Inhibition of mitochondrial carnitine/acylcarnitine transporter by H2O2: Molecular mechanism and possible implication in pathophysiology

► H2O2 inhibits the mitochondrial carnitine/acylcarnitine transporter. ► Only mutants containing C136 and C155 were inactivated and inhibition was reverted by DTE. ► H2O2 reacts with C136 and C155 and inactivates the transporter by forming a disulphide. ► Inhibition by H2O2 could play a regulatory role of the mitochondrial ß-oxidation.