Description
Product Characteristics: Synonyms: IDS, iduronate 2 sulfatase, Idursulfase
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).
Target Information: 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. [provided by RefSeq, Oct 2009]