Oxidative stress, mitochondrial and proteostasis malfunction in adrenoleukodystrophy: A paradigm for axonal degeneration von Fourcade, Stéphane, Ferrer, Isidre, Pujol, Aurora

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Sphingolipid desaturase DEGS1 is essential for mitochondria-associated membrane integrity von Planas-Serra, Laura, Launay, Nathalie, Goicoechea, Leire, Heron, Bénédicte, Jou, Cristina, Juliá-Palacios, Natalia, Ruiz, Montserrat, Fourcade, Stéphane, Casasnovas, Carlos, De La Torre, Carolina, Gelot, Antoinette, Marsal, Maria, Loza-Alvarez, Pablo, García-Cazorla, Àngels, Fatemi, Ali, Ferrer, Isidre, Portero-Otin, Manel, Area-Gómez, Estela, Pujol, Aurora

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Antioxidants halt axonal degeneration in a mouse model of X-adrenoleukodystrophy von López-Erauskin, Jone, Fourcade, Stéphane, Galino, Jorge, Ruiz, Montserrat, Schlüter, Agatha, Naudi, Alba, Jove, Mariona, Portero-Otin, Manuel, Pamplona, Reinald, Ferrer, Isidre, Pujol, Aurora

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Biomarker Identification, Safety, and Efficacy of High-Dose Antioxidants for Adrenomyeloneuropathy: a Phase II Pilot Study von Casasnovas, Carlos, Ruiz, Montserrat, Schlüter, Agatha, Naudí, Alba, Fourcade, Stéphane, Veciana, Misericordia, Castañer, Sara, Albertí, Antonia, Bargalló, Nuria, Johnson, Maria, Raymond, Gerald V., Fatemi, Ali, Moser, Ann B., Villarroya, Francesc, Portero-Otín, Manuel, Artuch, Rafael, Pamplona, Reinald, Pujol, Aurora

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Imbalanced mitochondrial dynamics contributes to the pathogenesis of X-linked adrenoleukodystrophy von Launay, Nathalie, Lopez-Erauskin, Jone, Bianchi, Patrizia, Guha, Sanjib, Parameswaran, Janani, Coppa, Andrea, Torreni, Lorenzo, Schlüter, Agatha, Fourcade, Stéphane, Paredes-Fuentes, Abraham J, Artuch, Rafael, Casasnovas, Carlos, Ruiz, Montserrat, Pujol, Aurora

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Mitochondrial dysfunction and oxidative damage cooperatively fuel axonal degeneration in X-linked adrenoleukodystrophy von Fourcade, Stéphane, López-Erauskin, Jone, Ruiz, Montserrat, Ferrer, Isidre, Pujol, Aurora

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Epigenomic signature of adrenoleukodystrophy predicts compromised oligodendrocyte differentiation von Schlüter, Agatha, Sandoval, Juan, Fourcade, Stéphane, Díaz‐Lagares, Angel, Ruiz, Montserrat, Casaccia, Patrizia, Esteller, Manel, Pujol, Aurora

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The evolutionary origin of peroxisomes: an ER-peroxisome connection von Schlüter, Agatha, Fourcade, Stéphane, Ripp, Raymond, Mandel, Jean Louis, Poch, Olivier, Pujol, Aurora

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Aberrant regulation of the GSK‐3β/NRF2 axis unveils a novel therapy for adrenoleukodystrophy von Ranea‐Robles, Pablo, Launay, Nathalie, Ruiz, Montserrat, Calingasan, Noel Ylagan, Dumont, Magali, Naudí, Alba, Portero‐Otín, Manuel, Pamplona, Reinald, Ferrer, Isidre, Beal, M Flint, Fourcade, Stéphane, Pujol, Aurora

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The peroxisomal fatty acid transporter ABCD1/PMP-4 is required in the C. elegans hypodermis for axonal maintenance: A worm model for adrenoleukodystrophy von Coppa, Andrea, Guha, Sanjib, Fourcade, Stéphane, Parameswaran, Janani, Ruiz, Montserrat, Moser, Ann B., Schlüter, Agatha, Murphy, Michael P., Lizcano, Jose Miguel, Miranda-Vizuete, Antonio, Dalfó, Esther, Pujol, Aurora

