The IDUA gene provides instructions for producing an enzyme called alpha-L-iduronidase, which is essential for the breakdown of large sugar molecules called glycosaminoglycans (GAGs). Through a process called hydrolysis, alpha-L-iduronidase uses water molecules to break down a molecule known as unsulfated alpha-L-iduronic acid, which is present in two GAGs called heparan sulfate and dermatan sulfate. Alpha-L-iduronidase is located in lysosomes, compartments within cells that digest and recycle different types of molecules.
Other Names
alpha-L-iduronidase; IDUA_HUMAN
Related Genetics Topics
Identifiers
- NCBI Gene: 3425
- OMIM: 252800
Source: MedlinePlus Genetics, U.S. National Library of Medicine.
