| General Information |
| MoonProt ID | 335 |
| First appeared in release | 2.0 |
| Name(s) | Methylglutaconyl-CoA hydratase, mitochondrial, AUH, Gene: AUH |
| UniProt ID | Q13825 (AUHM_HUMAN) |
| GO terms | GO:0006552 leucine catabolic process
GO:0006635 fatty acid beta-oxidation
GO:0008152 metabolic process
GO:0009083 branched-chain amino acid catabolic process
GO:0003723 RNA binding
GO:0003730 mRNA 3'-UTR binding
GO:0003824 catalytic activity
GO:0004300 enoyl-CoA hydratase activity
GO:0004490 methylglutaconyl-CoA hydratase activity
GO:0016829 lyase activity
GO:0005739 mitochondrion
GO:0005759 mitochondrial matrix |
| Organisms for which functions have been demonstrated | Homo sapiens (human, a mammal) |
| Sequence length | 339 amino acids |
| FASTA sequence | >sp|Q13825|AUHM_HUMAN Methylglutaconyl-CoA hydratase, mitochondrial OS=Homo sapiens GN=AUH PE=1 SV=1
MAAAVAAAPGALGSLHAGGARLVAACSAWLCPGLRLPGSLAGRRAGPAIWAQGWVPAAGGPAPKRGYSSEMKTEDELRVRHLEEENRGIVVLGINRAYGKNSLSKNLIKMLSKAVDALKSDKKVRTIIIRSEVPGIFCAGADLKERAKMSSSEVGPFVSKIRAVINDIANLPVPTIAAIDGLALGGGLELALACDIRVAASSAKMGLVETKLAIIPGGGGTQRLPRAIGMSLAKELIFSARVLDGKEAKAVGLISHVLEQNQEGDAAYRKALDLAREFLPQGPVAMRVAKLAINQGMEVDLVTGLAIEEACYAQTIPTKDRLEGLLAFKEKRPPRYKGE |
| Structure Information |
| PDB ID | 1HZD |
| Quaternary structure | |
| SCOP | ClpP/crotonase |
| CATH | 3.90.226.10, 1.10.12.10 |
| TM Helix Prediction | no TM helices |
| DisProt Annotation | Not in DisProt |
| Predicted Disorder Regions | 1-12, 59-72, 333-339 |
| Connections to Disease |
| OMIM ID | |
| Function 1 |
| Function description | 3-methylglutaconyl-CoA hydratase that catalyzes the conversion of 3-methylglutaconyl-CoA to 3-hydroxy-3-methylglutaryl CoA (HMG-CoA) in leucine catabolism |
| References for function | Mack,M., Schniegler-Mattox,U., Peters,V., Hoffmann,G.F., Liesert,M., Buckel,W. and Zschocke,J. (2006) Biochemical characterization of human 3-methylglutaconyl-CoA hydratase and its role in leucine metabolism. FEBS J., 273, 2012–2022. |
| E.C. number | 4.2.1.18 |
| Location of functional site(s) | |
| Cellular location of function | |
| Comments | |
| Function 2 |
| Function description | RNA binding |
| References for function | Richman TR, Davies SM, Shearwood AM, Ermer JA, Scott LH, Hibbs ME, Rackham O, Filipovska A. A bifunctional protein regulates mitochondrial protein synthesis. Nucleic Acids Res. 2014 May;42(9):5483-94. doi: 10.1093/nar/gku179. PMID: 24598254 |
| E.C. number | |
| Location of functional site(s) | |
| Cellular location of function | mitochondrion |
| Comments | |