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33, 34 In some patients, fibroblasts obtained from a skin biopsy were cultured

Eur J Pediatr 1995;154:871-7

Notably, no redox biomarkers were collected in this trial, which limits mechanistic interpretation

Los steres de cidos grasos (de la CoA) se forman casi exclusivamente en el citosol y no se transportan a travs de la membrana, inhibe tambin enzimas de ciclo de Krebs, por ende la oxidacin de cidos grasos requiere la formacin de acilcarnitinas y su translocacin hacia las mitocondrias, donde se vuelven a formar steres de CoA y se metabolizan produciendo energa

The products of fatty-acid oxidation and how they enter the citric acid cycle are dependent on the length of the fatty acid, specifically whether the fatty acid is odd or even in length