Iduronate-2-sulfatase is required for the lysosomal degradation of heparan sulfate and dermatan sulfate. Mutations in this X-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 similarity with human arylsulfatases A, B, and C, and human glucosamine-6-sulfatase. Multiple alternatively spliced transcript variants that encode different protein isoforms have been described.
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Protein Aliases: Alpha-L-iduronate sulfate sulfatase; IDS; Iduronate 2-sulfatase; iduronate 2-sulfatase (Hunter syndrome); Iduronate 2-sulfatase 14 kDa chain; Iduronate 2-sulfatase 42 kDa chain; iduronate sulfatase; iduronate-2-sulfatase; Idursulfase; MPS2; SIDS
Gene Aliases: AW214631; IDS; MPS2; SIDS
Molecular Function: hydrolase