Université de Fribourg

Proteasomal degradation induced by DPP9-mediated processing competes with mitochondrial protein import

Finger, Yannik ; Habich, Markus ; Gerlich, Sarah ; Urbanczyk, Sophia ; Logt, Erik ; Koch, Julian ; Schu, Laura ; Lapacz, Kim Jasmin ; Ali, Muna ; Petrungaro, Carmelina ; Salscheider, Silja Lucia ; Pichlo, Christian ; Baumann, Ulrich ; Mielenz, Dirk ; Dengjel, Jörn ; Brachvogel, Bent ; Hofmann, Kay ; Riemer, Jan

In: The EMBO Journal, 2020, vol. 39, no. 19, p. e103889

Plasticity of the proteome is critical to adapt to varying conditions. Control of mitochondrial protein import contributes to this plasticity. Here, we identified a pathway that regulates mitochondrial protein import by regulated N-terminal processing. We demonstrate that dipeptidyl peptidases 8/9 (DPP8/9) mediate the N-terminal processing of adenylate kinase 2 (AK2) en route to mitochondria....

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...