Article
Cell Biology
Sandra Backes, Yury S. Bykov, Tamara Flohr, Markus Raeschle, Jialin Zhou, Svenja Lenhard, Lena Kraemer, Timo Muehlhaus, Chen Bibi, Cosimo Jann, Justin D. Smith, Lars M. Steinmetz, Doron Rapaport, Zuzana Storchova, Maya Schuldiner, Felix Boos, Johannes M. Herrmann
Summary: Tom70 protein functions at the interface of cytosol and mitochondria, recruiting cytosolic chaperones to the outer mitochondrial membrane to reduce proteotoxicity of mitochondrial precursor proteins.
Article
Cell Biology
Maria Livia Sassano, Alexander R. R. van Vliet, Ellen Vervoort, Sofie Van Eygen, Chris van den Haute, Benjamin Pavie, Joris Roels, Johannes V. V. Swinnen, Marco Spinazzi, Leen Moens, Kristina Casteels, Isabelle Meyts, Paolo Pinton, Saverio Marchi, Leila Rochin, Francesca Giordano, Blanca Felipe-Abrio, Patrizia Agostinis
Summary: ER-mitochondria appositions play a critical role in the transfer of ions and phospholipids between these organelles and impact mitochondrial bioenergetics. However, the molecular mechanisms involved in this process are not well understood. This study reveals that PERK acts as an adaptor to recruit the lipid transfer protein E-Syt1 at ER-mitochondria contact sites, enabling lipid transport and maintaining mitochondrial homeostasis. These findings provide insights into the molecular components and functions of the lipid trafficking machinery at ER-mitochondria contact sites.
JOURNAL OF CELL BIOLOGY
(2023)
Review
Biochemistry & Molecular Biology
Mary Oluwadamilola Haastrup, Kunwar Somesh Vikramdeo, Seema Singh, Ajay Pratap Singh, Santanu Dasgupta
Summary: Mitochondria are vital organelles involved in various cellular functions. They have their own genome and machinery for synthesizing essential proteins, while the majority of the proteins are produced in the cytosol and imported into mitochondria. Proper functioning of the mitochondrial protein import system is crucial for mitochondrial and cellular homeostasis. Impaired protein import can lead to proteotoxic stress and altered mitochondrial unfolded protein response, which are associated with various diseases. This review highlights the mechanisms of nuclear-encoded mitochondrial protein import, the consequences of defective import, and the pathological conditions associated with altered unfolded protein response.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Cell Biology
Jialin Zhou, Martin Jung, Kai S. Dimmer, Doron Rapaport
Summary: In this study, we investigated the biogenesis of multi-span proteins in the mitochondrial outer membrane. Our findings suggest that the targeting specificity and efficiency of these proteins are influenced by their length, as well as the behavior of the membrane. Additionally, cytosolic proteins do not significantly contribute to the biogenesis process.
JOURNAL OF CELL BIOLOGY
(2022)
Article
Biochemistry & Molecular Biology
Sotirios D. Zervopoulos, Aristeidis E. Boukouris, Bruno Saleme, Alois Haromy, Saymon Tejay, Gopinath Sutendra, Evangelos D. Michelakis
Summary: The study reveals a noncanonical pathway for nuclear import of PDC and the role of mitochondria clustering around the nucleus in this process, providing new insights into mitochondria-to-nucleus communication and nuclear metabolism.
Article
Multidisciplinary Sciences
Hironori Takeda, Akihisa Tsutsumi, Tomohiro Nishizawa, Caroline Lindau, Jon Busto, Lena-Sophie Wenz, Lars Ellenrieder, Kenichiro Imai, Sebastian P. Straub, Waltraut Mossmann, Jian Qiu, Yu Yamamori, Kentaro Tomii, Junko Suzuki, Takeshi Murata, Satoshi Ogasawara, Osamu Nureki, Thomas Becker, Nikolaus Pfanner, Nils Wiedemann, Masahide Kikkawa, Toshiya Endo
Summary: The study reveals that beta-barrel proteins in the mitochondrial outer membrane are inserted by the multisubunit mitochondrial SAM complex. The structure of the SAM complex, determined using cryo-electron microscopy, shows the involvement of proteins such as Sam50, Sam35, Sam37, and Mdm10 in the process.
Article
Multidisciplinary Sciences
Chun-Shik Shin, Shuxia Meng, Spiros D. Garbis, Annie Moradian, Robert W. Taylor, Michael J. Sweredoski, Brett Lomenick, David C. Chan
Summary: This study reveals that the mitochondrial protease LONP1 is essential for the mtHSP70 chaperone system, regardless of its protease activity. LONP1 plays a critical role in maintaining the solubility of DNAJA3 and mtHSP70 in the mitochondria, showing intrinsic chaperone-like activity and collaborating with mtHSP70 to stabilize protein folding intermediates.
