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Abstract
Mitochondria control cellular fate by various mechanisms and are key drivers of cellular metabolism. Although the main function of mitochondria is energy production, they are also involved in cellular detoxification, cellular stabilization, as well as control of ketogenesis and glucogenesis. Conditions like neurodegenerative disease, insulin resistance, endocrine imbalances, liver and kidney disease are intimately linked to metabolic disorders or inflexibility and to mitochondrial dysfunction. Mitochondrial dysfunction due to a relative lack of micronutrients and substrates is implicated in the development of many chronic diseases. l-carnitine is one of the key nutrients for proper mitochondrial function and is notable for its role in fatty acid oxidation. l-carnitine also plays a major part in protecting cellular membranes, preventing fatty acid accumulation, modulating ketogenesis and glucogenesis and in the elimination of toxic metabolites. l-carnitine deficiency has been observed in many diseases including organic acidurias, inborn errors of metabolism, endocrine imbalances, liver and kidney disease. The protective effects of micronutrients targeting mitochondria hold considerable promise for the management of age and metabolic related diseases. Preventing nutrient deficiencies like l-carnitine can be beneficial in maintaining metabolic flexibility via the optimization of mitochondrial function. This paper reviews the critical role of l-carnitine in mitochondrial function, metabolic flexibility and in other pathophysiological cellular mechanisms.
Highlights
L-carnitine is a vital molecule that is found in all living cells
L-Carnitine, Mitochondria and Cellular Metabolism Metabolism is a coordinated series of chemical reactions that occur in cells of l2ivofin16g organisms, needed to create and sustain life
L-carnitine has a key role in the regulation of acyl-Coenzyme A (CoA) and acetyl-CoA, and in the preservation of free CoA levels within the mitochondria, which is crucial for maintaining metabolic flexibility
Summary
L-carnitine is a vital molecule that is found in all living cells It is a quaternary amine (3-hydroxy-4-N-trimethylaminobutyrate) whose main function in mammalian cells is the transfer of long chain fatty acids across the inner mitochondrial membrane for β- oxidation and generation of ATP energy. This process requires specific enzymes and transporters and any defects in these can cause disorders of the carnitine cycle. L-Carnitine, Mitochondria and Cellular Metabolism Metabolism is a coordinated series of chemical reactions that occur in cells of l2ivofin16g organisms, needed to create and sustain life These biochemical processes are important for the storage, freeing, and use of energy, and for the proliferation and repair of cells.
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