The AUH gene provides instructions for producing an enzyme called 3-methylglutaconyl-CoA hydratase. This enzyme is found in cell structures called mitochondria, which convert energy from food into a form that cells can use. Within mitochondria, this enzyme plays an important role in breaking down proteins into smaller molecules that cells can use to produce energy. Specifically, 3-methylglutaconyl-CoA hydratase is responsible for the fifth step in breaking down the protein building block (amino acid) leucine. The enzyme converts a molecule called 3-methylglutaconyl-CoA into another molecule called 3-hydroxy-3-methylglutaryl-CoA.
3-methylglutaconyl-CoA hydratase also has the ability to attach (bind) to RNA, a chemical cousin of DNA. Researchers are working to determine the purpose of this RNA-binding ability.
Other Names
3-methylglutaconyl Coenzyme A hydratase; 3-methylglutaconyl-CoA hydratase; AU RNA binding protein/enoyl-CoA hydratase; AU RNA binding protein/enoyl-Coenzyme A hydratase; AU RNA-binding protein/enoyl-Coenzyme A hydratase; AU-specific RNA-binding protein; AUMH_HUMAN; enoyl-Coenzyme A hydratase; methylglutaconyl-CoA hydratase
Related Genetics Topics
Identifiers
- NCBI Gene: 549
- OMIM: 600529
Source: MedlinePlus Genetics, U.S. National Library of Medicine.
