Biochemistry · Enzyme
Succinyl-CoA:3-ketoacid CoA transferase (SCOT) !
SCOT lets muscle, heart and brain use ketones: it moves CoA from succinyl-CoA onto acetoacetate.
Updated 2026-11-01
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Key facts
- Acetoacetate + succinyl-CoA → acetoacetyl-CoA + succinate; thiolase then gives 2 acetyl-CoA.
- Missing in the liver: that is why the liver exports ketones instead of burning them.
- Uses a Krebs cycle intermediate (succinyl-CoA): the GTP of succinyl-CoA synthetase is forgone.
Regulation
- Its activity depends on the supply of succinyl-CoA and acetoacetate.
- It rises in brain and muscle during prolonged fasting.
Inhibitors and toxins
- No inhibitors in clinical use
Clinical notes
- Its inherited deficiency causes severe intermittent ketoacidosis from infancy, with ketosis even after meals.
- Together with β-ketothiolase deficiency, it causes ketoacidosis with normal glucose in children.
Pathways
Related conditions
Related
- Acetoacetate (acetoacetic acid)
- β-Hydroxybutyrate
- Acetyl-CoA
- β-Hydroxybutyrate dehydrogenase (BDH1)
- Krebs cycle (citric acid cycle)
Test yourself
Think of the answer, then tap the question to see it.
Why can the liver not use the ketone bodies it makes?
It lacks SCOT, the enzyme that activates acetoacetate to acetoacetyl-CoA.
Where does SCOT take its coenzyme A from?
From Krebs cycle succinyl-CoA, which is left as succinate.
What does inherited SCOT deficiency cause?
Episodes of severe ketoacidosis with normal glucose and persistent ketosis even after eating.
Educational information, not medical advice. For any symptom, see a health professional.
Sources: OXCT1: Succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial (human) (UniProt, 2026); OXCT1 gene: MedlinePlus Genetics (MedlinePlus, US National Library of Medicine, 2026); Succinyl-CoA:3-ketoacid CoA transferase deficiency | MedlinePlus Genetics (MedlinePlus, US National Library of Medicine, 2026); 3DLX: Crystal structure of human 3-oxoacid CoA transferase 1 (RCSB Protein Data Bank, 2026).
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