AU RNA binding methylglutaconyl-CoA hydratase

AU RNA-binding protein/enoyl-Coenzyme A hydratase

AU RNA binding protein/enoyl-Coenzyme A hydratase

AU RNA binding protein/enoyl-CoA hydratase

Methylglutaconyl-CoA hydratase, mitochondrial

AU-specific RNA-binding enoyl-CoA hydratase

Itaconyl-CoA hydratase

3-MG-CoA hydratase

AU-binding protein/enoyl-CoA hydratase

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].

Varaico: Literature-Extracted Genetic Variants

AUH

RefSeq Summary

Pfam Domains

Gene Aliases

HGNC Name

HGNC Previous Names