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Impaired degradation of keratan sulphate by Morquio A fibroblasts.


ABSTRACT: Upon incubation of keratan [35S]sulphate with normal fibroblasts both [35S]sulphate and N-acetylglucosamine 6-[35S]sulphate are liberated. From the products obtained after digestion with various mutant fibroblasts and with purified N-acetylgalactosamine 6-sulphate sulphatase we suggest that (i) [35S]sulphate is released almost exclusively from galactose 6-sulphate residues; (ii) N-acetylgalactosamine 6-sulphate sulphatase exhibits galactose 6-sulphate sulphatase activity; (iii) both sulphatase activities are deficient in Morquio disease type A.

SUBMITTER: Glossl J 

PROVIDER: S-EPMC1158229 | biostudies-other | 1982 Apr

REPOSITORIES: biostudies-other

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