NATURE COMMUNICATIONS
(2021)
Article
Multidisciplinary Sciences
Samuel Rout, Silke Oeljeklaus, Abhijith Makki, Jan Tachezy, Bettina Warscheid, Andre Schneider
Summary: The study reveals that the mitochondrial import receptors ATOM46 and ATOM69 of Trypanosoma brucei have different substrate preferences, with ATOM46 favoring hydrophilic proteins and ATOM69 preferring hydrophobic substrates. While ATOM46 mainly uses electrostatic interactions for substrate binding, ATOM69 primarily uses hydrophobic interactions.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2021)
Article
Cell Biology
Marcel Morgenstern, Christian D. Peikert, Philipp Luebbert, Ida Suppanz, Cinzia Klemm, Oliver Alka, Conny Steiert, Nataliia Naumenko, Alexander Schendzielorz, Laura Melchionda, Wignand W. D. Muehlhaeuser, Bettina Knapp, Jakob D. Busch, Sebastian B. Stiller, Stefan Dannenmaier, Caroline Lindau, Mariya Licheva, Christopher Eickhorst, Riccardo Galbusera, Ralf M. Zerbes, Michael T. Ryan, Claudine Kraft, Vera Kozjak-Pavlovic, Friedel Drepper, Sven Dennerlein, Silke Oeljeklaus, Nikolaus Pfanner, Nils Wiedemann, Bettina Warscheid
Summary: Researchers have classified and identified human mitochondrial proteins, establishing a high-confidence mitochondrial proteome containing over 1,100 proteins, which account for 7% of cellular proteins and are linked to various diseases.
Article
Biology
Robert Coukos, David Yao, Mateo Sanchez, Eric T. Strand, Meagan E. Olive, Namrata D. Udeshi, Jonathan S. Weissman, Steven A. Carr, Michael C. Bassik, Alice Y. Ting
Summary: The study demonstrates that gene perturbation technology combined with HiLITR tool can be used to explore protein trafficking processes by converting protein colocalization into a transcription factor driving expression of selected genes. By using HiLITR with FACS screening, genes that influence the trafficking of mitochondrial and ER tail-anchored proteins were identified, revealing the roles of SUMO E1 component SAE1 and EMC10 in mitochondrial and ER systems.
Article
Biochemistry & Molecular Biology
Naintara Jain, Ridhima Gomkale, Olaf Bernhard, Peter Rehling, Luis Daniel Cruz-Zaragoza
Summary: Mitochondria play a crucial role in cellular energy production and metabolism, and errors in mitochondrial protein import can lead to metabolic disorders. This study establishes a fluorescence-based import assay to analyze protein import into mitochondria, offering the advantage of quantifying import with high sensitivity. The assay can be adapted to a screening-compatible format and used to monitor the assembly of the F1F0 ATP synthase in purified mitochondria.
Article
Multidisciplinary Sciences
F. X. Reymond Sutandy, Ines Goessner, Georg Tascher, Christian Muench
Summary: This study demonstrates that cytosol-mitochondrial reactive oxygen species (mtROS) and accumulation of mitochondrial protein precursors in the cytosol (c-mtProt) play a critical role in initiating the mitochondrial unfolded protein response (UPRmt). The findings reveal a link between mitochondrial and cytosolic proteostasis and provide molecular insight into UPRmt signaling in human cells.
Article
Chemistry, Medicinal
Edita Polachova, Kathrin Bach, Elena Heuten, Stancho Stanchev, Anezka Ticha, Philipp Lampe, Pavel Majer, Thomas Langer, Marius K. Lemberg, Kvido Strisovsky
Summary: The study reveals that the inhibition of PARL can activate the PINK1/Parkin pathway, promoting mitophagy in the context of Parkinson's disease. This highlights the potential of ketoamide inhibitors for cell biological studies of rhomboid proteases.
JOURNAL OF MEDICINAL CHEMISTRY
(2023)
Article
Biochemistry & Molecular Biology
Katharina Weinhaupl, Yong Wang, Audrey Hessel, Martha Brennich, Kresten Lindorff-Larsen, Paul Schanda
Summary: The formation of TIM9.10 and TIM9.10.12 is related to a dynamic equilibrium among protein subunits and their large conformational dynamics, providing a new understanding of how mitochondria control the level of inter-membrane space chaperones.
Article
Biology
Barbara Uszczynska-Ratajczak, Sreedevi Sugunan, Monika Kwiatkowska, Maciej Migdal, Silvia Carbonell-Sala, Anna Sokol, Cecilia L. Winata, Agnieszka Chacinska
Summary: Most mitochondrial proteins are encoded by nuclear genes, synthesized in the cytosol, and targeted into the organelle. The presence of nuclear-encoded mRNA5 in the mitochondrial fraction confirms that large proteins with specific properties, like transmembrane domains, are predominantly encoded. Under proteostatic stress conditions, the population of transcripts on the mitochondrial surface is further restricted, allowing only the largest and most evolutionarily conserved proteins to be synthesized there.
