Mutations in TIMM50 compromise cell survival in OxPhos‐dependent metabolic conditions by Aurelio Reyes, Laura Melchionda, Alberto Burlina, Alan J Robinson, Daniele Ghezzi, Massimo Zeviani

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Effective AAV‐mediated gene therapy in a mouse model of ethylmalonic encephalopathy by Ivano Di Meo, Alberto Auricchio, Costanza Lamperti, Alberto Burlina, Carlo Viscomi, Massimo Zeviani

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How do human cells react to the absence of mitochondrial DNA? by Rossana Mineri, Norman Pavelka, Erika Fernandez-Vizarra, Paola Ricciardi-Castagnoli, Massimo Zeviani, Valeria Tiranti

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Rapamycin rescues mitochondrial myopathy via coordinated activation of autophagy and lysosomal biogenesis by Gabriele Civiletto, Sukru Anil Dogan, Raffaele Cerutti, Gigliola Fagiolari, Maurizio Moggio, Costanza Lamperti, Cristiane Benincá, Carlo Viscomi, Massimo Zeviani

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A nonsense mutation of human XRCC4 is associated with adult‐onset progressive encephalocardiomyopathy by Leonardo Bee, Alessia Nasca, Alice Zanolini, Filippo Cendron, Pio d'Adamo, Rodolfo Costa, Costanza Lamperti, Lucia Celotti, Daniele Ghezzi, Massimo Zeviani

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Inhibition of proteasome rescues a pathogenic variant of respiratory chain assembly factor COA7 by Karthik Mohanraj, Michal Wasilewski, Cristiane Benincá, Dominik Cysewski, Jaroslaw Poznanski, Paulina Sakowska, Zaneta Bugajska, Markus Deckers, Sven Dennerlein, Erika Fernandez‐Vizarra, Peter Rehling, Michal Dadlez, Massimo Zeviani, Agnieszka Chacinska

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APOPT1/COA8 assists COX assembly and is oppositely regulated by UPS and ROS by Alba Signes, Raffaele Cerutti, Anna S Dickson, Cristiane Benincá, Elizabeth C Hinchy, Daniele Ghezzi, Rosalba Carrozzo, Enrico Bertini, Michael P Murphy, James A Nathan, Carlo Viscomi, Erika Fernandez‐Vizarra, Massimo Zeviani

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Modelling POLG mutations in mice unravels a critical role of POLγΒ in regulating phenotypic severity by Samantha Corrà, Alessandro Zuppardo, Sebastian Valenzuela, Louise Jenninger, Raffaele Cerutti, Sirelin Sillamaa, Emily Hoberg, Katarina A. S. Johansson, Urska Rovsnik, Sara Volta, Pedro Silva-Pinheiro, Hannah Davis, Aleksandra Trifunovic, Michal Minczuk, Claes M. Gustafsson, Anu Suomalainen, Massimo Zeviani, Bertil Macao, Xuefeng Zhu, Maria Falkenberg, Carlo Viscomi

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Defective PITRM1 mitochondrial peptidase is associated with Aβ amyloidotic neurodegeneration by Dario Brunetti, Janniche Torsvik, Cristina Dallabona, Pedro Teixeira, Pawel Sztromwasser, Erika Fernandez‐Vizarra, Raffaele Cerutti, Aurelio Reyes, Carmela Preziuso, Giulia D'Amati, Enrico Baruffini, Paola Goffrini, Carlo Viscomi, Ileana Ferrero, Helge Boman, Wenche Telstad, Stefan Johansson, Elzbieta Glaser, Per M Knappskog, Massimo Zeviani, Laurence A Bindoff

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miR‐181a/b downregulation exerts a protective action on mitochondrial disease models by Alessia Indrieri, Sabrina Carrella, Alessia Romano, Alessandra Spaziano, Elena Marrocco, Erika Fernandez‐Vizarra, Sara Barbato, Mariateresa Pizzo, Yulia Ezhova, Francesca M Golia, Ludovica Ciampi, Roberta Tammaro, Jorge Henao‐Mejia, Adam Williams, Richard A Flavell, Elvira De Leonibus, Massimo Zeviani, Enrico M Surace, Sandro Banfi, Brunella Franco

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Rare primary mitochondrial DNA mutations and probable synergistic variants in Leber's hereditary optic neuropathy. by Alessandro Achilli, Luisa Iommarini, Anna Olivieri, Maria Pala, Baharak Hooshiar Kashani, Pascal Reynier, Chiara La Morgia, Maria Lucia Valentino, Rocco Liguori, Fabio Pizza, Piero Barboni, Federico Sadun, Anna Maria De Negri, Massimo Zeviani, Helene Dollfus, Antoine Moulignier, Ghislaine Ducos, Christophe Orssaud, Dominique Bonneau, Vincent Procaccio, Beate Leo-Kottler, Sascha Fauser, Bernd Wissinger, Patrizia Amati-Bonneau, Antonio Torroni, Valerio Carelli

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Neural stem cells traffic functional mitochondria via extracellular vesicles. by Luca Peruzzotti-Jametti, Joshua D Bernstock, Cory M Willis, Giulia Manferrari, Rebecca Rogall, Erika Fernandez-Vizarra, James C Williamson, Alice Braga, Aletta van den Bosch, Tommaso Leonardi, Grzegorz Krzak, Ágnes Kittel, Cristiane Benincá, Nunzio Vicario, Sisareuth Tan, Carlos Bastos, Iacopo Bicci, Nunzio Iraci, Jayden A Smith, Ben Peacock, Karin H Muller, Paul J Lehner, Edit Iren Buzas, Nuno Faria, Massimo Zeviani, Christian Frezza, Alain Brisson, Nicholas J Matheson, Carlo Viscomi, Stefano Pluchino

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