Fatty Acid Accumulation and Resulting PPAR Activation in Fibroblasts due to Trifunctional Protein Deficiency
Figure 1
Acyl-CoA dehydrogenase activity and FFA/TG contents in fibroblasts. Assay methods were, respectively, described in Section 2. (a) Indicates palmitoyl-CoA (open bar, C16) and octanoyl-CoA (closed bar, C8) dehydrogenase activities, respectively. (b) Indicates FFA (open bar) and TG (closed bar) contents, respectively. P1–P6, individual patient’s fibroblast; C1–C3, individual control fibroblast; P, means ± SD in six patients’ fibroblasts; C, means ± SD in three control fibroblasts. * versus controls.
