Now showing items 1-4 of 4

    • Activation of sirtuin 1 as therapy for the peroxisomal disease adrenoleukodystrophy 

      Morató, Laia; Ruiz, M.; Boada Pallàs, Jordi; Calingasan, Noel Ylagan; Galino, Jorge; Guilera, Cristina; Jové Font, Mariona; Naudí i Farré, Alba; Ferrer, Isidre; Pamplona Gras, Reinald; Serrano, M.; Portero Otín, Manuel; Beal, M. F.; Fourcade, Stéphane; Pujol, Aurora (Macmillan Publishers, 2015)
      Oxidative stress and mitochondrial failure are prominent factors in the axonal degeneration process. In this study, we demonstrate that sirtuin 1 (SIRT1), a key regulator of the mitochondrial function, is impaired in the ...
    • Altered glycolipid and glycerophospholipid signaling drive inflammatory cascades in adrenomyeloneuropathy 

      Ruiz, Montserrat; Jové Font, Mariona; Schlüter, Agatha; Casasnovas, Carlos; Villarroya, Francesc; Guilera, Cristina; Ortega, Francisco J.; Naudí i Farré, Alba; Pamplona Gras, Reinald; Gimeno, Ramón; Fourcade, Stéphane; Portero Otín, Manuel; Pujol, Aurora (Oxford University Press, 2015)
      X-linked adrenomyeloneuropathy (AMN) is an inherited neurometabolic disorder caused by malfunction of the ABCD1 gene, characterized by slowly progressing spastic paraplegia affecting corticospinal tracts, and adrenal ...
    • Oxidative damage compromises energy metabolism in the axonal degeneration mouse model of X-Adrenoleukodystrophy 

      Fourcade, Stéphane; Schlüter, Agatha; López Erauskin, Jone; Guilera, Cristina; Jové Font, Mariona; Naudí i Farré, Alba; García Arumí, Elena; Andreu, Antoni L.; Starkov, Anatoly A.; Pamplona Gras, Reinald; Ferrer, Isidre; Portero Otín, Manuel; Pujol, Aurora (Mary Ann Liebert, Inc., 2011)
      Aims: Chronic metabolic impairment and oxidative stress are associated with the pathogenesis of axonal dysfunction in a growing number of neurodegenerative conditions. To investigate the intertwining of both noxious factors, ...
    • Valproic acid induces antioxidant effects in X-linked adrenoleukodystrophy 

      Fourcade, Stéphane; Ruiz, Montserrat; Guilera, Cristina; Hahnen, Eric; Brichta, Lars; Naudí i Farré, Alba; Portero Otín, Manuel; Dacremont, Georges; Cartier, Nathalie; Wanders, Ronald; Kemp, Stephan; Mandel, Jean Louis; Wirth, Brunhilde; Pamplona Gras, Reinald; Aubourg, Patrick; Pujol, Aurora (Oxford University Press, 2010)
      X-linked adrenoleukodystrophy (X-ALD) is a fatal, axonal demyelinating, neurometabolic disease. It results from the functional loss of a member of the peroxisomal ATP-binding cassette transporter subfamily D (ABCD1), ...