Nora Vögtle
ZMBH Research Group Leader
ZMBH
Im Neuenheimer Feld 345
69120 Heidelberg, Germany
Tel. +49 (0) 6221 - 54 6866
n.voegtle@zmbh.uni-heidelberg.de
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Welcome to the Vögtle lab!
Mitochondria are essential organelles well known for their role in ATP synthesis, multiple metabolic pathways or programmed cell death. They form a dynamic elongated network and are integrated into the cellular signalling network. To fulfill their various functions mitochondria have to build and maintain a complex proteome of more than 1000 different proteins. The vast majority of these mitochondrial proteins is imported post-translationally into the organelle and requires proteolytic processing to mature into functional and stable proteins. We are interested in the proteases that perform this processing and subsequent quality control of the incoming precursors. Maintenance of the mitochondrial proteome can also be challenged by stress or disease mutations and the cell protects mitochondrial proteostasis by eliciting transcriptionally responses ("mitochondrial unfolded protein response"). We are investigating how disease mutations affect mitochondrial proteostasis, how the cell is sensing these imbalances and how mitochondria are molecularly remodeled to cope with and overcome stress in form of toxic protein aggregates.
Selected publications Original Research Publications Taskin AA, Shankar S, Peselj C, Flotho A, Gomez-Fabra Gala M, Poveda-Huertes D, Myketin L, Mutlu D, Marada A, Schuck S, Jeske M, Büttner S, Luzarowski M, Meisinger C*, Vögtle FN*. (2026) Uncovering the initial response: Intra-mitochondrial surveillance activates the UPRmt. Mol. Cell 86: 2157-2172.e10. doi: 10.1016/j.molcel.2026.05.002. Marada A, Walter C, Suhm T, Shankar S, Nandy A, Brummer T, Dhaouadi I, Vögtle FN*, Meisinger C*. (2024) DYRK1A signalling synchronizes the mitochondrial import pathways for metabolic rewiring. Nat. Commun. 15: 5265. doi: 10.1038/s41467-024-49611-4. Moretti-Horten DN, Peselj C, Taskin AA, Myketin L, Schulte U, Einsle O, Drepper F, Luzarowski M, Vögtle FN. (2024) Synchronized assembly of the oxidative phosphorylation system controls mitochondrial respiration in yeast. Dev. Cell 59: 1043-1057.e8. doi: 10.1016/j.devcel.2024.02.011. Poveda-Huertes D, Taskin AA, Dhaouadi I, Myketin L, Marada A, Habernig L, Büttner S, Vögtle FN. (2021) Increased mitochondrial protein import and cardiolipin remodelling upon early mtUPR. PLoS Genet. 17: e1009664. doi: 10.1371/journal.pgen.1009664. Poveda-Huertes D, Matic S, Marada A, Habernig L, Licheva M, Myketin L, Gilsbach R, Tosal-Castano S, Papinski D, Mulica P, Kretz O, Kücükköse C, Taskin AA, Hein L, Kraft C, Büttner S, Meisinger C*, Vögtle FN*. (2020) An Early mtUPR: Redistribution of the Nuclear Transcription Factor Rox1 to Mitochondria Protects against Intramitochondrial Proteotoxic Aggregates. Mol. Cell 77: 180-188.e9. doi: 10.1016/j.molcel.2019.09.026. Vögtle FN*,#, Brändl B#, Larson A#, Pendziwiat M#, Friederich MW, White SM, Basinger A, Kücükköse C, Muhle H, Jähn JA, Keminer O, Helbig KL, Delto CF, Myketin L, Mossmann D, Burger N, Miyake N, Burnett A, van Baalen A, Lovell MA, Matsumoto N, Walsh M, Yu HC, Shinde DN, Stephani U, Van Hove JLK, Müller FJ, Helbig I*. (2018) Mutations in PMPCB Encoding the Catalytic Subunit of the Mitochondrial Presequence Protease Cause Neurodegeneration in Early Childhood. Am. J. Hum. Genet. 102: 557-573. doi: 10.1016/j.ajhg.2018.02.014. Vögtle FN#, Burkhart JM#, Gonczarowska-Jorge H, Kücükköse C, Taskin AA, Kopczynski D, Ahrends R, Mossmann D, Sickmann A, Zahedi RP, Meisinger C. (2017) Landscape of submitochondrial protein distribution. Nat. Commun. 8: 290. doi: 10.1038/s41467-017-00359-0. Eldomery MK#, Akdemir ZC#, Vögtle FN#, Charng WL, Mulica P, Rosenfeld JA, Gambin T, Gu S, Burrage LC, Al Shamsi A, Penney S, Jhangiani SN, Zimmerman HH, Muzny DM, Wang X, Tang J, Medikonda R, Ramachandran PV, Wong LJ, Boerwinkle E, Gibbs RA, Eng CM, Lalani SR, Hertecant J, Rodenburg RJ, Abdul-Rahman OA, Yang Y, Xia F, Wang MC, Lupski JR, Meisinger C, Sutton VR. MIPEP recessive variants cause a syndrome of left ventricular non-compaction, hypotonia, and infantile death. Genome Med. 8: 106. doi: 10.1186/s13073-016-0360-6. 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. (2014) Amyloid-β peptide induces mitochondrial dysfunction by inhibition of preprotein maturation. Cell Metab. 20: 662-669. doi: 10.1016/j.cmet.2014.07.024. Vögtle FN#, Wortelkamp S#, Zahedi RP, Becker D, Leidhold C, Gevaert K, Kellermann J, Voos W, Sickmann A, Pfanner N, Meisinger C. (2009) Global analysis of the mitochondrial N-proteome identifies a processing peptidase critical for protein stability. Cell 139: 428-439. doi: 10.1016/j.cell.2009.07.045. * corresponding
Review Articles Gomez-Fabra Gala M, Vögtle FN. (2021) Mitochondrial proteases in human diseases. FEBS Lett. 595: 1205-1222. doi: 10.1002/1873-3468.14039. Vögtle FN. (2021) Open questions on the mitochondrial unfolded protein response. FEBS J. 288: 2856-2869. doi: 10.1111/febs.15569. Poveda-Huertes D, Mulica P, Vögtle FN. (2017) The versatility of the mitochondrial presequence processing machinery: cleavage, quality control and turnover. Cell Tissue Res. 367: 73-81. doi: 10.1007/s00441-016-2492-9.
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