Effect of heat stress and bezafibrate on mitochondrial β-oxidation: Comparison between cultured cells from normal and mitochondrial fatty acid oxidation disorder children using in vitro probe acylcarnitine profiling assay

摘要: Abstract Hyperpyrexia occasionally triggers acute life-threatening encephalopathy-like illnesses, including influenza-associated encephalopathy (IAE) in childhood, and can be responsible for impaired fatty acid β-oxidation (FAO). In this regard, patients with FAO may more susceptible to febrile episodes. The effects of heat stress a hypolipidemic drug, bezafibrate, on mitochondrial were investigated using cultured cells from children disorders normal controls, an vitro probe acylcarnitine (AC) profiling assay. Fibroblasts incubated medium loaded unlabelled palmitic 96 h at 37 41 °C, or without bezafibrate. AC profiles culture analyzed by electrospray ionization tandem mass spectrometry. Heat stress, introduced significantly increased acetylcarnitine (C2) but slightly decreased the other acylcarnitines (ACs) controls medium-chain acyl-CoA dehydrogenase (MCAD)-deficient cells. On hand, very long-chain (VLCAD)-deficient cells, accumulation ACs enhanced compared that 37 °C. contrast, bezafibrate significant increase C2 both control VLCAD-deficient These data suggest specifically inhibits FAO, whereas recovers FAO. Our approach is simple promising strategy evaluate therapeutic drugs