UniProt ID: Q6AZA0 |
FUNCTION: This is one of the enzymes that catalyzes the last step of the mitochondrial beta-oxidation pathway, an aerobic process breaking down fatty acids into acetyl-CoA. Using free coenzyme A/CoA, catalyzes the thiolytic cleavage of medium- to long-chain 3-oxoacyl-CoAs into acetyl-CoA and a fatty acyl-CoA shortened by two carbon atoms. The activity of the enzyme is reversible and it can also catalyze the condensation of two acetyl-CoA molecules into acetoacetyl-CoA. Thereby, it plays a major role in ketone body metabolism. {ECO:0000250|UniProtKB:P24752}. CATALYTIC ACTIVITY: Reaction=2 acetyl-CoA = acetoacetyl-CoA + CoA; Xref=Rhea:RHEA:21036, ChEBI:CHEBI:57286, ChEBI:CHEBI:57287, ChEBI:CHEBI:57288; EC=2.3.1.9; Evidence={ECO:0000255|PROSITE-ProRule:PRU10020}; PhysiologicalDirection=left-to-right; Xref=Rhea:RHEA:21037; Evidence={ECO:0000250|UniProtKB:P24752}; PhysiologicalDirection=right-to-left; Xref=Rhea:RHEA:21038; Evidence={ECO:0000250|UniProtKB:P24752}; CATALYTIC ACTIVITY: Reaction=acetyl-CoA + propanoyl-CoA = 2-methyl-3-oxobutanoyl-CoA + CoA; Xref=Rhea:RHEA:30719, ChEBI:CHEBI:57287, ChEBI:CHEBI:57288, ChEBI:CHEBI:57335, ChEBI:CHEBI:57392; Evidence={ECO:0000250|UniProtKB:P24752}; PhysiologicalDirection=left-to-right; Xref=Rhea:RHEA:30720; Evidence={ECO:0000250|UniProtKB:P24752}; PhysiologicalDirection=right-to-left; Xref=Rhea:RHEA:30721; Evidence={ECO:0000250|UniProtKB:P24752}; PATHWAY: Lipid metabolism; fatty acid beta-oxidation. {ECO:0000250|UniProtKB:P24752}. SUBUNIT: Homotetramer. {ECO:0000250|UniProtKB:P24752}. SUBCELLULAR LOCATION: Mitochondrion {ECO:0000250|UniProtKB:P24752}. SIMILARITY: Belongs to the thiolase-like superfamily. Thiolase family. {ECO:0000305}. |
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