The IDS gene provides instructions for producing an enzyme called iduronate 2-sulfatase (I2S), which is essential for the breakdown of large sugar molecules called glycosaminoglycans (GAGs). Specifically, I2S removes a chemical group known as a sulfate from a molecule called sulfated alpha-L-iduronic acid, which is present in two GAGs called heparan sulfate and dermatan sulfate. I2S is located in lysosomes, which are compartments within cells that digest and recycle different types of molecules.
Other Names
IDS_HUMAN; iduronate 2-sulfatase (Hunter syndrome); iduronate-2-sulfatase
Related Genetics Topics
Identifiers
- NCBI Gene: 3423
- OMIM: 309900
Source: MedlinePlus Genetics, U.S. National Library of Medicine.
