L-carnitine is formed endogenously from lysine and methionine in the cells of the
of the liver, kidneys and brain, and is supplied to the body with
food, mainly beef and dairy. L-carnitine is involved
involved in lipid metabolism by transporting long-chain
fatty acids into the mitochondria, and also participates in the
the conversion of acetyl-coenzymeA (acetyl-CoA) to acetylcarnitine and
Coenzyme A (CoA), thereby increasing the availability of CoA needed for the
oxidative decarboxylation of pyruvate in the Krebs cycle. W
nervous system, acetylcarnitine is a donor of acetyl groups
necessary for the formation of acetylcholine. L-carnitine also has
antioxidant activity. In the body, L-carnitine occurs in the
free form or as esters of L-acetylcarnitine and
L-propionylcarnitine.
L-carnitine determination is performed in cases of suspected
primary or secondary deficiencies.
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