Normal rates of whole-body fat oxidation and gluconeogenesis after overnight fasting and moderate-intensity exercise in patients with medium-chain acyl-CoA dehydrogenase deficiency.
Huidekoper HH, Ackermans MT, Koopman R, van Loon LJ, Sauerwein HP, Wijburg FA
Journal of inherited metabolic disease, 2013 Sep
Abstract
Impairments in gluconeogenesis have been implicated in the pathophysiology of fasting hypoglycemia in medium-chain acyl-CoA dehydrogenase deficiency. However, whole body glucose and fat metabolism have never been studied in vivo.