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Iduronate sulfatase

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Iduronate sulfatase

(Science: enzyme) An enzyme that specifically cleaves the ester sulfate of iduronic acid, and is required for the desulfation of 2-sulfate iduronate residues in heparan sulfate. It is also required in dermatan sulfate degradation.

Its deficiency has been demonstrated in hunters syndrome, which is characterised by an excess of dermatan sulfate and heparan sulfate.

chemical name: L-iduronate-2-sulfate 2-sulfohydrolase

registry number: EC 3.1.6.13