Iduronate 2-sulfatase (IDS) hydrolyses 2-sulfate groups from L-iduronate 2-sulfate units of dermatan sulfate in the lysosome. Defects in IDS are the cause of mucopolysaccharidosis type II (MPSII, MIM:309900), also called Hunter syndrome (Wilson et al. 1990).
Morris, CP, Bielicki, J, Occhiodoro, T, Hopwood, JJ, Anson, DS, Clements, PR, Wilson, PJ
iduronate-2-sulfatase activity of IDS dimer [lysosomal lumen]
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