Protocol to study human mitochondrial ribosome using quantitative density gradient analysis by mass spectrometry and complexome profiling analysis by Petra Páleníková, Michal Minczuk, Pedro Rebelo-Guiomar

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Dealing with an Unconventional Genetic Code in  Mitochondria: The Biogenesis and Pathogenic  Defects of the 5‐Formylcytosine Modification in  Mitochondrial tRNAMet... by Lindsey Van Haute, Christopher A. Powell, Michal Minczuk

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The catalytic activity of methyltransferase METTL15 is dispensable for its role in mitochondrial ribosome biogenesis by Christian D. Mutti, Lindsey Van Haute, Michal Minczuk

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Compact zinc finger base editors that edit mitochondrial or nuclear DNA in vitro and in vivo by Julian C. W. Willis, Pedro Silva-Pinheiro, Lily Widdup, Michal Minczuk, David R. Liu

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Energetic costs of cellular and therapeutic control of stochastic mitochondrial DNA populations. by Hanne Hoitzing, Payam A Gammage, Lindsey Van Haute, Michal Minczuk, Iain G Johnston, Nick S Jones

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In vivo mitochondrial base editing via adeno-associated viral delivery to mouse post-mitotic tissue by Pedro Silva-Pinheiro, Pavel A. Nash, Lindsey Van Haute, Christian D. Mutti, Keira Turner, Michal Minczuk

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The FASTK family proteins fine-tune mitochondrial RNA processing by Akira Ohkubo, Lindsey Van Haute, Danielle L. Rudler, Maike Stentenbach, Florian A. Steiner, Oliver Rackham, Michal Minczuk, Aleksandra Filipovska, Jean-Claude Martinou

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The FASTK family proteins fine-tune mitochondrial RNA processing. by Akira Ohkubo, Lindsey Van Haute, Danielle L Rudler, Maike Stentenbach, Florian A Steiner, Oliver Rackham, Michal Minczuk, Aleksandra Filipovska, Jean-Claude Martinou

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Maturation of selected human mitochondrial tRNAs requires deadenylation by Sarah F Pearce, Joanna Rorbach, Lindsey Van Haute, Aaron R D’Souza, Pedro Rebelo-Guiomar, Christopher A Powell, Ian Brierley, Andrew E Firth, Michal Minczuk

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Amino acid starvation has opposite effects on mitochondrial and cytosolic protein synthesis. by Mark A Johnson, Sara Vidoni, Romina Durigon, Sarah F Pearce, Joanna Rorbach, Jiuya He, Gloria Brea-Calvo, Michal Minczuk, Aurelio Reyes, Ian J Holt, Antonella Spinazzola

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Linear mitochondrial DNA is rapidly degraded by components of the replication machinery by Viktoriya Peeva, Daniel Blei, Genevieve Trombly, Sarah Corsi, Maciej J. Szukszto, Pedro Rebelo-Guiomar, Payam A. Gammage, Alexei P. Kudin, Christian Becker, Janine Altmüller, Michal Minczuk, Gábor Zsurka, Wolfram S. Kunz

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Severe neonatal onset neuroregression with paroxysmal dystonia and apnoea: Expanding the phenotypic and genotypic spectrum of CARS2‐related mitochondrial disease by Jessie Poquérusse, Melinda Nolan, David R. Thorburn, Johan L. K. Van Hove, Marisa W. Friederich, Donald R. Love, Juliet Taylor, Christopher A. Powell, Michal Minczuk, Russell G. Snell, Klaus Lehnert, Emma Glamuzina, Jessie C. Jacobsen

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Disruption of the TCA cycle reveals an ATF4-dependent integration of redox and amino acid metabolism by Dylan Gerard Ryan, Ming Yang, Hiran A Prag, Giovanny Rodriguez Blanco, Efterpi Nikitopoulou, Marc Segarra-Mondejar, Christopher A Powell, Tim Young, Nils Burger, Jan Lj Miljkovic, Michal Minczuk, Michael P Murphy, Alex von Kriegsheim, Christian Frezza

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A late-stage assembly checkpoint of the human mitochondrial ribosome large subunit by Pedro Rebelo-Guiomar, Simone Pellegrino, Kyle C. Dent, Aldema Sas-Chen, Leonor Miller-Fleming, Caterina Garone, Lindsey Van Haute, Jack F. Rogan, Adam Dinan, Andrew E. Firth, Byron Andrews, Alexander J. Whitworth, Schraga Schwartz, Alan J. Warren, Michal Minczuk

