Browsing by Author Pujol Onofre, Aurora

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Issue DateTitleAuthor(s)
Dec-2017Loss of SIRT2 leads to axonal degeneration and locomotor disability associated with redox and energy imbalanceFourcade, Stéphane; Morató, Laia; Parameswaran, Janani; Ruiz, Montserrat; Ruiz-Cortés, Tatiana; Jove, Mariona; Naudi, Alba; Martínez Redondo, Paloma; Dierssen, Mara; Ferrer, Isidro (Ferrer Abizanda); Villarroya i Gombau, Francesc; Pamplona, Reinald; Vaquero García, Alejandro; Portero Otin, Manuel; Pujol Onofre, Aurora
1-Jan-2019Loss of the sphingolipid desaturase DEGS1 causes hypomyelinating leukodystrophyPant, Devesh C.; Dorboz, Imen; Schlüter, Agatha; Fourcade, Stéphane; Launay, Nathalie; Joya, Javier; Aguilera Albesa, Sergio; Yoldi, Maria Eugenia; Casasnovas Pons, Carlos; Willis, Mary J.; Ruiz, Montserrat; Ville, Dorothée; Lesca, Gaetan; Siquier Pernet, Karine; Desguerre, Isabelle; Yan, Huifang; Wang, Jingmin; Burmeister, Margit; Brady, Lauren; Tarnopolsky, Mark; Cornet, Carles; Rubbini, Davide; Terriente, Javier; James, Kiely N.; Musaev, Damir; Zaki, Maha S.; Patterson, Marc C.; Lanpher, Brendan C.; Klee, Eric W.; Pinto e Vairo, Filippo; Wohler, Elizabeth; Sobreira, Nara Lygia de M.; Cohen, Julie S.; Maroofian, Reza; Galehdari, Hamid; Mazaheri, Neda; Shariati, Gholamrez; Colleaux, Laurence; Rodriguez, Diana; Gleeson, Joseph G.; Pujades, Cristina; Fatemi, Ali; Boespflug‐Tanguy, Odile; Pujol Onofre, Aurora
1-Apr-2015Neuroinflammatory signals in alzheimer disease and app/psS1 transgenic mice: correlations with plaques, tangles, and oligomeric speciesLópez González, Irene; Schlüter, Agatha; Aso Pérez, Ester; Garcia Esparcia, Paula; Ansoleaga, Belén; Llorens Torres, Franc; Carmona Murillo, Margarita; Moreno Castro, Jesús; Fuso, Andrea; Portero Otin, Manuel; Pamplona, Reinald; Pujol Onofre, Aurora; Ferrer, Isidro (Ferrer Abizanda)
13-Apr-2021Neutralizing Autoantibodies to Type I IFNs in >10% of Patients with Severe COVID-19 Pneumonia Hospitalized in Madrid, SpainTroya, Jesús; Bastard, Paul; Planas Serra, Laura; Ryan, Pablo; Ruiz, Montserrat; Carranza, María de; Torres, Juan; Martínez, Amalia; Abel, Laurent; Casanova, Jean-Laurent; Pujol Onofre, Aurora
Oct-2011Oxidative damage compromises energy metabolism in the axonal degeneration mouse model of X-adrenoleukodystrophyGalino, Jorge; Ruiz, Montserrat; Fourcade, Stéphane; Schlüter, Agatha; López Erauskin, Jone; Guilera, Cristina; Jove, Mariona; Naudi, Alba; García Arumí, Elena; Andreu, Antoni L.; Starkov, Anatoly A.; Pamplona, Reinald; Ferrer, Isidro (Ferrer Abizanda); Portero Otin, Manuel; Pujol Onofre, Aurora
26-Dec-2017Oxidative stress and mitochondrial dynamics malfunction are linked in Pelizaeus-Merzbacher diseaseRuiz, Montserrat; Bégou, Mélina; Launay, Nathalie; Ranea Robles, Pablo; Bianchi, Patrizia; López Erauskin, Jone; Morató, Laia; Guilera, Cristina; Petit, Bérengère; Vaurs‐Barriere, Catherine; Guéret‐Gonthier, Céline; Bonnet‐Dupeyron, Marie‐Noëlle; Fourcade, Stéphane; Auwerx, Johan; Boespflug‐Tanguy, Odile; Pujol Onofre, Aurora
Jul-2010Pathomechanisms Underlying X-Adrenoleukodystrophy: A Three-hit HypothesisSingh, Inderjit; Pujol Onofre, Aurora
13-Mar-2014PDR-1/hParkin negatively regulates the phagocyosis of apoptotic cell corpses in Caenorhabditis elegansCabello, J.; Sämann, J.; Gómez Orte, E.; Erazo, T.; Coppa, Andrea; Pujol Onofre, Aurora; Büssing, I.; Schulze, B.; Lizcano, José Miguel; Baumeister, R.; Ferrer, Isidro (Ferrer Abizanda); Dalfó Capella, Esther
Jan-2010PeroxisomeDB 2.0: an integrative view of the global peroxisomal metabolomeSchlüter, Agatha; Real Chicharro, Alejandro; Gabaldón, Toni; Sánchez Jiménez, Francisca; Pujol Onofre, Aurora
28-Nov-2006PeroxisomeDB: a database for the peroxisomal proteome, functional genomics and diseaseSchlüter, Agatha; Fourcade, Stéphane; Domènech Estévez, Enric; Gabaldón, Toni; Huerta Cepas, Jaime; Berthommier, Guillaume; Ripp, Raymond; Wanders, Ronald J. A.; Poch, Olivier; Pujol Onofre, Aurora
22-Apr-2020Phenotypic correlations in a large single center cohort of patients with BSCL2 nerve disorders: a clinical, neurophysiological and muscle MRI studyFernández Eulate, Gorka; Fernández Torrón, Roberto; Guisasola, Amaia; Iglesias Gaspar, Maria Teresa; Diaz Manera, Jordi; Maneiro, Miren; Zulaica, Miren; Olasagasti, Vicente; Formica, Alejandro Francisco; Espinal, Juan Bautista; Ruiz, Montserrat; Schlüter, Agatha; Pujol Onofre, Aurora; Poza, Juan José; López de Munain, Adolfo
1-Apr-2020POLR3A variants with striatal involvement and extrapyramidal movement disorderHarting, Inga; Al-Saady, Murtadha; Krägeloh-Mann, Ingeborg; Bley, Annette; Hempel, Maja; Bierhals, Tatjana; Karch, Stephanie; Moog, Ute; Bernard, Geneviève; Huntsman, Richard; van Spaendonk, Rosalina M. L.; Vreeburg, Maaike; Rodríguez Palmero, Agustí; Pujol Onofre, Aurora; van der Knaap, Marjo S.; Pouwels, Petra J. W.; Wolf, Nicole I.
