IUBMB Enzyme Nomenclature


Accepted name: iduronate-2-sulfatase

Reaction: Hydrolysis of the 2-sulfate groups of the L-iduronate 2-sulfate units of dermatan sulfate, heparan sulfate and heparin

Other name(s): chondroitinsulfatase; idurono-2-sulfatase; iduronide-2-sulfate sulfatase; L-iduronosulfatase; L-idurono sulfate sulfatase; iduronate sulfatase; sulfo-L-iduronate sulfatase; L-iduronate 2-sulfate sulfatase; sulfoiduronate sulfohydrolase; 2-sulfo-L-iduronate 2-sulfatase; iduronate-2-sulfate sulfatase; iduronate sulfate sulfatase

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

Links to other databases: BRENDA, EXPASY, KEGG, Metacyc, PDB, CAS registry number: 50936-59-9


1. Archer, I.M., Harper, P.S. and Wusteman, F.S. Multiple forms of iduronate 2-sulphate sulphatase in human tissues and body fluids. Biochim. Biophys. Acta 708 (1982) 134-140. [PMID: 6816283]

2. Bach, J., Eisenberg, F., Cantz, M. and Neufeld, E.C. The defect in the Hunter syndrome: deficiency of sulfoiduronate sulfatase. Proc. Natl. Acad. Sci. USA 70 (1973) 2134-2138. [PMID: 4269173]

3. DiNatale, P. and Ronsivalle, L. Identification and partial characterization of two enzyme forms of iduronate sulfatase from human placenta. Biochim. Biophys. Acta 661 (1981) 106-111. [PMID: 6945876]

4. Yutaka, T., Fluharty, A.L., Stevens, R.L. and Kihara, H. Purification and some properties of human liver iduronate sulfatase. J. Biochem. (Tokyo) 91 (1982) 433-441. [PMID: 6950934]

[EC created 1984]

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