Published in:
01-01-2017 | Editorial
Ketones and inborn errors of metabolism: old friends revisited
Published in: Journal of Inherited Metabolic Disease | Issue 1/2017
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During exercise fat and carbohydrates are the principal substrates that fuel aerobic ATP synthesis in human skeletal muscle. During low-intensity exercise, skeletal muscle relies predominantly on fat-based fuels, thereby sparing the intracellular glycogen stores. During high-intensity exercise, however, there is a shift in fuel selection from fat towards glycolysis and the utilization of glycogen reserves. When glycogen reserves turn low during prolonged intense exercise, but also upon fasting and starvation, ketone bodies such as 3-β-hydroxybutyrate (β-HB) and acetoacetate which are produced by the liver, are increasingly important sources of ATP (Fig. 1).
Fig. 1
Schematic representation of ketolysis. The ketone bodies β-hydroxybutyrate (β-HB) and acetoacetate (AcAc) are broken down to acetyl-CoA via aceto-acetyl-CoA (AcAc-CoA). A ketone ester supplement remodelled substrate utilization of intracellular glycogen stores, free-fatty acids (FFA) and branched-chain amino acids (BCAA) oxidation during exercise (Cox et al 2016). A non-exhaustive list of inborn errors of metabolism that may benefit from the therapeutic potential of this ketone ester supplement are listed. Suc-CoA: succinyl-CoA
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