Patients with medium-chain acyl-coenzyme A dehydrogenase deficiency have impaired oxidation of fat during exercise but no effect of L-carnitine supplementation
Madsen KL, Preisler N, Orngreen MC
Published in The Journal of Clinical Endocrinology and Metabolism
Methodology
Stable isotope and indirect calorimetry study of 1-hour cycling in 4 patients with MCAD deficiency and 11 controls, before and after 4 weeks of 100 mg/kg/day L-carnitine
Key Findings
Palmitate oxidation and total fatty acid oxidation rates were unchanged by the L-carnitine supplementation
Conclusions
Carnitine does not rescue fatty acid oxidation when the enzymatic block is the limiting step
Limitations
Only four patients; rare metabolic disorder
Supplements Studied
Palmitate oxidation and FAO rates were unchanged by the l-carnitine supplementation.
Outcomes Measured
Palmitate oxidation and FAO rates were unchanged by the l-carnitine supplementation.
Study Details
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