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Overview

Uniprot IDQ9JLZ3
Protein NameMethylglutaconyl-CoA hydratase, mitochondrial
Gene NameAuh
OrganismMus musculus

Kla Sites from experimental identification

Position Flanking peptide
119 FCAGADLKERAKMHS
123 ADLKERAKMHSSEVG
179 RVAASSAKMGLVETK
75 GINRAYGKNALSKNL

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) (By similarity). 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 (PubMed:10072761). 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 (By similarity)

Protein Sequence

10 MAAAAPGALG 20 ALRTGRVRLV 30 AACCARLGPA 40 AWARGTAPRR 50 GYSSEVKTED 60 ELRVRHLEEE 70 NRGIVVLGIN 80 RAYGKNALSK 90 NLLKMLSKAV 100 DALKSDKKVR 110 TIIIRSEVPG 120 IFCAGADLKE 130 RAKMHSSEVG 140 PFVSKIRSVI 150 NDIANLPVPT 160 IAAIDGLALG 170 GGLELALACD 180 IRVAASSAKM 190 GLVETKLAII 200 PGGGGTQRLP 210 RAIGMSLAKE 220 LIFSARVLDG 230 QEAKAVGLIS 240 HVLEQNQEGD 250 AAYRKALDLA 260 REFLPQGPVA 270 MRVAKLAINQ 280 GMEVDLVTGL 290 AIEEACYAQT 300 ISTKDRLEGL 310 LAFKEKRPPR YKGE

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] Zhuo W, Zhang M, Tan J, Gao Y, Wang Y et al.. Lysine lactylation analysis of proteins in the heart of the Kawasaki disease mouse model.. Front Cell Dev Biol 13:1550220. 2025. PMID: 40114965.

[2] Wu D, Tang Y, Li X, Xiong S, Zhang Z et al.. Characterization of protein lactylation in healthy and ischemic mouse hearts.. Front Cardiovasc Med 12:1644886. 2025. PMID: 41089239.