Abstract

ABSTRACTDuring the evolution from yeast to mammals the Mia40 protein, the regulator of the redox-sensitive mitochondrial intermembrane space import machinery, has lost its membrane-anchorage segment to become CHCHD4, which interacts with the flavoprotein apoptosis-inducing factor (AIF). Our results establish CHCHD4 as the missing link between AIF deficiency and dysfunctional biogenesis of respiratory chain complexes.

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