Succinyl CoA:3-oxoacid CoA transferase (SCOT) deficiency

Definition:

Succinyl CoA:3-oxoacid CoA transferase (SCOT) deficiency is an inborn error of ketone body metabolism. SCOT/OXCT1 is a key mitochondrial enzyme in the metabolism of ketone bodies in various organs. Deficiency of SCOT activity inhibits peripheral ketone body utilization and causes episodes of severe ketoacidosis.

References:

[1]. Jörn Oliver Sass, et al. Inborn errors of ketogenesis and ketone body utilization. J Inherit Metab Dis. 2012 Jan;35(1):23-8. [Content Brief]

[2]. K E Niezen-Koning, et al. Succinyl-CoA:acetoacetate transferase deficiency: identification of a new patient with a neonatal onset and review of the literature. Eur J Pediatr. 1997 Nov;156(11):870-3. [Content Brief]

[3]. Toshiyuki Fukao, et al. Clinical and molecular characterization of five patients with succinyl-CoA:3-ketoacid CoA transferase (SCOT) deficiency. Biochim Biophys Acta. 2011 May;1812(5):619-24. [Content Brief]

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