l-Carnitine therapy improves right heart dysfunction through Cpt1-dependent fatty acid oxidation.

There was a nonsignificant trend towards a decrease in acylcarnitine to free carnitine ratio in the RV of R899X mice that were treated with l ‐carnitine (Figure 3d ). l ‐Carnitine increases fatty acid mediated mitochondrial respiration and β‐oxidation of fatty acids To assess whether l ‐carnitine supplementation improved fatty acid‐mediated mitochondrial respiration, a subset of mice were killed and the RV tissue was immediately permeabilized for measurement of oxygen respiration in response to various substrates and inhibitors of mitochondrial respiration.

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