Multiple sulfohydrolase deficiency

Multiple sulfohydrolase deficiency Cerebroside 3-sulfate, steroid sulfate, and heparan sulfate Arylsulphohydrolases A, B, C, iduronate sulfate sulfohydrolase, )V-acetyl galactosamine 6-sulfohydrolase Brain, bone... 

It is well known that the activities of various lysosomal hydrolases are strongly inhibited by heparan sulfate and other glycosaminoglycans (Avila and Convit, 1976). A possibility therefore exists that the primary defect in multiple sulfohydrolase deficiency is a mutation of hydrolase acting on heparan sulfate. An accumulation of this glycosaminoglycan may then inhibit other sulfohydrolases (Farooqui and Horrocks, 1984b). This possibility has not been tested. 

Fiddler, M. B., Vine, D., Shapira, E., and Nadler, H. L., Is multiple sulfatase deficiency due to defective regulation or sulfohydrolase expression. Nature (London) 282, 98 (1979). 

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