16-Jun-2021Regulation of lipid and redox metabolism in X-linked Adrenoleukodystrophy (X-ALD): therapeutic implicationsGoicoechea Barrenechea, Leire
1-Mar-2021Shorter androgen receptor polyQ alleles protect against life-threatening COVID-19 disease in European malesBaldassarri, Margherita; Picchiotti, Nicola; Fava, Francesca; Fallerini, Chiara; Benetti, Elisa; Daga, Sergio; Valentino, Floriana; Doddato, Gabriella; Furini, Simone; Giliberti, Annarita; Tita, Rossella; Amitrano, Sara; Bruttini, Mirella; Croci, Susanna; Meloni, Ilaria; Pinto, Anna Maria; Iuso, Nicola; Gabbi, Chiara; Sciarra, Francesca; Venneri, Mary Anna; Gori, Marco; Sanarico, Maurizio; Crawley, Francis P.; Pagotto, Uberto; Fanelli, Flaminia; Mezzullo, Marco; Dominguez Garrido, Elena; Planas Serra, Laura; Schlüter, Agatha; Colobran, Roger; Soler Palacín, Pere; Lapunzina, Pablo; Tenorio, Jair; Pujol Onofre, Aurora; Castagna, Maria Grazia; Marcelli, Marco; Isidori, Andrea M.; Renieri, Alessandra; Frullanti, Elisa; Mari, Francesca; Spanish Covid HGE, GENCOVID Multicenter Study
1-Mar-2018SIRT2 in age-related neurodegenerative disordersFourcade, Stéphane; Outeiro, Tiago F.; Pujol Onofre, Aurora
23-May-2023Sphingolipid desaturase DEGS1 is essential for mitochondria-associated membrane integrityPlanas Serra, Laura; Launay, Nathalie; Goicoechea, Leire; Heron, Bénédicte; Jou, Cristina; Juliá Palacios, Natalia; Ruiz, Montserrat; Fourcade, Stéphane; Casasnovas Pons, Carlos; Torre, Carolina de la; Gelot, Antoinette; Marsal, Maria; Loza Álvarez, Pablo; García Cazorla, Àngels; Fatemi, Ali; Ferrer, Isidro (Ferrer Abizanda); Portero Otin, Manuel; Area Gómez, Estela; Pujol Onofre, Aurora
25-Oct-2010Staging anti-inflammatory therapy in Alzheimer’s diseaseLichtenstein, Mathieu P.; Carriba Domínguez, Paulina; Masgrau, Roser; Pujol Onofre, Aurora; Galea, Elena
Feb-2017Tauroursodeoxycholic bile acid arrests axonal degeneration by inhibiting the unfolded protein response in X-linked adrenoleukodystrophyLaunay, Nathalie; Ruiz, Montserrat; Grau, Laia; Ortega González, Fco. Javier; Ilieva, Ekaterina V.; Martínez, Juan José; Galea, Elena; Ferrer, Isidro (Ferrer Abizanda); Knecht, Erwin; Pujol Onofre, Aurora; Fourcade, Stéphane
1-Feb-2020The peroxisomal fatty acid transporter ABCD1/PMP-4 is required in the C. elegans hypodermis for axonal maintenance: A worm model for adrenoleukodystrophyCoppa, Andrea; Guha, Sanjib; Fourcade, Stéphane; Parameswaran, Janani; Ruiz, Montserrat; Moser, Ann B.; Schlüter, Agatha; Murphy, Michael P.; Lizcano, José Miguel; Miranda Vizuete, Antonio; Dalfó Capella, Esther; Pujol Onofre, Aurora
14-Oct-2020The Value of Mouse Models of Rare Diseases: A Spanish ExperienceMurillo Cuesta, Silvia; Artuch Iriberri, Rafael; Asensio, Fernando; Villa, Pedro de la; Dierssen, Mara; Enriquez, Jose Antonio; Fillat i Fonts, Cristina; Fourcade, Stéphane; Ibánez, Borja; Montoliu, Lluis; Oliver, Eduardo; Pujol Onofre, Aurora; Salido, Eduardo; Vallejo, Mario; Varela Nieto, Isabel; Working Group on Animal Models of Rare Diseases; Networking Center for Rare Diseases (CIBERER)