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The Value of Mouse Models of Rare Diseases: A Spanish Experience von Murillo-Cuesta, Silvia, Artuch, Rafael, Asensio, Fernando, de la Villa, Pedro, Dierssen, Mara, Enriquez, Jose Antonio, Fillat, Cristina, Fourcade, Stephane, Ibanez, Borja, Montoliu, Lluis, Oliver, Eduardo, Pujol, Aurora, Salido, Eduardo, Vallejo, Mario, Varela-Nieto, Isabel

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Oxidative stress modulates mitochondrial failure and cyclophilin D function in X-linked adrenoleukodystrophy von López-Erauskin, Jone, Galino, Jorge, Bianchi, Patrizia, Fourcade, Stéphane, Andreu, Antoni L., Ferrer, Isidre, Muñoz-Pinedo, Cristina, Pujol, Aurora

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Activating cannabinoid receptor 2 preserves axonal health through GSK-3β/NRF2 axis in adrenoleukodystrophy von Parameswaran, Janani, Goicoechea, Leire, Planas-Serra, Laura, Pastor, Antoni, Ruiz, Montserrat, Calingasan, Noel Y., Guilera, Cristina, Aso, Ester, Boada, Jordi, Pamplona, Reinald, Portero-Otín, Manuel, de la Torre, Rafael, Ferrer, Isidre, Casasnovas, Carlos, Pujol, Aurora, Fourcade, Stéphane

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Tauroursodeoxycholic bile acid arrests axonal degeneration by inhibiting the unfolded protein response in X-linked adrenoleukodystrophy von Launay, Nathalie, Ruiz, Montserrat, Grau, Laia, Ortega, Francisco J., Ilieva, Ekaterina V., Martínez, Juan José, Galea, Elena, Ferrer, Isidre, Knecht, Erwin, Pujol, Aurora, Fourcade, Stéphane

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Oxidative stress regulates the ubiquitin―proteasome system and immunoproteasome functioning in a mouse model of X-adrenoleukodystrophy von LAUNAY, Nathalie, RUIZ, Montserrat, FOURCADE, Stéphane, SCHLÜTER, Agatha, GUILERA, Cristina, FERRER, Isidre, KNECHT, Erwin, PUJOL, Aurora

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Functional genomic analysis unravels a metabolic-inflammatory interplay in adrenoleukodystrophy von Schlüter, Agatha, Espinosa, Lluís, Fourcade, Stéphane, Galino, Jorge, López, Eva, Ilieva, Ekaterina, Morató, Laia, Asheuer, Muriel, Cook, Ted, McLaren, Alistair, Reid, Juliet, Kelly, Fiona, Bates, Stewart, Aubourg, Patrick, Galea, Elena, Pujol, Aurora

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High‐dose biotin restores redox balance, energy and lipid homeostasis, and axonal health in a model of adrenoleukodystrophy von Fourcade, Stéphane, Goicoechea, Leire, Parameswaran, Janani, Schlüter, Agatha, Launay, Nathalie, Ruiz, Montserrat, Seyer, Alexandre, Colsch, Benoit, Calingasan, Noel Ylagan, Ferrer, Isidre, Beal, M. Flint, Sedel, Frédéric, Pujol, Aurora

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SIRT2 in age-related neurodegenerative disorders von Fourcade, Stéphane, Outeiro, Tiago F, Pujol, Aurora

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Early oxidative damage underlying neurodegeneration in X-adrenoleukodystrophy von Fourcade, Stéphane, López-Erauskin, Jone, Galino, Jorge, Duval, Carine, Naudi, Alba, Jove, Mariona, Kemp, Stephan, Villarroya, Francesc, Ferrer, Isidre, Pamplona, Reinald, Portero-Otin, Manuel, Pujol, Aurora

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Steroid Hormones Control Circadian Elovl3 Expression in Mouse Liver von Brolinson, Annelie, Fourcade, Stéphane, Jakobsson, Andreas, Pujol, Aurora, Jacobsson, Anders

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