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Importantly, secondary FAD depletion has been observed in diseases without primary flavoprotein mutationssuch as L-2-hydroxyglutaric aciduria and Friedreichs ataxia

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This mitochondrial fraction is functionally critical, as demonstrated by the restoration of ferroptosis sensitivity in CHAC1-null cells through expression of a mitochondrially targeted CHAC1 (Ward et al., 2024)