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Proteins/UNIPROT:Q13825
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. 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..
Function from UniProtKB (Q13825), read 2026-09-23. CC BY 4.0.
Curated compounds routed: 0
1 row
68 statements
68 statements · 7 reference databases · Subject: AUH · Sources: Bgee, Hetionet, Human Protein Atlas, Human-GEM, Reactome, Rhea, WikiPathways
10 reactions
3-methyl-(2E)-glutaconyl-CoA+waterCHEBI:15467
Catalysed by AUH
Reaction pageReversibleBalancedEC 4.2.1.18mitochondrial matrix
Human-GEM MAR03775CC BY 4.0Reference data
3-methylbut-2-enoyl-CoA+water3-hydroxyisovaleryl-CoA
Catalysed by AUH
Reaction pageLeft to rightBalancedEC 4.2.1.-mitochondrial matrix
Human-GEM MAR05999CC BY 4.0Reference data
4 names
Resolves to: AUH
Catalysed by AUH
Reaction pageLeft to rightBalancedEC 4.2.1.56mitochondrial matrix
Human-GEM MAR08664CC BY 4.0Reference data
water+MAM03031mMAM00773m
Catalysed by AUH
Reaction pageLeft to rightBalancedEC 4.2.1.18mitochondrial matrix
Human-GEM MAR09565CC BY 4.0Reference data
MAM01579m+waterMAM00773m
Catalysed by AUH
Reaction pageLeft to rightBalancedEC 4.2.1.18mitochondrial matrix
Human-GEM MAR09567CC BY 4.0Reference data
water+MAM03964mMAM03222m
Catalysed by AUH
Reaction pageLeft to rightBalancedEC 4.2.1.-mitochondrial matrix
Human-GEM MAR11514CC BY 4.0Reference data
(3S)-citramalyl-CoAitaconyl-CoA+water
Catalysed by AUH
Reaction pageRight to leftBalanced
Rhea RHEA:13785CC BY 4.0Reference data
(3S)-3-hydroxy-3-methylglutaryl-CoA3-methyl-(2E)-glutaconyl-CoA+water
Catalysed by AUH
Reaction pageRight to leftBalanced
Rhea RHEA:21536CC BY 4.0Reference data
3-hydroxyisovaleryl-CoA3-methylbut-2-enoyl-CoA+water
Reaction pageRight to leftBalanced
Rhea RHEA:31079CC BY 4.0Reference data
CHEBI:177916(2E)-glutaconyl-CoA+water
Catalysed by AUH
Reaction pageRight to leftBalanced
Rhea RHEA:68456CC BY 4.0Reference data