FIG. 7.
Effect of 4PBA treatment (5 mM) on VLCFA (C24:0) β-oxidation specific activity in mitochondrial fatty acid metabolism mutants. Fibroblasts with mutations affecting CPT1, (V)LCAD, MCAD, and SCAD were exposed to 4PBA for 3 days. Cells lacking the ability to either transport LCFA across the mitochondrial membrane (CPT1) or metabolize LCFA [(V)LCAD] did not respond to 4PBA. However, MCAD- and SCAD-deficient cells, with normal LCFA metabolism, responded to 4PBA with increased LCFA (C16:0) and peroxisomal VLCFA (C24:0) β-oxidation activity. Results are shown as means ± the standard deviations with the number of experiments performed in parentheses.