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Overview

Uniprot IDF1LU71
Protein NameMethylglutaconyl-CoA hydratase, mitochondrial
Gene NameAuh
OrganismRattus norvegicus

Kla Sites from experimental identification

Position Flanking peptide
120 FCAGADLKERAKMHS
124 ADLKERAKMHSSEVG
180 RVAASSAKMGLVETK
76 GINRAYGKNSLSKNL
89 NLLKMLSKAVDALKS

Function

Catalyzes the fifth step in the leucine degradation pathway, the reversible hydration of 3-methylglutaconyl-CoA (3-MG-CoA) to 3-hydroxy-3-methylglutaryl-CoA (HMG-CoA). Can catalyze the reverse reaction but at a much lower rate in vitro. HMG-CoA is then quickly degraded by another enzyme (such as HMG-CoA lyase) to give acetyl-CoA and acetoacetate. Uses other substrates such as (2E)-glutaconyl-CoA efficiently in vitro, and to a lesser extent 3-methylcrotonyl-CoA (3-methyl-(2E)-butenoyl-CoA), crotonyl-CoA ((2E)-butenoyl-CoA) and 3-hydroxybutanoyl-CoA (the missing carboxylate reduces affinity to the active site). Originally it was identified as an RNA-binding protein as it binds to AU-rich elements (AREs) in vitro. AREs direct rapid RNA degradation and mRNA deadenylation (By similarity). Might have itaconyl-CoA hydratase activity, converting itaconyl-CoA into citramalyl-CoA in the C5-dicarboxylate catabolism pathway. The C5-dicarboxylate catabolism pathway is required to detoxify itaconate, an antimicrobial metabolite and immunomodulator produced by macrophages during certain infections, that can act as a vitamin B12-poisoning metabolite (PubMed:13783048)

Protein Sequence

10 MAAAAAPGAL 20 GALSAGRVRL 30 VAACCARLGS 40 AAWARGTAPR 50 RGYSSEVKTE 60 DELRVRHLEE 70 ENRGIVVLGI 80 NRAYGKNSLS 90 KNLLKMLSKA 100 VDALKSDKKV 110 RTIIIRSEVP 120 GIFCAGADLK 130 ERAKMHSSEV 140 GPFVSKIRAV 150 INDIANLPVP 160 TIAAIDGLAL 170 GGGLELALAC 180 DIRVAASSAK 190 MGLVETKLAI 200 IPGGGGTQRL 210 PRAIGMALAK 220 ELIFSARVLD 230 GQEAKAVGLI 240 SHVLEQNQEG 250 DAAYRKALDL 260 AREFLPQGPV 270 AMRVAKLAIN 280 QGMEVDLVTG 290 LAIEEACYAQ 300 TISTKDRLEG 310 LLAFKEKRPP RYKGE

Gene Ontology

Classification GO ID Description
Cellular Component GO:0005739 mitochondrion
Molecular Function GO:0004300 enoyl-CoA hydratase activity
Molecular Function GO:0050011 itaconyl-CoA hydratase activity
Molecular Function GO:0004490 methylglutaconyl-CoA hydratase activity
Molecular Function GO:0003730 mRNA 3'-UTR binding
Molecular Function GO:0003723 RNA binding
Biological Process GO:0006635 fatty acid beta-oxidation
Biological Process GO:0006552 L-leucine catabolic process

Reference

[1] Yao Y, Bade R, Li G, Zhang A, Zhao H et al.. Global-Scale Profiling of Differential Expressed Lysine-Lactylated Proteins in the Cerebral Endothelium of Cerebral Ischemia-Reperfusion Injury Rats.. Cell Mol Neurobiol 43(5):1989-2004. 2023 Jul. PMID: 36030297.

[2] Chen Y, Sun W, Sun Z, Zhao H, Wu T et al.. Effect of electroacupuncture on hippocampal protein lactylation in a rat model of vascular dementia.. Front Neurol 16:1629474. 2025. PMID: 40963935.