Université de Fribourg

Vectorial import via a metastable disulfide-linked complex allows for a quality control step and import by the mitochondrial disulfide relay

Habich, Markus ; Salscheider, Silja Lucia ; Murschall, Lena Maria ; Hoehne, Michaela Nicole ; Fischer, Manuel ; Schorn, Fabian ; Petrungaro, Carmelina ; Ali, Muna ; Erdogan, Alican J. ; Abou-Eid, Shadi ; Kashkar, Hamid ; Dengjel, Jörn ; Riemer, Jan

In: Cell Reports, 2019, vol. 26, no. 3, p. 759-774.e5

Disulfide formation in the mitochondrial intermembrane space (IMS) is an essential process. It is catalyzed by the disulfide relay machinery, which couples substrate import and oxidation. The machinery relies on the oxidoreductase and chaperone CHCHD4-Mia40. Here, we report on the driving force for IMS import and on a redox quality control mechanism. We demonstrate that unfolded reduced...