Maria Sharapova (and hundreds of other elite athletes) took meldonium, a drug developed at the time of the USSR for the treatment of heart attack and stroke, though it has never been approved for use anywhere outside of the former Soviet Union. Meldonium is an inhibitor of -butyrobetaine hydroxylase, an enzyme involved in the carnitine biosynthetic pathway.1 Intake results in a reduction of tissue carnitine content, including the heart and skeletal muscles. Carnitine plays a critical role in transferring long-chain fatty acids across mitochondrial inner membrane into the mitochondrial matrix, to enable entry of the fatty acid moiety into the oxidation pathway, to synthesise ATP aerobically.2 In cases when intracellular carnitine availability is compromised, such as in neonatal primary carnitine deficiency,3 those affected experience hypoglycaemia, fatigue, seizures and cardiomyopathy.3 Carnitine-deficient individuals are sensitive to prodrugs containing the pivaloyl moiety, as pivalic acid...
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