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By helping transport fatty acids into the mitochondria, L-carnitine plays a key role in turning stored fat into usable energy

The hearts constant need for energy expenditure and its heavy dependence on fatty acids (the cardiac muscle obtains 5070% of its energy from fatty acids) explain why patients with primary carnitine deficiency can develop cardiomyopathy, usually between 1 and 4 years of age, that responds poorly to standard therapy [3, 90, 106108]

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