Iduronate-2-sulfatase is required for the lysosomal degradation of heparan sulfate and dermatan sulfate.

Mutations in thi sX-chromosome gene that result in enzymatic deficiency lead to the sex-linked Mucopolysaccharidosis Type II, also known as Hunter Syndrome.

Iduronate-2-sulfatase has a strong sequence homology with human arylsufatases A, B, C and human glucosamine-6-sulfatase. A splice variant of this gene has been described.