Fatty Acid Accumulation and Resulting PPAR Activation in Fibroblasts due to Trifunctional Protein Deficiency
Figure 4
Effects of the MK886 or fenofibrate treatment on MCAD expression. The fibroblasts were plated in dishes and allowed to grow to 80% confluence. MK886 (30 μM final concentration) and fenofibrate (200 μM final concentration) were added to cell culture media, respectively. Both chemicals were dissolved in DMSO, and the final concentration of DMSO in media was maintained at 0.05% (v/v) in all cases. After 6 h, fibroblasts were harvested and used to analyze MCAD expression. (a) Shows relative quantification of expression level of MCAD protein. Upper panel indicates protein bands in immunoblot analysis. The band of actin was used as the loading control. Protein bands of two patients (P1 and P2) and a control (C1) are shown due to space limitation. Lower panel indicates relative protein amounts obtained by immunoblot and densitometric analyses. (b) Shows relative mRNA expression. Open bar, no treatment; gray bar, MK886 treatment; closed bar, fenofibrate (FF) treatment. 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; #, no treatment versus MK886 or fenofibrate treatment.
