PPARA and short chain acyl-CoA dehydrogenase deficiency: We also evaluated the effectof Bezafibrate (B+) (200 and 400 μmol/L) which is a pan-peroxisomeproliferator activated receptor (PPAR) agonist reported to restore deficientFAO rates in CPT2 and VLCAD fibroblasts [26].We initially evaluated the effects of Bezafibrate on the long-chain FAOD group,with the expectation that restoration of FAO would reduce oxidative stress.Bezafibrate has so far not been investigated in SCADD, however, if short-chainFAO were also restored by Bezafibrate, viability in the SCADD fibroblastsmight similarly be enhanced.