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MORQUIO'S SYNDROME: A DEFICIENCY OF CHONDROITIN SULFATE N-ACETYLHEXOSAMINE SULFATE SULFATASE

Morquio's syndrome, an autosomal recessive disease exhibiting severe skeletal deformities and platyspondyly, is characterized by excretion of keratan sulfate and chondroitin sulfate. Extracts of cultured skin fibroblasts were found to contain normal levels of arylsulfatases A, B and C, β-N-acetylhexosaminidase, (β-glucuronidase and β-galactosidase. Incubation of extracts of Morquio fibroblasts with [35S]chondroitin sulfate (prepared from embryonic chick epiphyses) resulted in release of 2% of the 35SO4 in contrast to the release of 10-15% by extracts of normal, Hurler, Hunter or Sanfilippo A fibroblasts. Similar results were obtained when a heptasaccharide, GalNAc 35SO4-(GlcUA-GalNAc 35SO4)3, prepared from chondroitin sulfate, was incubated with lysosomal preparations from cultured fibroblasts. These findings indicate that Morquio's syndrome is due to a deficiency of a specific N-acetylhexosamine sulfate sulfatase. On the basis of the structures of the compounds excreted in Morquio's disease, it is likely that the deficient enzyme hydrolyzes 6-0-sulfate linkages in glycosaminoglycans. Supported by USPHS Grant Nos. AM-05996, HD-04583, AM-05589-06 and 2-Mol RR-00305-08.