Reactome | OXCT dimers transfer CoA from SUCC-CoA to ACA, forming ACA-CoA

OXCT dimers transfer CoA from SUCC-CoA to ACA, forming ACA-CoA

Stable Identifier

R-HSA-74177

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix

Synonyms

acetoacetate + succinyl-CoA <=> acetoacetyl-CoA + succinate

ReviewStatus

5/5

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OXCT dimers transfer CoA from SUCC-CoA to ACA, forming ACA-CoA

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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.

Literature References

PubMed ID	Title	Journal	Year
8751852	Succinyl CoA: 3-oxoacid CoA transferase (SCOT): human cDNA cloning, human chromosomal mapping to 5p13, and mutation detection in a SCOT-deficient patient Kondo, N, Pérez-Cerda, C, Kassovska-Bratinova, S, Vobecky, S, Fukao, T, Ugarte, M, Robert, MF, Duncan, AM, Mitchell, GA, Chartrand, C, Song, XQ, Chen, HS	Am J Hum Genet	1996
11756565	Cloning and characterization of a human orthologue of testis-specific succinyl CoA: 3-oxo acid CoA transferase (Scot-t) cDNA Kohroki, J, Tanaka, H, Nishimune, Y, Onishi, M, Iguchi, N	Mol. Hum. Reprod.	2002