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Iduronate 2-sulfatase (IDS) is required for the lysosomal degradation of heparan sulfate and dermatan sulfate. Mutations causing IDS deficiency in humans result in the lysosomal storage of these glycosaminoglycans and Hunter syndrome, an X chromosome-linked disease. IDS is synthesized as two precursor forms of 76 and 90 kDa that are converted, through a 62 kDa intermediate, to 55 and 45 kDa mature polypeptides due to an internal proteolytic cleavage (PMID: 10838181).