material, showed dermatan sulphate. Excessive incorporation of [35S]sulphate in the intracellular mucopolysaccharide of cultured fibroblasts and deficiency of arylsulphatase B in such cells indicate that these cats are affected by Maroteaux‐Lamy disease. They should thus be considered the first European case of feline mucopolysaccharidosis VI.