CHCHD4 links AIF to the biogenesis of respiratory chain complex I.
During 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.
Equipe 11 labellisée Ligue Nationale contre le Cancer, Center de Recherche des Cordeliers, Paris, France; Institut National de la Santé et de la Recherche Médicale, U1138, Paris, France; Institut Gustave Roussy Cancer Campus, Villejuif, France; Faculty of Medicine, University of Paris Sud, Kremlin-Bicêtre, France.
Nazanine
N
Modjtahedi
Equipe 11 labellisée Ligue Nationale contre le Cancer, Center de Recherche des Cordeliers, Paris, France; Institut National de la Santé et de la Recherche Médicale, U1138, Paris, France; Institut Gustave Roussy Cancer Campus, Villejuif, France; Metabolomics and Cell Biology Platforms, Gustave Roussy Cancer Campus, Villejuif, France; Université Paris Descartes, Sorbonne Paris Cité, Paris, France; Université Pierre et Marie Curie, Paris, France; Pôle de Biologie, Hôpital Européen Georges Pompidou, AP-HP, Paris, France.
Guido
G
Kroemer
Disulfide relay; mitochondrial import; mitochondriopathy; respiratory chain complexes