Impaired fat oxidation during exercise in multiple acyl-CoA dehydrogenase deficiency.

ABSTRACT: We investigated the in vivo skeletal muscle metabolism in patients with multiple acyl-CoA dehydrogenase deficiency (MADD) during exercise, and the effect of a glucose infusion. Two adults with MADD on riboflavin and l-carnitine treatment and 10 healthy controls performed an incremental exercise test measuring maximal oxidative capacity (VO_2max) and a submaximal exercise test (≤1 hour) on a cycle ergometer. During submaximal exercise, we studied fat and carbohydrate oxidation, using stable isotope tracer methodology and indirect calorimetry. On another day, the patients repeated the submaximal exercise receiving a 10% glucose infusion. The patients had a lower VO_2max than controls and stopped the submaximal exercise test at 51 and 58 minutes due to muscle pain and exha

SUBMITTER: Madsen KL 

PROVIDER: S-EPMC6498824 | biostudies-literature | 2019 Mar

REPOSITORIES: biostudies-literature