LIFE SCIENCE ALLIANCE
(2022)
Article
Cell Biology
Vivien Krueger, Thomas Becker, Lars Becker, Malayko Montilla-Martinez, Lars Ellenrieder, F. -Nora Voegtle, Helmut E. Meyer, Michael T. Ryan, Nils Wiedemann, Bettina Warscheid, Nikolaus Pfanner, Richard Wagner, Chris Meisinger
JOURNAL OF CELL BIOLOGY
(2017)
Article
Cell Biology
Ayelen Gonzalez Montoro, Kathrin Auffarth, Carina Hoenscher, Maria Bohnert, Thomas Becker, Bettina Warscheid, Fulvio Reggiori, Martin van der Laan, Florian Froehlich, Christian Ungermann
DEVELOPMENTAL CELL
(2018)
Article
Biochemistry & Molecular Biology
Heike Rampelt, Florian Wollweber, Carolin Gerke, Rinse de Boer, Ida J. van der Klei, Maria Bohnert, Nikolaus Pfanner, Martin van der Laan
JOURNAL OF MOLECULAR BIOLOGY
(2018)
Article
Endocrinology & Metabolism
Daqiang Pan, Caroline Lindau, Simon Lagies, Nils Wiedemann, Bernd Kammerer
Article
Cell Biology
Lena Boettinger, Christoph U. Martensson, Jiyao Song, Nicole Zufall, Nils Wiedemann, Thomas Becker
MOLECULAR BIOLOGY OF THE CELL
(2018)
Article
Multidisciplinary Sciences
Alexandra I. C. Hohr, Caroline Lindau, Christophe Wirth, Jian Qiu, David A. Stroud, Stephan Kutik, Bernard Guiard, Carola Hunte, Thomas Becker, Nikolaus Pfanner, Nils Wiedemann
Review
Cell Biology
Stefan Schorr, Martin van der Laan
SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY
(2018)
Article
Chemistry, Analytical
Stefan Dannenmaier, Sebastian B. Stiller, Marcel Morgenstern, Philipp Luebbert, Silke Oeljeklaus, Nils Wiedemann, Bettina Warscheid
ANALYTICAL CHEMISTRY
(2018)
Article
Biochemistry & Molecular Biology
Alexis Hofherr, Claudia Seger, Fiona Fitzpatrick, Tilman Busch, Elisabeth Michel, Jingting Luan, Lea Osterried, Frieder Linden, Albrecht Kramer-Zucker, Barbara Wakimoto, Conny Schuetze, Nils Wiedemann, Anna Artati, Jerzy Adamski, Gerd Walz, Edmund R. S. Kunji, Craig Montell, Terry Watnick, Michael Koettgen
Article
Biochemistry & Molecular Biology
Katharina Weinhaupl, Caroline Lindau, Audrey Hessel, Yong Wang, Conny Schuetze, Tobias Jores, Laura Melchionda, Birgit Schoenfisch, Hubert Kalbacher, Beate Bersch, Doron Rapaport, Martha Brennich, Kresten Lindorff-Larsen, Nils Wiedemann, Paul Schanda
Review
Cell Biology
Nikolaus Pfanner, Bettina Warscheid, Nils Wiedemann
NATURE REVIEWS MOLECULAR CELL BIOLOGY
(2019)
Article
Multidisciplinary Sciences
Yuhei Araiso, Akihisa Tsutsumi, Jian Qiu, Kenichiro Imai, Takuya Shiota, Jiyao Song, Caroline Lindau, Lena-Sophie Wenz, Haruka Sakaue, Kaori Yunoki, Shin Kawano, Junko Suzuki, Marilena Wischnewski, Conny Schutze, Hirotaka Ariyama, Toshio Ando, Thomas Becker, Trevor Lithgow, Nils Wiedemann, Nikolaus Pfanner, Masahide Kikkawa, Toshiya Endo
Article
Biochemistry & Molecular Biology
Daqiang Pan, Nils Wiedemann, Bernd Kammerer
Article
Biology
Heike Rampelt, Iva Sucec, Beate Bersch, Patrick Horten, Inge Perschil, Jean-Claude Martinou, Martin van der Laan, Nils Wiedemann, Paul Schanda, Nikolaus Pfanner
Article
Biochemistry & Molecular Biology
Irina Kurokin, Anna Andrea Lauer, Daniel Janitschke, Jakob Winkler, Elena Leoni Theiss, Lea Victoria Griebsch, Sabrina Melanie Pilz, Veronika Matschke, Martin van der Laan, Heike Sabine Grimm, Tobias Hartmann, Marcus Otto Walter Grimm
Summary: The study investigated potential alterations in the lipid composition of mitochondria in a cellular AD model, revealing specific lipid changes in mitochondria that cannot be predicted from total cell analysis. The observed lipid alterations are accompanied by changes in the carnitine carrier system, suggesting altered mitochondrial functionality, which may be related to differences in APP processing.
