Carnitine (Levocarnitine)
Pharmacology
Levocarnitine (l-carnitine) is an endogenous carboxylic acid that facilitates transport of long-chain fatty acids into mitochondria for beta-oxidation and prevents intracellular accumulation of toxic acyl-CoA. l-Carnitine is ubiquitous in diets rich in meats and dairy products and is also synthesized in the body from the amino acids lysine and methionine. Although dietary deficiencies are rare, hypocarnitinemia can result from certain medical conditions and inborn errors of metabolism, and it may develop in patients receiving multiple anticonvulsant medications. It is hypothesized that valproic acid (VPA [See Valproic acid]) causes carnitine deficiency, resulting in mitochondrial dysfunction. The resultant impaired beta-oxidation favors production of toxic VPA metabolites via microsomal oxidation. These metabolites are implicated in hepatotoxicity and urea cycle disruption causing hyperammonemia. Supplementation with l-carnitine has been shown to be beneficial in both the prevention and the treatment of hyperammonemia associated with VPA therapy, and it may improve the outcome in cases of VPA-induced hepatotoxicity and encephalopathy.
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