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J Clin Invest (2020) 130(9):501126

The most robust links between oxidative stress or mitochondrial dysfunction and epilepsy are clear in mitochondrial encephalopathies such as myclonic epilepsy with ragged-red fibers (MERFF) and Leigh syndrome

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The series of biochemical transformations begins with the sidechain cleavage catalyzed by the cytochrome P450 enzyme CYP11A1 to give pregnenolone (Figure 7)