AUH, AU RNA binding methylglutaconyl-CoA hydratase

AUH, AU RNA binding methylglutaconyl-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]

• Gene Synonyms (methylglutaconyl-CoA hydratase, mitochondrial, 3-methylglutaconyl-CoA hydratase, AU RNA binding protein/enoyl-CoA hydratase, AU RNA-binding protein/enoyl-Coenzyme A hydratase, AU-binding protein/Enoyl-CoA hydratase, AU-specific RNA-binding enoyl-CoA hydratase, itaconyl-CoA hydratase,)
• NCBI Gene ID: 549
• Species: Homo sapiens (Human)
• UNIPROT ID#>>B4DYI6
  UNIPROT ID#>>Q13825
• View the NCBI Database for this Gene »

Gene products are often involved in multiple pathways and networks within a living cell. Learn more about other interacting partners.

AU RNA binding methylglutaconyl-CoA hydratase interacts with:

• 3'(2'), 5'-bisphosphate nucleotidase 1
  AU RNA binding methylglutaconyl-CoA hydratase
  branched chain keto acid dehydrogenase E1 subunit alpha
  catenin beta 1
  cytochrome c oxidase subunit 6C
  cytochrome c oxidase subunit 7B
  DnaJ heat shock protein family (Hsp40) member C11
  enoyl-CoA hydratase domain containing 2
  family with sequence similarity 107 member A
  heat shock protein 90 beta family member 1
  lactamase beta
  leucine rich pentatricopeptide repeat containing
  mitochondrial ribosomal protein L21
  nipsnap homolog 1
  nipsnap homolog 2
  oxidoreductase like domain containing 1
  prolyl 4-hydroxylase subunit alpha 1
  pyruvate dehydrogenase E1 subunit beta
  RAB5 interacting factor

Paste a protein or nucleic acid sequence in the box below to confirm that it matches this gene’s reference sequence(s). Click on a link under RELATED ORF CLONES to see how a sequence matches to an experimentally-validated ORF clone.