Frommitochondrial biogenesisto mitochondrial dysfunction and protective cellular responses
Mitochondria are essential, dynamic organelles that are critical for cell survival, produce most of the cellular ATP and host key metabolic processes. To fulfil all their diverse roles, mitochondria require an extensive proteome of more than 1000 different proteins. Mitochondrial dysfunction is associated with many metabolic and neurodegenerative disorders, but the molecular causes of these dysfunctions and how cells cope with mitochondrial defects are often elusive. In our laboratory, we investigate how mitochondria build and maintain their extensive proteome by analyzing mitochondrial protein biogenesis and characterizing the subsequent protein quality control mechanisms that maintain proteome stability. In particular, we aim to uncover the molecular mechanisms that preserve mitochondrial activity by protecting mitochondria from proteotoxic stress and to identify the protective mechanisms that restore mitochondrial proteostasis.
Selected publications
Marada A, Walter C, Suhm T, Shankar S, Nandy A, Brummer T, Dhaouadi I, Vögtle FN*, Meisinger C*
Nat. Commun. 2024
DYRK1A signalling synchronizes the mitochondrial import pathways for metabolic rewiring.
Moretti-Horten DN, Peselj C, Taskin AA, Myketin L, Schulte U, Einsle O, Drepper F, Luzarowski M, Vögtle FN
Dev. Cell 2024
Synchronized assembly of the oxidative phosphorylation system controls mitochondrial respiration in yeast.
Poveda-Huertes D, Taskin AA, Dhaouadi I, Myketin L, Marada A, Habernig L, Büttner S, Vögtle FN
PLoS Genet. 2021
Increased mitochondrial protein import and cardiolipin remodelling upon early mtUPR.
Landscape of submitochondrial protein distribution.
Vögtle FN*, Keller M, Taskin AA, Horvath SE, Guan XL, Prinz C, Opalińska M, Zorzin C, van der Laan M, Wenk MR, Schubert R, Wiedemann N, Holzer M, Meisinger C*
J. Cell Biol. 2015
The fusogenic lipid phosphatidic acid promotes the biogenesis of mitochondrial outer membrane protein Ugo1.
Mossmann D, Vögtle FN, Taskin AA, Teixeira PF, Ring J, Burkhart JM, Burger N, Pinho CM, Tadic J, Loreth D, Graff C, Metzger F, Sickmann A, Kretz O, Wiedemann N, Zahedi RP, Madeo F, Glaser E, Meisinger C
Cell Metab. 2014
Amyloid-β peptide induces mitochondrial dysfunction by inhibition of preprotein maturation.
Vögtle FN, Wortelkamp S, Zahedi RP, Becker D, Leidhold C, Gevaert K, Kellermann J, Voos W, Sickmann A, Pfanner N, Meisinger C
Cell 2009
Global analysis of the mitochondrial N-proteome identifies a processing peptidase critical for protein stability.