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L-Carnitine is a quaternary ammonium compound derived from the amino acids lysine and methionine. It plays a critical role in cellular energy metabolism by facilitating the transport of long-chain fatty acids across the inner mitochondrial membrane via the carnitine shuttle system. This process enables β-oxidation of fatty acids, leading to the generation of acetyl-CoA and subsequent ATP production within the tricarboxylic acid cycle, particularly during periods of increased energy demand such as exercise or fasting. [mdpi.com], [encyclopedia.pub]
Endogenously, L-Carnitine is synthesized primarily in the liver and kidneys through a multi-step pathway involving Nε-trimethyllysine hydroxylase and related enzymes. Its homeostasis is maintained through renal reabsorption and regulated excretion. While the human body can produce sufficient amounts under normal conditions, L-Carnitine is also obtained from dietary sources, predominantly animal-based foods such as red meat and dairy products, which provide the majority of exogenous intake. [mdpi.com], [en.wikipedia.org] [ods.od.nih.gov]
Physiologically, approximately 95% of total body L-Carnitine is stored in skeletal and cardiac muscle, reflecting its importance in tissues with high oxidative capacity. Beyond its role in fatty acid oxidation, L-Carnitine contributes to maintaining mitochondrial acetyl-CoA/CoA balance and supports metabolic flexibility. [mdpi.com], [encyclopedia.pub]