Mitochondria are well known for their crucial role in oxygen dependent cellular respiration to synthesize ATP, required for intracellular energy transfer. We study the biogenesis of mitochondria by in vivo and in vitro experiments, e.g. by mutagenesis of genes encoding crucial proteins for mitochondrial protein transport, isolation and analysis of mitochondrial protein complexes and by import assays of precursor proteins into isolated mitochondria. We will analyse how different metabolic and stress-conditions alter the dynamics and composition of mitochondrial protein complexes, required for processes from biogenesis to degradation. In addition, we aim to identify rare and transient regulatory assemblies of mitochondrial super-complexes, to characterize of the plasticity of mitochondrial membrane protein complexes as signalling hubs controlling the regulation of mitochondrial biogenesis, respiration and morphology.

Keywords:

mitochondria, assembly, biogenesis, protein transport, respiration, super-complex, translocase, regulation

10 selected publications:

• Quantitative high-confidence human mitochondrial proteome and its dynamics in cellular context.
  Morgenstern M, Peikert CD, Lübbert P, Suppanz I, Klemm C, Alka O, Steiert C, Naumenko N, Schendzielorz A, Melchionda L, Mühlhäuser WWD, Knapp B, Busch JD, Stiller SB, Dannenmaier S, Lindau C, Licheva M, Eickhorst C, Galbusera R, Zerbes RM, Ryan MT, Kraft C, Kozjak-Pavlovic V, Drepper F, Sven Dennerlein S, Oeljeklaus S, Pfanner N, Wiedemann N*, Warscheid B* (2021)
  Cell Metab. 33, 2464-2483.e18. (* corresponding)
• Mitochondrial sorting and assembly machinery operates by β-barrel switching.
  Takeda H, Tsutsumi A, Nishizawa T, Lindau C, Busto JV, Wenz L-S, Ellenrieder L, Imai K, Straub SP, Mossmann W, Qiu J, Yamamori Y, Tomii K, Suzuki J, Murata T, Ogasawara S, Nureki O, Becker T, Pfanner N, Wiedemann N, Kikkawa N, Endo T (2021)
  Nature 590, 163–169.
• Membrane protein insertion through a mitochondrial β-barrel gate.
  Höhr AIC, Lindau C, Wirth C, Qiu J, Stroud DA, Kutik S, Guiard B, Hunte C, Becker T, Pfanner N, Wiedemann N (2018).
  Science 359, eaah6834.
• Structural basis of membrane protein chaperoning through the mitochondrial intermembrane space.
  Weinhäupl K, Lindau C, Hessel A, Wang Y, Schütze C, Jores T, Melchionda L, Schönfisch B, Kalbacher H, Bersch B, Rapaport D, Brennich M, Lindorff-Larsen K, Wiedemann N*, Schanda P* (2018)
  Cell 175, 1365-1379.e25. (*corresponding)
• Definition of a High-Confidence Mitochondrial Proteome at Quantitative Scale.
  Morgenstern M, Stiller SB, Lübbert P, Peikert CD, Dannenmeier S, Drepper F, Weill U, Höß P, Feuerstein R, Gebert M, Bohnert M, van der Laan M, Schuldiner M, Schütze C, Oeljeklaus S, Pfanner N, Wiedemann N*, Warscheid B* (2017).
  Cell Rep. 19, 2836–2852. (* corresponding)
• Mitochondrial OXA translocase plays a major role in biogenesis of inner-membrane proteins.
  Stiller SB, Höpker J, Oeljeklaus S, Schütze C, Schrempp SG, Vent-Schmidt J, Horvath SE, Frazier AE, Gebert N, van der Laan M, Bohnert M, Warscheid B, Pfanner N, Wiedemann N (2016).
  Cell Metabolism 23, 901–908.
• Coupling of Mitochondrial Import and Export Translocases by Receptor-Mediated Supercomplex Formation.
  Qiu J, Wenz L-S, Zerbes RM, Oeljeklaus S, Bohnert M, Stroud DA, Wirth C, Ellenrieder L, Thornton N, Kutik S, Wiese S, Schulze-Specking A, Zufall N, Chacinska A, Guiard B, Hunte C, Warscheid B, van der Laan M, Pfanner N*, Wiedemann N*, Becker T (2013).
  Cell 154, 596–608. (* corresponding)
• Dual Function of Sdh3 in the Respiratory Chain and TIM22 Protein Translocase of the Mitochondrial Inner Membrane.
  Gebert N, Gebert M, Oeljeklaus S, Malsburg von der K, Stroud DA, Kulawiak B, Wirth C, Zahedi RP, Dolezal P, Wiese S, Simon O, Schulze-Specking A, Truscott KN, Sickmann A, Rehling P, Guiard B, Hunte C, Warscheid B, van der Laan M, Pfanner N, Wiedemann N (2011).
  Mol Cell 44, 811–818.
• Dissecting membrane insertion of mitochondrial β-barrel proteins.
  Kutik S, Stojanovski D, Becker L, Becker T, Meinecke M, Krüger V, Prinz C, Meisinger C, Guiard B, Wagner R, Pfanner N and Wiedemann N (2008).
  Cell 132, 1011-1024.
• Machinery for protein sorting and assembly in the mitochondrial outer membrane.
  Wiedemann N, Kozjak V, Chacinska A, Schönfisch B, Rospert S, Ryan MT, Pfanner N and Meisinger C (2003).
  Nature 424, 565-571.