Aliases for AUH Gene
- AU RNA Binding Methylglutaconyl-CoA Hydratase 2 3
- AU RNA Binding Protein/Enoyl-CoA Hydratase 2 5
- AU RNA-Binding Protein/Enoyl-Coenzyme A Hydratase 2
- AU RNA Binding Protein/Enoyl-Coenzyme A Hydratase 2
- AU-Specific RNA-Binding Enoyl-CoA Hydratase 4
- AU-Binding Protein/Enoyl-CoA Hydratase 4
- EC 126.96.36.199 4
External Ids for AUH Gene
Previous GeneCards Identifiers for AUH Gene
This gene encodes bifunctional mitochondrial protein that has both RNA-binding and hydratase activities. The encoded protein is a methylglutaconyl-CoA hydratase that catalyzes the hydration of 3-methylglutaconyl-CoA to 3-hydroxy-3-methyl-glutaryl-CoA, a critical step in the leucine degradation pathway. This protein also binds AU-rich elements (AREs) found in the 3' UTRs of rapidly decaying mRNAs including c-fos, c-myc and granulocyte/ macrophage colony stimulating factor. ARE elements are involved in directing RNA to rapid degradation and deadenylation. This protein is localizes to the mitochondrial matrix and the inner mitochondrial membrane and may be involved in mitochondrial protein synthesis. Mutations in this gene are the cause of 3-methylglutaconic aciduria, type I. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Sep 2015]
GeneCards Summary for AUH Gene
AUH (AU RNA Binding Methylglutaconyl-CoA Hydratase) is a Protein Coding gene. Diseases associated with AUH include 3-Methylglutaconic Aciduria, Type I and 3-Methylglutaconic Aciduria. Among its related pathways are Metabolism and Valine, leucine and isoleucine degradation. GO annotations related to this gene include RNA binding and enoyl-CoA hydratase activity. An important paralog of this gene is ECHDC2.
UniProtKB/Swiss-Prot for AUH Gene
Catalyzes the conversion of 3-methylglutaconyl-CoA to 3-hydroxy-3-methylglutaryl-CoA. Has very low enoyl-CoA hydratase activity. Was originally identified as RNA-binding protein that binds in vitro to clustered 5-AUUUA-3 motifs.