ABSTRACT

Introduction L-Carnitine (β-hydroxy-γ-trimethyl-amino-butyric acid) is a conditionally essential nutrient that was discovered more than a century ago (Bieber 1988). It is widely distributed in biological tissues of all mammals and performs several functions in addition to its essential role in membrane trafficking of fatty acids and facilitating long-chain fatty acid oxidation (Fritz 1963). In an average 70-kg adult, the total amount of carnitine is estimated to be around 100 mmol (≈16 g) but can vary depending on the muscle mass, which contains approximately 98% of this amount. Only 1.6% of the L-carnitine is found in the liver and kidneys, with the remainder found in the plasma and all other tissues (Engel and Rebouche 1984). These facts

Introduction ...................................................................................................... 11 Carnitine homeostasis ..................................................................................... 12 Carnitine system components and associated carnitine insufficiency ..... 14 Carnitine metabolic effects on cardiac and skeletal muscle ....................... 18

A role for CAT in acetyl CoA buffering: Implications in cardiac metabolic remodeling ................................................................................. 18 Potential role for CAT in malonyl CoA axis ............................................ 21 Carnitine in insulin resistance and metabolic inflexibility .................... 22

Conclusion ........................................................................................................ 26 Acknowledgments ........................................................................................... 26 References .......................................................................................................... 27

profoundly emphasize the critical role of carnitine in the physiology of cardiac and skeletal muscle.