| Function | Catalyzes the fifth step in the leucine degradation pathway, the reversible hydration of 3-methylglutaconyl-CoA (3-MG-CoA) to 3-hydroxy-3-methylglutaryl-CoA (HMG-CoA). Can catalyze the reverse reaction but at a much lower rate in vitro. HMG-CoA is then quickly degraded by another enzyme (such as HMG-CoA lyase) to give acetyl-CoA and acetoacetate. Uses other substrates such as (2E)-glutaconyl-CoA efficiently in vitro, and to a lesser extent 3-methylcrotonyl-CoA (3-methyl-(2E)-butenoyl-CoA), crotonyl-CoA ((2E)-butenoyl-CoA) and 3-hydroxybutanoyl-CoA (the missing carboxylate reduces affinity to the active site). Originally it was identified as an RNA-binding protein as it binds to AU-rich elements (AREs) in vitro. AREs direct rapid RNA degradation and mRNA deadenylation. Might have itaconyl-CoA hydratase activity, converting itaconyl-CoA into citramalyl-CoA in the C5-dicarboxylate catabolism pathway. The C5-dicarboxylate catabolism pathway is required to detoxify itaconate, an antimicrobial metabolite and immunomodulator produced by macrophages during certain infections, that can act as a vitamin B12-poisoning metabolite. |
| Protein Name | Methylglutaconyl-Coa Hydratase - Mitochondrial3-Mg-Coa HydrataseAu-Specific Rna-Binding Enoyl-Coa HydrataseAu-Binding Protein/Enoyl-Coa HydrataseItaconyl-Coa Hydratase |
| Database Links | Reactome: R-HSA-70895 |
| Cellular Localisation | Mitochondrion |
| Alternative Antibody Names | Anti-Methylglutaconyl-Coa Hydratase - Mitochondrial antibodyAnti-3-Mg-Coa Hydratase antibodyAnti-Au-Specific Rna-Binding Enoyl-Coa Hydratase antibodyAnti-Au-Binding Protein/Enoyl-Coa Hydratase antibodyAnti-Itaconyl-Coa Hydratase antibodyAnti-AUH antibody |
Information sourced from Uniprot.org
