Neurodegeneration with Brain Iron Accumulation Disorders: Valuable Models Aimed at Understanding the Pathogenesis of Iron Deposition by Sonia Levi, Valeria Tiranti

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Ethylmalonic encephalopathy: Clinical course and therapy response in an uncommon mild case with a severe ETHE1 mutation by Melike Ersoy, Valeria Tiranti, Massimo Zeviani

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Inherited Disorders of Coenzyme A Biosynthesis: Models, Mechanisms, and Treatments by Chiara Cavestro, Daria Diodato, Valeria Tiranti, Ivano Di Meo

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Mitochondrial Transplantation in Mitochondrial Medicine: Current Challenges and Future Perspectives by Marco D’Amato, Francesca Morra, Ivano Di Meo, Valeria Tiranti

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Therapeutic Approaches to Treat Mitochondrial Diseases: “One-Size-Fits-All” and “Precision Medicine” Strategies by Emanuela Bottani, Costanza Lamperti, Alessandro Prigione, Valeria Tiranti, Nicola Persico, Dario Brunetti

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Gene Therapy for Mitochondrial Diseases: Current Status and Future Perspective by Alessia Di Donfrancesco, Giulia Massaro, Ivano Di Meo, Valeria Tiranti, Emanuela Bottani, Dario Brunetti

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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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Modeling human Coenzyme A synthase mutation in yeast reveals altered mitochondrial function, lipid content and iron metabolism by Camilla Ceccatelli Berti, Cristina Dallabona, Mirca Lazzaretti, Sabrina Dusi, Elena Tosi, Valeria Tiranti, Paola Goffrini

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Mitochondrial iron and energetic dysfunction distinguish fibroblasts and induced neurons from pantothenate kinase-associated neurodegeneration patients by Paolo Santambrogio, Sabrina Dusi, Michela Guaraldo, Luisa Ida Rotundo, Vania Broccoli, Barbara Garavaglia, Valeria Tiranti, Sonia Levi

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Generation of two human iPSC lines, FINCBi002-A and FINCBi003-A, carrying heteroplasmic macrodeletion of mitochondrial DNA causing Pearson’s syndrome by Camille Peron, Roberta Mauceri, Angelo Iannielli, Andrea Cavaliere, Andrea Legati, Ambra Rizzo, Francesca L. Sciacca, Vania Broccoli, Valeria Tiranti

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PKAN hiPS-Derived Astrocytes Show Impairment of Endosomal Trafficking: A Potential Mechanism Underlying Iron Accumulation by Maddalena Ripamonti, Maddalena Ripamonti, Paolo Santambrogio, Gabriella Racchetti, Anna Cozzi, Ivano Di Meo, Valeria Tiranti, Sonia Levi, Sonia Levi

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Generation of two human iPSC lines, HMGUi004-A and FINCBi004-A, from fibroblasts of MPAN patients carrying pathogenic recessive mutations in the gene C19orf12 by Enrica Zanuttigh, Ejona Rusha, Camille Peron, Dario Brunetti, Giovanna Zorzi, Anna Pertek, Polyxeni Nteli, Juliane Winkelmann, Valeria Tiranti, Arcangela Iuso

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Massive iron accumulation in PKAN-derived neurons and astrocytes: light on the human pathological phenotype by Paolo Santambrogio, Maddalena Ripamonti, Anna Cozzi, Marzia Raimondi, Chiara Cavestro, Ivano Di Meo, Alicia Rubio, Stefano Taverna, Valeria Tiranti, Sonia Levi

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PPAR Gamma Agonist Leriglitazone Recovers Alterations Due to Pank2-Deficiency in hiPS-Derived Astrocytes by Paolo Santambrogio, Anna Cozzi, Ivano Di Meo, Chiara Cavestro, Cristina Vergara, Laura Rodríguez-Pascau, Marc Martinell, Pilar Pizcueta, Valeria Tiranti, Sonia Levi

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Generation of a human iPSC line, FINCBi001-A, carrying a homoplasmic m.G3460A mutation in MT-ND1 associated with Leber’s Hereditary optic Neuropathy (LHON) by Camille Peron, Roberta Mauceri, Tommaso Cabassi, Alice Segnali, Alessandra Maresca, Angelo Iannielli, Ambra Rizzo, Francesca L. Sciacca, Vania Broccoli, Valerio Carelli, Valeria Tiranti

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Neuronal Ablation of CoA Synthase Causes Motor Deficits, Iron Dyshomeostasis, and Mitochondrial Dysfunctions in a CoPAN Mouse Model by Ivano Di Meo, Chiara Cavestro, Silvia Pedretti, Tingting Fu, Simona Ligorio, Antonello Manocchio, Lucrezia Lavermicocca, Paolo Santambrogio, Maddalena Ripamonti, Sonia Levi, Sophie Ayciriex, Nico Mitro, Valeria Tiranti

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MR-1S Interacts with PET100 and PET117 in Module-Based Assembly of Human Cytochrome c Oxidase by Sara Vidoni, Michael E. Harbour, Sergio Guerrero-Castillo, Alba Signes, Shujing Ding, Ian M. Fearnley, Robert W. Taylor, Valeria Tiranti, Susanne Arnold, Erika Fernandez-Vizarra, Massimo Zeviani

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Generation of iPSCs from identical twin, one affected by LHON and one unaffected, both carrying a combination of two mitochondrial variants: m.14484 T>C and m.10680G>A by Camille Peron, Andrea Cavaliere, Chiara Fasano, Angelo Iannielli, Manuela Spagnolo, Andrea Legati, Maria Nicol Colombo, Ambra Rizzo, Francesca L. Sciacca, Valerio Carelli, Vania Broccoli, Costanza Lamperti, Valeria Tiranti

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Arabidopsis thaliana alternative dehydrogenases: a potential therapy for mitochondrial complex I deficiency? Perspectives and pitfalls by Alessia Catania, Arcangela Iuso, Juliette Bouchereau, Laura S. Kremer, Marina Paviolo, Caterina Terrile, Paule Bénit, Allan G. Rasmusson, Thomas Schwarzmayr, Valeria Tiranti, Pierre Rustin, Malgorzata Rak, Holger Prokisch, Manuel Schiff

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Harmful Iron-Calcium Relationship in Pantothenate kinase Associated Neurodegeneration by Paolo Santambrogio, Maddalena Ripamonti, Chiara Paolizzi, Celeste Panteghini, Miryam Carecchio, Luisa Chiapparini, Marzia Raimondi, Alicia Rubio, Ivano Di Meo, Anna Cozzi, Stefano Taverna, Giuseppe De Palma, Valeria Tiranti, Sonia Levi

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