Gene/Proteome Database (LMPD)

LMPD ID
LMP001474
Gene ID
549
Species
Homo sapiens (Human)
Gene Name
AU RNA binding protein/enoyl-CoA hydratase
Gene Symbol
AUH
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
View orthologs and multiple alignments for AUH

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
549
Gene Name
AU RNA binding protein/enoyl-CoA hydratase
Gene Symbol
AUH
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:0003730 IDA:UniProtKB F mRNA 3'-UTR binding
GO:0004490 EXP:Reactome F methylglutaconyl-CoA hydratase activity
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

InterPro Annotations

Accession Description
IPR029045 ClpP/crotonase-like domain
IPR014748 Crontonase, C-terminal
IPR001753 Crotonase_core_superfam
IPR018376 Enoyl-CoA hydratase/isomerase, conserved site

UniProt Annotations

Entry Information

Gene Name
AU RNA binding protein/enoyl-CoA hydratase
Protein Entry
AUHM_HUMAN
UniProt ID
Q13825
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]