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L-Karnitin, genellikle karacier ve bbreklerde sentezlenen, lizin ve metiyonin amino asitlerinden oluan bir drdncl amonyum katyonudur

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This pathway can be interrupted by the introduction of VPA, which leads to a reduction of free coenzyme A, acetyl-CoA, and carnitine, and results in the decreased availability of cofactors necessary for the function of urea cycle and an increase in NH3 production.[15,16] Because VPA-induced metabolic disorders including hyperammonemia could be mediated at least in part by carnitine deficiency, it has been hypothesized that levocarnitine supplementation may prevent, correct, or attenuate these adverse effects[15,17] Several studies or isolated clinical cases have suggested the potential value of oral levocarnitine in reversing carnitine deficiency or preventing adverse effects due to VPA-induced dysfunction of beta-oxidation