Gene/Proteome Database (LMPD)
LMPD ID
LMP001474
Gene ID
Species
Homo sapiens (Human)
Gene Name
AU RNA binding protein/enoyl-CoA hydratase
Gene Symbol
Synonyms
-
Alternate Names
methylglutaconyl-CoA hydratase, mitochondrial; 3-methylglutaconyl-CoA hydratase; AU-binding protein/Enoyl-CoA hydratase; AU-specific RNA-binding enoyl-CoA hydratase; AU RNA binding protein/enoyl-Coenzyme A hydratase; AU RNA-binding protein/enoyl-Coenzyme A hydratase
Chromosome
9
Map Location
9q22.31
EC Number
4.2.1.18
Summary
The methylglutaconyl-CoA hydratase, mitochondrial protein binds to the AU-rich element (ARE), a common element found in the 3' UTR of rapidly decaying mRNA such as c-fos, c-myc and granulocyte/ macrophage colony stimulating factor. ARE elements are involved in directing RNA to rapid degradation and deadenylation. AUH is also homologous to enol-CoA hydratase, an enzyme involved in fatty acid degradation, and has been shown to have intrinsic hydratase enzymatic activity. AUH is thus a bifunctional chimera between RNA binding and metabolic enzyme activity. A possible subcellular localization in the mitochondria has been demonstrated for the mouse homolog of this protein which shares 92% identity with the human protein. It has been suggested that AUH may have a novel role as a mitochondrial located AU-binding protein. Human AUH is expressed as a single mRNA species of 1.8 kb, and translated as a 40-kDa precursor protein which is subsequently processed to a 32-kDa mature form. [provided by RefSeq, May 2010]
Orthologs
Proteins
methylglutaconyl-CoA hydratase, mitochondrial precursor | |
---|---|
Refseq ID | NP_001689 |
Protein GI | 4502327 |
UniProt ID | Q13825 |
mRNA ID | NM_001698 |
Length | 339 |
RefSeq Status | REVIEWED |
MAAAVAAAPGALGSLHAGGARLVAACSAWLCPGLRLPGSLAGRRAGPAIWAQGWVPAAGGPAPKRGYSSEMKTEDELRVRHLEEENRGIVVLGINRAYGKNSLSKNLIKMLSKAVDALKSDKKVRTIIIRSEVPGIFCAGADLKERAKMSSSEVGPFVSKIRAVINDIANLPVPTIAAIDGLALGGGLELALACDIRVAASSAKMGLVETKLAIIPGGGGTQRLPRAIGMSLAKELIFSARVLDGKEAKAVGLISHVLEQNQEGDAAYRKALDLAREFLPQGPVAMRVAKLAINQGMEVDLVTGLAIEEACYAQTIPTKDRLEGLLAFKEKRPPRYKGE | |
transit_peptide: 1..67 calculated_mol_wt: 6431 peptide sequence: MAAAVAAAPGALGSLHAGGARLVAACSAWLCPGLRLPGSLAGRRAGPAIWAQGWVPAAGGPAPKRGY mat_peptide: 68..339 product: methylglutaconyl-CoA hydratase, mitochondrial calculated_mol_wt: 29196 peptide sequence: SSEMKTEDELRVRHLEEENRGIVVLGINRAYGKNSLSKNLIKMLSKAVDALKSDKKVRTIIIRSEVPGIFCAGADLKERAKMSSSEVGPFVSKIRAVINDIANLPVPTIAAIDGLALGGGLELALACDIRVAASSAKMGLVETKLAIIPGGGGTQRLPRAIGMSLAKELIFSARVLDGKEAKAVGLISHVLEQNQEGDAAYRKALDLAREFLPQGPVAMRVAKLAINQGMEVDLVTGLAIEEACYAQTIPTKDRLEGLLAFKEKRPPRYKGE |
Gene Information
Entrez Gene ID
Gene Name
AU RNA binding protein/enoyl-CoA hydratase
Gene Symbol
Species
Homo sapiens
Gene Ontology (GO Annotations)
GO ID | Source | Type | Description |
---|---|---|---|
GO:0005759 | TAS:Reactome | C | mitochondrial matrix |
GO:0005739 | ISS:UniProtKB | C | mitochondrion |
GO:0004300 | IDA:UniProtKB | F | enoyl-CoA hydratase activity |
GO:0004490 | EXP:Reactome | F | methylglutaconyl-CoA hydratase activity |
GO:0003730 | IDA:UniProtKB | F | mRNA 3'-UTR binding |
GO:0009083 | TAS:Reactome | P | branched-chain amino acid catabolic process |
GO:0034641 | TAS:Reactome | P | cellular nitrogen compound metabolic process |
