Antioxidantes/uso terapéutico; Encefalopatías Metabólicas Innatas/tratamiento farmacológico; Carnitina/uso terapéutico; Suplementos Dietéticos; Fármacos Neuroprotectores/uso terapéutico; Animales; Carnitina/deficiencia; Carnitina/farmacología; Humanos; Fármacos Neuroprotectores/farmacología

4-hydroxy-2-nonenal; ALC; ATP; Antioxidants; BCAA; BCKAD; CAT; CNS; CPT; GPx; GSH; H(2)O(2); HD; HNE; HSP; Huntington's disease; LC; LCAD; LCHAD; MCAD; MDA; MSUD; Neurometabolic disorders; Oxidative stress; PI3K; PKU; PLC; ROS; SOD; TAR; TBARS; VLCAD; acetyl-l-carnitine; adenosine triphosphate; branched-chain amino acids; branched-chain α-keto acid dehydrogenase complex; carnitine palmitoyltransferase; catalase; central nervous system; glutathione peroxidase; heat shock protein; hydrogen peroxide; l-Carnitine; long-chain 3-hydroxyacyl-CoA dehydrogenase; long-chain acyl-CoA dehydrogenase; malondialdehyde; maple syrup urine disease; medium-chain acyl-coenzyme A dehydrogenase; phenylketonuria; phosphoinositol-3 kinase; propionyl-l-carnitine; reactive oxygen species; reduced glutathione; superoxide dismutase; thiobarbituric acid reactive species; total antioxidant reactivity; very-long-chain acyl-CoA dehydrogenase