| Identification |
| HMDB Protein ID
| CDBP00920 |
| Secondary Accession Numbers
| Not Available |
| Name
| Methylglutaconyl-CoA hydratase, mitochondrial |
| Description
| Not Available |
| Synonyms
|
- AU-binding protein/enoyl-CoA hydratase
- AU-specific RNA-binding enoyl-CoA hydratase
|
| Gene Name
| AUH |
| Protein Type
| Enzyme |
| Biological Properties |
| General Function
| Involved in catalytic activity |
| Specific Function
| Catalyzes the conversion of 3-methylglutaconyl-CoA to 3-hydroxy-3-methylglutaryl-CoA. Has very low enoyl-CoA hydratase activity. Was originally identified as RNA-binding protein that binds in vitro to clustered 5'-AUUUA-3' motifs.
|
| GO Classification
|
| Biological Process |
| branched-chain amino acid catabolic process |
| leucine catabolic process |
| mRNA catabolic process |
| Cellular Component |
| mitochondrial matrix |
| Function |
| catalytic activity |
| Molecular Function |
| mRNA 3'-UTR binding |
| enoyl-CoA hydratase activity |
| methylglutaconyl-CoA hydratase activity |
| Process |
| metabolic process |
|
| Cellular Location
|
- Mitochondrion
|
| Pathways
|
| Name | SMPDB/Pathwhiz | KEGG | | Valine, Leucine and Isoleucine Degradation |    |  | | L-leucine degradation | Not Available | Not Available | | Beta-Ketothiolase Deficiency |    | Not Available | | 2-Methyl-3-Hydroxybutryl CoA Dehydrogenase Deficiency |    | Not Available | | Propionic Acidemia |    | Not Available |
|
| Gene Properties |
| Chromosome Location
| 9 |
| Locus
| 9q22.31 |
| SNPs
| AUH |
| Gene Sequence
|
>1020 bp
ATGGCGGCCGCGGTGGCGGCGGCACCTGGGGCCTTGGGATCCCTGCATGCTGGCGGCGCC
CGCCTGGTGGCCGCTTGCAGTGCGTGGCTCTGCCCGGGGTTGAGGCTGCCCGGCTCGTTG
GCAGGCCGGCGAGCGGGCCCGGCGATCTGGGCCCAGGGCTGGGTACCTGCGGCCGGGGGT
CCCGCCCCGAAAAGGGGCTACAGCTCTGAGATGAAGACGGAGGACGAGCTGCGGGTGCGG
CACCTGGAGGAGGAGAACCGAGGAATTGTGGTGCTTGGAATAAACAGAGCTTATGGCAAA
AATTCACTCAGTAAAAATCTTATAAAAATGCTATCAAAAGCTGTGGATGCTTTGAAATCT
GATAAGAAAGTACGGACCATAATAATCAGGAGTGAAGTCCCAGGGATATTCTGTGCTGGT
GCTGACCTTAAGGAAAGAGCCAAAATGAGTTCCAGTGAAGTTGGTCCTTTTGTCTCCAAA
ATAAGAGCAGTGATTAACGATATTGCTAATCTTCCAGTGCCAACAATTGCAGCAATAGAT
GGACTCGCTTTAGGTGGTGGTCTTGAACTGGCTTTAGCCTGTGATATACGAGTAGCAGCT
TCCTCTGCAAAAATGGGCCTGGTTGAAACAAAATTGGCGATTATTCCTGGTGGAGGGGGG
ACACAGCGATTGCCACGCGCCATTGGAATGTCCCTGGCCAAGGAGCTCATATTCTCTGCG
CGAGTCCTCGATGGCAAAGAAGCCAAAGCAGTGGGCTTAATCAGCCACGTTCTGGAACAG
AACCAGGAGGGAGACGCGGCCTACAGGAAGGCCTTGGACCTGGCGAGAGAGTTTTTACCT
CAGGGACCTGTTGCAATGAGAGTGGCAAAATTAGCAATTAATCAAGGGATGGAGGTCGAT
TTAGTAACAGGGTTAGCCATAGAAGAAGCTTGTTATGCTCAGACCATTCCAACAAAAGAC
AGACTTGAAGGTCTTCTTGCTTTTAAAGAGAAAAGGCCCCCTCGCTATAAAGGAGAATAA
|
| Protein Properties |
| Number of Residues
| 339 |
| Molecular Weight
| 35608.18 |
| Theoretical pI
| 9.476 |
| Pfam Domain Function
|
|
| Signals
|
Not Available
|
|
Transmembrane Regions
|
Not Available
|
| Protein Sequence
|
>Methylglutaconyl-CoA hydratase, mitochondrial
MAAAVAAAPGALGSLHAGGARLVAACSAWLCPGLRLPGSLAGRRAGPAIWAQGWVPAAGG
PAPKRGYSSEMKTEDELRVRHLEEENRGIVVLGINRAYGKNSLSKNLIKMLSKAVDALKS
DKKVRTIIIRSEVPGIFCAGADLKERAKMSSSEVGPFVSKIRAVINDIANLPVPTIAAID
GLALGGGLELALACDIRVAASSAKMGLVETKLAIIPGGGGTQRLPRAIGMSLAKELIFSA
RVLDGKEAKAVGLISHVLEQNQEGDAAYRKALDLAREFLPQGPVAMRVAKLAINQGMEVD
LVTGLAIEEACYAQTIPTKDRLEGLLAFKEKRPPRYKGE
|
| External Links |
| GenBank ID Protein
| 780241 |
| UniProtKB/Swiss-Prot ID
| Q13825 |
| UniProtKB/Swiss-Prot Entry Name
| AUHM_HUMAN |
| PDB IDs
|
|
| GenBank Gene ID
| X79888 |
| GeneCard ID
| AUH |
| GenAtlas ID
| AUH |
| HGNC ID
| HGNC:890 |
| References |
| General References
| Not Available |