GO:0006552 | IEA:UniProtKB-UniPathway | P | leucine catabolic process |
GO:0044281 | TAS:Reactome | P | small molecule metabolic process |
KEGG Pathway Links
KEGG Pathway ID | Description |
---|---|
hsa01100 | Metabolic pathways |
BIOCYC Pathway Links
BIOCYC Pathway ID | Description |
---|---|
LEU-DEG2-PWY | leucine degradation I |
REACTOME Pathway Links
REACTOME Pathway ID | Description |
---|---|
REACT_197 | Branched-chain amino acid catabolism |
Domain Information
UniProt Annotations
Entry Information
Gene Name
AU RNA binding protein/enoyl-CoA hydratase
Protein Entry
AUHM_HUMAN
UniProt ID
Species
Human
Comments
Comment Type | Description |
---|---|
Alternative Products | Event=Alternative splicing; Named isoforms=2; Name=1; IsoId=Q13825-1; Sequence=Displayed; Name=2; IsoId=Q13825-2; Sequence=VSP_008336; Note=No experimental confirmation available.; |
Catalytic Activity | (S)-3-hydroxy-3-methylglutaryl-CoA = trans-3- methylglutaconyl-CoA + H(2)O. |
Disease | 3-methylglutaconic aciduria 1 (MGA1) [MIM |
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. {ECO |
Pathway | Amino-acid degradation; L-leucine degradation; (S)-3- hydroxy-3-methylglutaryl-CoA from 3-isovaleryl-CoA |
Similarity | Belongs to the enoyl-CoA hydratase/isomerase family. |
Subcellular Location | Mitochondrion . |
Subunit | Homohexamer. |
Identical and Related Proteins
Unique RefSeq proteins for LMP001474 (as displayed in Record Overview)
Protein GI | Database | Accession | Length | Protein Name |
---|---|---|---|---|
4502327 | RefSeq | NP_001689 | 339 | methylglutaconyl-CoA hydratase, mitochondrial precursor |
Identical Sequences to LMP001474 proteins
Reference | Database | Accession | Length | Protein Name |
---|---|---|---|---|
GI:4502327 | EMBL | CAA56260.1 | 339 | AU-binding protein/Enoyl-CoA hydratase [Homo sapiens] |
GI:4502327 | GenBank | AAO99137.1 | 339 | Sequence 8 from patent US 6511833 |
GI:4502327 | GenBank | EAW62794.1 | 339 | AU RNA binding protein/enoyl-Coenzyme A hydratase, isoform CRA_a [Homo sapiens] |
GI:4502327 | GenBank | AFX58143.1 | 339 | Sequence 98 from patent US 8309323 |
GI:4502327 | GenBank | AHD72066.1 | 339 | Sequence 7564 from patent US 8586006 |
GI:4502327 | SwissProt | Q13825.1 | 339 | RecName: Full=Methylglutaconyl-CoA hydratase, mitochondrial; AltName: Full=AU-specific RNA-binding enoyl-CoA hydratase; Short=AU-binding protein/enoyl-CoA hydratase; Flags: Precursor [Homo sapiens] |
Related Sequences to LMP001474 proteins
Reference | Database | Accession | Length | Protein Name |
---|---|---|---|---|
GI:4502327 | GenBank | JAA03290.1 | 339 | AU RNA binding protein/enoyl-CoA hydratase [Pan troglodytes] |
GI:4502327 | GenBank | JAA11905.1 | 339 | AU RNA binding protein/enoyl-CoA hydratase [Pan troglodytes] |
GI:4502327 | GenBank | JAA23736.1 | 339 | AU RNA binding protein/enoyl-CoA hydratase [Pan troglodytes] |
GI:4502327 | GenBank | JAA43638.1 | 339 | AU RNA binding protein/enoyl-CoA hydratase [Pan troglodytes] |
GI:4502327 | RefSeq | XP_520124.1 | 339 | PREDICTED: methylglutaconyl-CoA hydratase, mitochondrial isoform X1 [Pan troglodytes] |
GI:4502327 | RefSeq | XP_009440990.1 | 339 | PREDICTED: methylglutaconyl-CoA hydratase, mitochondrial-like isoform X1 [Pan troglodytes] |