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Proteins/UNIPROT:Q8N0X4
Mitochondrial enzyme required to detoxify vitamin B12-poisoning metabolites. Acts as a citramalyl-CoA lyase by converting citramalyl-CoA into acetyl-CoA and pyruvate in the C5-dicarboxylate catabolism pathway, a pathway required to detoxify itaconate, a vitamin B12-poisoning metabolite. Also acts as a malyl-CoA thioesterase to detoxify malyl-CoA, a side product of citric acid cycle enzymes, which is toxic for the vitamin B12-dependent enzyme MMUT. Also acts as a malate synthase in vitro, converting glyoxylate and acetyl-CoA to malate. Also acts as a beta-methylmalate synthase in vitro, by mediating conversion of glyoxylate and propionyl-CoA to beta-methylmalate. Also has very weak citramalate synthase activity in vitro..
Function from UniProtKB (Q8N0X4), read 2026-09-23. CC BY 4.0.
Curated compounds routed: 0
62 statements
62 statements · 6 reference databases · Subject: CLYBL · Sources: gene ontology, Hetionet, Human Protein Atlas, Human-GEM, Rhea, UniProt
6 reactions
CHEBI:36882CHEBI:15351+pyruvate
Catalysed by CLYBL
Reaction pageLeft to rightBalancedEC 4.1.3.25mitochondrial matrix
Human-GEM MAR08666CC BY 4.0Reference data
CHEBI:15351+glyoxylic acid+waterCHEBI:1146900+proton+(S)-malate
Catalysed by CLYBL
Reaction pageLeft to rightBalancedEC 2.3.3.9
6 names
Resolves to: CLYBL
Human-GEM MAR20111CC BY 4.0Reference data
glyoxylate+acetyl-CoA+water(S)-malate+coenzyme A(4-)+hydron
Catalysed by CLYBL
Reaction pageDirection not assertedBalanced
Rhea RHEA:18181CC BY 4.0Reference data
(3S)-citramalyl-CoApyruvate+acetyl-CoA
Catalysed by CLYBL
Reaction pageDirection not assertedBalanced
Rhea RHEA:22612CC BY 4.0Reference data
(S)-malyl-CoA+water(S)-malate+coenzyme A(4-)+hydron
Catalysed by CLYBL
Reaction pageLeft to rightBalanced
Rhea RHEA:38291CC BY 4.0Reference data
propanoyl-CoA+glyoxylate+water3-methylmalate+coenzyme A(4-)+hydron
Catalysed by CLYBL
Reaction pageDirection not assertedBalanced
Rhea RHEA:47628CC BY 4.0Reference data