Reaction: OXCT dimers transfer CoA from SUCC-CoA to ACA, forming ACA-CoA
- in pathway: Utilization of Ketone Bodies
Mitochondrial succinyl-CoA:3-ketoacid coenzyme A transferases 1 and 2 (OXCT1 and OXCT2) are key enzymes for the metabolism of ketone bodies, catalysing the first rate-limiting step of ketone body utilisation in peripheral tissues. In dimeric form, they mediate the transfer of a CoA moiety from succinyl-CoA (SUCC-CoA) to acetoacetate (ACA) to form acetoacetyl-CoA (ACA-CoA) and succinate (SUCCA) (Kassovska-Bratinova et al. 1996, Tanaka et al. 2002). ACA-CoA can be converted to acetyl-CoA which can be utilised by the tricarboxylic acid cycle for energy production.
Reaction - small molecule participants:
ACA-CoA [mitochondrial matrix]
SUCCA [mitochondrial matrix]
SUCC-CoA [mitochondrial matrix]
ACA [mitochondrial matrix]
Reactome.org reaction link: R-HSA-74177
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Reaction input - small molecules:
succinyl-CoA(5-)
acetoacetic acid
Reaction output - small molecules:
acetoacetyl-CoA(4-)
succinate(2-)
Reactome.org link: R-HSA-74177