The effects of carnitine on mitochondria may help prevent disease and is a possible therapeutic prospect of several illnesses, including long chain fatty acid oxidation disorder (LCFAOD), according to a new study published in the International Journal of Molecular Sciences.

The idea of carnitine supplementation helping to carry long-chain fatty acids (LCFA) into the mitochondria and improve oxidation seems logical. Carnitine has been on the map as a possible treatment for LCFAOD, however, there is still a lack of studies supporting this therapeutic approach. It is also not exempt from possible complications such as life-threatening arrhythmias or toxic intramitochondrial LCFA accumulation.

The authors aimed to review the crucial function of carnitine in mitochondrial function, metabolic flexibility, and other pathophysiological cellular mechanisms. They describe the development of several chronic diseases, including neurodegenerative diseases, insulin resistance, and more, linked to metabolic inflexibility and mitochondrial dysfunction. The dysfunction comes from a lack of substrates or micronutrients, and carnitine is one of the major players of adequate mitochondrial functioning and its primary role in fatty acid oxidation.

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“Together with body control systems, mitochondria are key players in maintaining energy requirements and cell function,” the authors said. “This will ensure a healthy body homeostasis and prevent the initiation of disease processes . . . Improving mitochondria function with a balanced diet and lifestyle but also correcting nutrient deficiencies can play a crucial role in maintaining metabolic flexibility and health.”

Moreover, the authors noted that carnitine deficiencies exist in multiple diseases, such as organic acidurias, inborn errors of metabolism, endocrine imbalances, and more.

“Extensive preclinical and clinical research studies have confirmed the positive role that l-carnitine treatment has,” they said. “New recent research is beginning to show that l-carnitine can also modulate gene expression and other critical biological processes, in addition to its key role in mitochondrial energy metabolism.”

Keeping in mind that carnitine likewise helps protect cellular membranes, eliminate toxic metabolites, protect cellular membranes, and modulate metabolic pathways like glucogenesis and ketogenesis, supplementation of carnitine may hold therapeutic and prevention potential in the future, even for LCFAOD. However, more studies are needed.

“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,” the authors concluded.


Virmani MA, Cirulli M. The role of L-carnitine in mitochondria, prevention of metabolic inflexibility and disease initiationInt J Mol Sci. 2022;23(5):2717. doi:10.3390/ijms23052717