AUH Catalyzes the conversion of 3-methylglutaconyl-CoA to 3-hydroxy-3-methylglutaryl-CoA. Also has itaconyl-CoA hydratase activity by converting itaconyl-CoA into citramalyl-CoA in the C5-dicarboxylate catabolism pathway. The C5-dicarboxylate catabolism pathway is required to detoxify itaconate, a vitamin B12-poisoning metabolite. Has very low enoyl-CoA hydratase activity. Was originally identified as RNA-binding protein that binds in vitro to clustered 5'-AUUUA-3' motifs. Belongs to the enoyl-CoA hydratase/isomerase family. 2 alternatively spliced human isoforms have been reported. Note: This description may include information from UniProtKB.
Protein type: Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC; Lyase; Mitochondrial; RNA-binding
Chromosomal Location of mouse Ortholog: 13|13 B1
Cellular Component:  mitochondrion
Molecular Function:  catalytic activity; enoyl-CoA hydratase activity; itaconyl-CoA hydratase activity; lyase activity; methylglutaconyl-CoA hydratase activity; mRNA 3'-UTR binding; RNA binding
Biological Process:  branched-chain amino acid catabolic process; fatty acid beta-oxidation; nuclear-transcribed mRNA catabolic process, deadenylation-dependent decay
Reference #:  Q9JLZ3 (UniProtKB)
Alt. Names/Synonyms: AU RNA binding protein/enoyl-coenzyme A hydratase; AU-binding enoyl-CoA hydratase; AU-specific RNA-binding enoyl-CoA hydratase; Auh; AUHM; C77140; Itaconyl-CoA hydratase; Methylglutaconyl-CoA hydratase, mitochondrial; muAUH; W91705
Gene Symbols: Auh
Molecular weight: 33,395 Da
Basal Isoelectric point: 9.56  Predict pI for various phosphorylation states
Select Structure to View Below


Protein Structure Not Found.

Cross-references to other databases:  AlphaFold  |  STRING  |  Reactome  |  BioGPS  |  Pfam  |  ENZYME  |  Phospho.ELM  |  NetworKIN  |  UniProtKB  |  Entrez-Gene  |  Ensembl Gene