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A homozygous MRPL24 mutation causes a complex movement disorder and affects the mitoribosome assembly by Michela Di Nottia, Maria Marchese, Daniela Verrigni, Christian Daniel Mutti, Alessandra Torraco, Romina Oliva, Erika Fernandez-Vizarra, Federica Morani, Giulia Trani, Teresa Rizza, Daniele Ghezzi, Anna Ardissone, Claudia Nesti, Gessica Vasco, Massimo Zeviani, Michal Minczuk, Enrico Bertini, Filippo Maria Santorelli, Rosalba Carrozzo

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Deficient methylation and formylation of mt-tRNAMet wobble cytosine in a patient carrying mutations in NSUN3 by Lindsey Van Haute, Sabine Dietmann, Laura Kremer, Shobbir Hussain, Sarah F. Pearce, Christopher A. Powell, Joanna Rorbach, Rebecca Lantaff, Sandra Blanco, Sascha Sauer, Urania Kotzaeridou, Georg F. Hoffmann, Yasin Memari, Anja Kolb-Kokocinski, Richard Durbin, Johannes A. Mayr, Michaela Frye, Holger Prokisch, Michal Minczuk

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Mitochondrially-targeted APOBEC1 is a potent mtDNA mutator affecting mitochondrial function and organismal fitness in Drosophila by Simonetta Andreazza, Colby L. Samstag, Alvaro Sanchez-Martinez, Erika Fernandez-Vizarra, Aurora Gomez-Duran, Juliette J. Lee, Roberta Tufi, Michael J. Hipp, Elizabeth K. Schmidt, Thomas J. Nicholls, Payam A. Gammage, Patrick F. Chinnery, Michal Minczuk, Leo J. Pallanck, Scott R. Kennedy, Alexander J. Whitworth

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GTPBP8 plays a role in mitoribosome formation in human mitochondria by Miriam Cipullo, Genís Valentín Gesé, Shreekara Gopalakrishna, Annika Krueger, Vivian Lobo, Maria A. Pirozhkova, James Marks, Petra Páleníková, Dmitrii Shiriaev, Yong Liu, Jelena Misic, Yu Cai, Minh Duc Nguyen, Abubakar Abdelbagi, Xinping Li, Michal Minczuk, Markus Hafner, Daniel Benhalevy, Aishe A. Sarshad, Ilian Atanassov, B. Martin Hällberg, Joanna Rorbach

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Cardiac mitochondrial function depends on BUD23 mediated ribosome programming by Matthew Baxter, Maria Voronkov, Toryn Poolman, Gina Galli, Christian Pinali, Laurence Goosey, Abigail Knight, Karolina Krakowiak, Robert Maidstone, Mudassar Iqbal, Min Zi, Sukhpal Prehar, Elizabeth J Cartwright, Julie Gibbs, Laura C Matthews, Antony D Adamson, Neil E Humphreys, Pedro Rebelo-Guiomar, Michal Minczuk, David A Bechtold, Andrew Loudon, David Ray

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TEFM variants impair mitochondrial transcription causing childhood-onset neurological disease by Lindsey Van Haute, Emily O’Connor, Héctor Díaz-Maldonado, Benjamin Munro, Kiran Polavarapu, Daniella H. Hock, Gautham Arunachal, Alkyoni Athanasiou-Fragkouli, Mainak Bardhan, Magalie Barth, Dominique Bonneau, Nicola Brunetti-Pierri, Gerarda Cappuccio, Nikeisha J. Caruana, Natalia Dominik, Himanshu Goel, Guy Helman, Henry Houlden, Guy Lenaers, Karine Mention, David Murphy, Bevinahalli Nandeesh, Catarina Olimpio, Christopher A. Powell, Veeramani Preethish-Kumar, Vincent Procaccio, Rocio Rius, Pedro Rebelo-Guiomar, Cas Simons, Seena Vengalil, Maha S. Zaki, Alban Ziegler, David R. Thorburn, David A. Stroud, Reza Maroofian, John Christodoulou, Claes Gustafsson, Atchayaram Nalini, Hanns Lochmüller, Michal Minczuk, Rita Horvath

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