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La L-Carnitina es un transportador de los cidos grasos (lpidos) a la mitocondria, encargada de la produccin de la energa de la clula, pero tambin es el lugar donde estos cidos grasos son convertidos en energa

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Primary systemic carnitine deficiency Primary systemic carnitine deficiency is a rare, autosomal recessive disorder caused by mutations (including deletions) in the SLC22A5 gene coding for carnitine transporter protein OCTN2 (organic cation transporter novel 2) (33)