期刊
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
卷 23, 期 5, 页码 -出版社
MDPI
DOI: 10.3390/ijms23052717
关键词
mitochondrial function; l-carnitine; fatty acid oxidation; glycolysis; ketogenesis; beta oxidation; metabolic inflexibility; diabetes; neurodegeneration; liver disease
Mitochondria play a critical role in cellular fate and metabolism, and their dysfunction is implicated in various diseases. l-carnitine, an important nutrient for proper mitochondrial function, is involved in fatty acid oxidation and has protective effects on cellular membranes. It also modulates ketogenesis, glucogenesis, and the elimination of toxic metabolites. Deficiency in l-carnitine is observed in many diseases and addressing nutrient deficiencies, like l-carnitine, holds promise for managing age and metabolic related diseases.
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.
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