Now showing items 1-3 of 3

    • 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 ...
    • Impaired mitochondrial oxidative phosphorylation in the peroxisomal disease X-linked adrenoleukodystrophy 

      López Erauskin, Jone; Galino, Jorge; Ruiz, M.; Cuezva, J. M.; Fabregat, I.; Cacabelos Barral, Daniel; Boada Pallàs, Jordi; Martínez, Juan José; Ferrer, Isidre; Pamplona Gras, Reinald; Villarroya, Francesc; Portero Otín, Manuel; Fourcade, Stéphane; Pujol, A. (Oxford Journals, 2013)
      X-linked adrenoleukodystrophy (X-ALD) is an inherited metabolic disorder of the nervous system characterized by axonopathy in spinal cords and/or cerebral demyelination, adrenal insufficiency and accumulation of very ...
    • Pioglitazone halts axonal degeneration in a mouse model of X-linked adrenoleukodystrophy 

      Morató, Laia; Galino, Jorge; Ruiz, Montserrat; Calingasan, Noel Ylagan; Starkov, Anatoly A.; Dumont, Magali; Naudí i Farré, Alba; Martínez, Juan José; Aubourg, Patrick; Portero Otín, Manuel; Pamplona Gras, Reinald; Galea, Elena; Flint Beal, M.; Ferrer, Isidre; Fourcade, Stéphane; Pujol, Aurora (Oxford Journals, 2013)
      X-linked adrenoleukodystrophy is a neurometabolic disorder caused by inactivation of the peroxisomal ABCD1 transporter of very long-chain fatty acids. In mice, ABCD1 loss causes late onset axonal degeneration in the spinal ...