Protein Information

General Information
MoonProt ID335
First appeared in release2.0
Name(s)Methylglutaconyl-CoA hydratase, mitochondrial, AUH, Gene: AUH
UniProt IDQ13825 (AUHM_HUMAN)
GO termsGO: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:0003723 RNA binding GO:0004490 methylglutaconyl-CoA hydratase activity GO:0016829 lyase activity GO:0005739 mitochondrion GO:0005759 mitochondrial matrix
Organisms for which functions have been demonstratedHomo sapiens (human)
Sequence length339 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 ID1HZD
Quaternary structure
Function 1
Function description3-methylglutaconyl-CoA hydratase that catalyzes the conversion of 3-methylglutaconyl-CoA to 3-hydroxy-3-methylglutaryl CoA (HMG-CoA) in leucine catabolism
References for functionMack,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. number4.2.1.18
Location of functional site(s)
Cellular location of function
Comments
Function 2
Function descriptionRNA binding
References for functionRichman 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 functionmitochondrion
Comments
Third Function
Function descriptionregulates mitochondrial protein synthesis, regulates mitochondrial RNA metabolism
References for function24598254
E.C. number
Location of functional site(s)
Cellular location of functionmitochondrion
CommentsRichman and coworkers reported:" the catalytic activity of AUH is required for its function in mitochondrial translation and biogenesis, since we do not observe changes in mitochondrial translation when we overexpress a catalytically inactive AUH protein in cells"