IDS : iduronate 2-sulfatase

Description

The IDS (iduronate 2-sulfatase) is a protein-coding gene located on chromosome X.

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.

Lysosomal enzyme involved in the degradation pathway of dermatan sulfate and heparan sulfate. {ECO:0000269|PubMed:10838181, ECO:0000269|PubMed:11731225, ECO:0000269|PubMed:28593992}

IDS is also known as ID2S, MPS2, SIDS.

Associated Diseases

• Mucopolysaccharidosis type 2, attenuated form
• Mucopolysaccharidosis, type II
• Mucopolysaccharidosis type 2, severe form
• Mucopolysaccharidosis type II