GTR Home > Conditions/Phenotypes > Succinyl-CoA acetoacetate transferase deficiency

Summary

Ketone bodies are major vectors of energy transfer from the liver to extrahepatic tissues and are the main source of lipid-derived energy for the brain. Mitchell et al. (1995) reviewed medical aspects of ketone body metabolism, including the differential diagnosis of abnormalities. As the first step of ketone body utilization, succinyl-CoA:3-oxoacid CoA transferase (SCOT, or OXCT1; EC 2.8.3.5) catalyzes the reversible transfer of CoA from succinyl-CoA to acetoacetate. [from OMIM]

Available tests

16 tests are in the database for this condition.

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  • Also known as: OXCT, SCOT, OXCT1
    Summary: 3-oxoacid CoA-transferase 1

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