CHCHD4 links AIF to the biogenesis of respiratory chain complex I

Nazanine Modjtahedi; Guido Kroemer

doi:10.1080/23723556.2015.1074332

CHCHD4 links AIF to the biogenesis of respiratory chain complex I

Mol Cell Oncol. 2015 Jul 29;3(2):e1074332. doi: 10.1080/23723556.2015.1074332. eCollection 2016 Mar.

Authors

Nazanine Modjtahedi¹, Guido Kroemer²

Affiliations

¹ 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.
² 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.

Abstract

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.

Keywords: Disulfide relay; mitochondrial import; mitochondriopathy; respiratory chain complexes.