Molecular and Supramolecular Structure of the Mitochondrial Oxidative Phosphorylation System: Implications for Pathology
Nesci, Salvatore, Trombetti, Fabiana, Pagliarani, Alessandra, Ventrella, Vittoria, Algieri, Cristina, Tioli, Gaia, Lenaz, Giorgio
Published in Life (Basel, Switzerland) (15.03.2021)
Published in Life (Basel, Switzerland) (15.03.2021)
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Journal Article
Complex I function in mitochondrial supercomplexes
Lenaz, Giorgio, Tioli, Gaia, Falasca, Anna Ida, Genova, Maria Luisa
Published in Biochimica et biophysica acta (01.07.2016)
Published in Biochimica et biophysica acta (01.07.2016)
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Journal Article
Biochemical and computational approaches to dissect the effect of MT-CYB pathogenic mutations on respiratory chain activity and assembly
Tioli, Gaia, Musiani, Francesco, Iommarini, Luisa, Porcelli, Anna Maria, Carelli, Valerio, Ghelli, Anna Maria
Published in Biochimica et biophysica acta. Bioenergetics (01.09.2024)
Published in Biochimica et biophysica acta. Bioenergetics (01.09.2024)
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Genetic variants of NQO1 affect the expression and activity of the protein, which determines the efficacy of idebenone treatment in Leber's hereditary optic neuropathy
Del Dotto, Valentina, Aleo, Serena J., Romagnoli, Martina, Fiorini, Claudio, Capirossi, Giada, Peron, Camille, Fasano, Chiara, Maresca, Alessandra, Caporali, Leonardo, Capristo, Mariantonietta, Tropeano, Concetta V., Zanna, Claudia, Porcelli, Anna M., Tioli, Gaia, Amore, Giulia, La Morgia, Chiara, Tiranti, Valeria, Carelli, Valerio, Ghelli, Anna
Published in Biochimica et biophysica acta. Bioenergetics (01.09.2024)
Published in Biochimica et biophysica acta. Bioenergetics (01.09.2024)
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Ketone bodies resume mitochondrial respiration in neuronal progenitors from iPSCs of patients with AGC1 deficiency
Lasorsa, Francesco M., Barile, Simona N., Magnifico, Maria C., Distelmaier, Felix, Poeta, Eleonora, Viggiano, Luigi, Pignataro, Antonella, Antonicelli, Marica, Palmieri, Luigi, Petralla, Sabrina, Hentschel, Julia, Porcelli, Vito, Tioli, Gaia, Pisano, Isabella, Porcelli, Anna Maria, Fiermonte, Giuseppe, Palmieri, Ferdinando, Anderson, Stewart A., Iommarini, Luisa, Wallace, Douglas C., Monti, Barbara
Published in Biochimica et biophysica acta. Bioenergetics (01.09.2024)
Published in Biochimica et biophysica acta. Bioenergetics (01.09.2024)
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Journal Article
Two separate though interconnected routes underlie NADH and succinate oxidation: kinetic evidence for different functional compartments of Coenzyme Q and/or Complex III
Tioli, Gaia, Falasca, Anna Ida, Lenaz, Giorgio, Genova, Maria Luisa
Published in Biochimica et biophysica acta. Bioenergetics (01.08.2016)
Published in Biochimica et biophysica acta. Bioenergetics (01.08.2016)
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Journal Article
New insights into idebenone therapy in relation to NQO1
La Morgia, Chiara, Aleo, Serena Jasmine, Del Dotto, Valentina, Romagnoli, Martina, Fiorini, Claudio, Capirossi, Giada, Peron, Camille, Maresca, Alessandra, Caporali, Leonardo, Capristo, Mariantonietta, Tropeano, Concetta Valentina, Zanna, Claudia, Ross‐Cisneros, Fred, Sadun, Alfredo A., Pignataro, Maria Gemma, Giordano, Carla, Fasano, Chiara, Porcelli, Anna Maria, Tioli, Gaia, Musiani, Francesco, Catania, Alessia, Lamperti, Costanza, Marzoli, Stefania Bianchi, De Negri, Annamaria, Cascavilla, Maria Lucia, Battista, Marco, Barboni, Piero, Carbonelli, Michele, Amore, Giulia, Smirnov, Dmitrii, Vasilescu, Catalina, Farzeen, Aiman, Blickhäuser, Beryll, Prokisch, Holger, Catarino, Claudia, Klopstock, Thomas, Tiranti, Valeria, Carelli, Valerio, Ghelli, Anna Maria
Published in Acta ophthalmologica (Oxford, England) (01.01.2024)
Published in Acta ophthalmologica (Oxford, England) (01.01.2024)
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Journal Article
Genetic variants affecting NQO1 protein levels impact the efficacy of idebenone treatment in Leber hereditary optic neuropathy
Aleo, Serena Jasmine, Del Dotto, Valentina, Romagnoli, Martina, Fiorini, Claudio, Capirossi, Giada, Peron, Camille, Maresca, Alessandra, Caporali, Leonardo, Capristo, Mariantonietta, Tropeano, Concetta Valentina, Zanna, Claudia, Ross-Cisneros, Fred N., Sadun, Alfredo A., Pignataro, Maria Gemma, Giordano, Carla, Fasano, Chiara, Cavaliere, Andrea, Porcelli, Anna Maria, Tioli, Gaia, Musiani, Francesco, Catania, Alessia, Lamperti, Costanza, Marzoli, Stefania Bianchi, De Negri, Annamaria, Cascavilla, Maria Lucia, Battista, Marco, Barboni, Piero, Carbonelli, Michele, Amore, Giulia, La Morgia, Chiara, Smirnov, Dmitrii, Vasilescu, Catalina, Farzeen, Aiman, Blickhaeuser, Beryll, Prokisch, Holger, Priglinger, Claudia, Livonius, Bettina, Catarino, Claudia B., Klopstock, Thomas, Tiranti, Valeria, Carelli, Valerio, Ghelli, Anna Maria
Published in Cell reports. Medicine (20.02.2024)
Published in Cell reports. Medicine (20.02.2024)
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