Now showing items 1-17 of 17

    • Accumulation of misfolded SOD1 in dorsal root ganglion degenerating propioceptive sensory neurons of transgenic mice with amyotrophic lateral sclerosis 

      Sábado, Javier; Casanovas i Llorens, Anna; Tarabal Mostazo, Olga; Hereu, Marta; Piedrafita Llorens, Lídia; Calderó i Pardo, Jordi; Esquerda Colell, Josep (Hindawi Publishing Corporation, 2014)
      Amyotrophic lateral sclerosis (ALS) is an adult-onset progressive neurodegenerative disease affecting upper and lower motoneurons (MNs). Although the motor phenotype is a hallmark for ALS, there is increasing evidence that ...
    • BAX and BAK proteins are required for cyclin-dependent kinase inhibitory drugs to cause apoptosis 

      Garrofé Ochoa, Xènia; Melero Fernández de Mera, Raquel M; Fernández Gómez, Francisco J.; Ribas i Fortuny, Judit; Jordán Bueso, Joaquín; Boix Torras, Jacint (American Association for Cancer Research, 2008)
      In previous reports we have shown in SH-SY5 cells that Olomoucine and Roscovitine, two inhibitory drugs of Cyclin Dependent Kinases, caused apoptosis independent of the extrinsic pathway. In this experimental paradigm, ...
    • Cell death induced by 2-phenylethynesulfonamide uncovers a pro-survival function of BAX 

      Mattiolo, Paolo; Barbero-Farran A.; Amigó J.; Ripamonti M.; Ribas i Fortuny, Judit; Boix Torras, Jacint (Elsevier, 2014)
      PES (2-phenylethynesulfonamide) was initially identified as an inhibitor of p53 translocation to mitochondria and named Pifithrin-µ. Further studies showed that PES selectively killed tumour cells and was thus a promising ...
    • Deficient endoplasmic reticulum-mitochondrial phosphatidylserine transfer causes liver disease 

      Hernández-Alvarez, María Isabel; Sebastián, David; Vives, Sara; Ivanova, Sa ka; Bartoccioni, Paola; Kakimoto, Pamela; Plana, Natalia; Veiga, Sónia R.; Hernández, Vanessa; Vasconcelos, Nuno; Gopalacharyulu, Peddinti; Adrover, Anna; Jové Font, Mariona; Pamplona Gras, Reinald; Berenguer-Llergo, Antonio; Gordaliza, Isabel; Calvo, Enrique; Cabré, Noemí; Castro, Rui; Kuzmanic, Antonija; Boutant, Marie; Sala, David; Hyotylainen, Tuulia; Oresic, Matej; Fort, Joana; Errasti-Murugarren, Ekaitz; Orozco, Modesto; Joven, Jorge; Cantó, Carles; Palacin, Manuel; Fernández-Veledo, Sonia; Vendrell, Joan; Zorzano, Antonio (Elsevier, 2019-05-02)
      Non-alcoholic fatty liver is the most common liver disease worldwide. Here, we show that the mitochondrial protein mitofusin 2 (Mfn2) protects against liver disease. Reduced Mfn2 expression was detected in liver biopsies ...
    • Depletion of oxidative and endoplasmic reticulum stress regulators in Pick disease 

      Ilieva, Ekaterina V.; Naudí i Farré, Alba; Kichev, Anton Vladimirov; Ferrer, Isidre; Pamplona Gras, Reinald; Portero Otín, Manuel (Elsevier, 2010)
      Both oxidative and endoplasmic reticulum (ER) stress is associated with multiple neurodegenerative, age-related diseases. The rare disorder Pick disease (PiD) shares some pathological hallmarks of other neurodegenerative ...
    • Drosophila expressing human SOD1 successfully recapitulates mitochondrial phenotypic features of familial amyotrophic lateral sclerosis 

      Gallart Palau, Xavier Ramon; Ng, Chee-Hoe; Ribera i Calvet, Joan; Sze, Siu Kwan; Lim, Kah-Leong (Elsevier, 2016)
      Mitochondrial pathology is a seminal pathogenic hallmark of familial amyotrophic lateral sclerosis (FALS) which is extensively manifested by human patients and mutant SOD1G93A mammalian models. Rodents expressing human ...
    • Effect of dietary bioactive compounds on mitochondrial and metabolic flexibility 

      Serrano Casasola, José Carlos Enrique; Cassanyé, Anna; Martín-Gari, Meritxell; Granado-Serrano, Ana Belén; Portero Otín, Manuel (MDPI, 2016-03)
      Metabolic flexibility is the capacity of an organism to adequately respond to changes in the environment, such as nutritional input, energetic demand, etc. An important player in the capacity of adaptation through different ...
    • Executioner Caspase-3 and 7 Deficiency Reduces Myocyte Number in the Developing Mouse Heart 

      Cardona, Maria; López, Juan Antonio; Serafín, Anna; Rongvaux, Anthony; Inserte Igual, Javier; García-Dorado, David; Flavell, Richard; Llovera i Tomàs, Marta; Cañas, Xavier; Vázquez, Jesús; Sanchis, Daniel (Public Library of Science, 2015-06-29)
      Executioner caspase-3 and -7 are proteases promoting cell death but non-apoptotic roles are being discovered. The heart expresses caspases only during development, suggesting they contribute to the organ maturation process. ...
    • Frataxin deficiency in neonatal rat ventricular myocytes targets mitochondria and lipid metabolism 

      Obis Monné, Èlia; Irazusta, Verónica Patricia; Sanchis, Daniel; Ros Salvador, Joaquim; Tamarit Sumalla, Jordi (Elsevier, 2014)
      Friedreich ataxia (FRDA) is a hereditary disease caused by deficient frataxin expression. This mitochondrial protein has been related to iron homeostasis, energy metabolism, and oxidative stress. Patients with FRDA experience ...
    • Impairment of Mitochondrial Redox Status in Peripheral Lymphocytes of Multiple Sclerosis Patients 

      Gonzalo Benito, Hugo; Nogueras Penabad, Lara; Gil-Sánchez, Anna; Vicente Hervás, José; Valcheva, Petya; González Mingot, Cristina; Martin-Gari, Meritxell; Canudes, Marc; Peralta Moncusí, Silvia; Solana Moga, M. José; Pamplona Gras, Reinald; Portero Otín, Manuel; Boada Pallàs, Jordi; Serrano Casasola, José Carlos Enrique; Brieva Ruiz, Luis (Frontiers Media, 2019)
      Literature suggests that oxidative stress (OS) may be involved in the pathogenesis of multiple sclerosis (MS), in which the immune system is known to play a key role. However, to date, the OS in peripheral lymphocytes and ...
    • Involvement of the mitochondrial nuclease EndoG in the regulation of cell proliferation through the control of reactive oxygen species 

      Blasco Angulo, Natividad; Beà Tàrrega, Aida; Barés Junqué, Gisel; Girón, Cristina; Navaridas Fernández de Bobadilla, Raúl; Irazoki, Andrea; López-Lluch, Guillermo; Zorzano, Antonio; Dolcet Roca, Xavier; Llovera i Tomàs, Marta; Sanchis, Daniel (Elsevier, 2020-10-05)
      The apoptotic nuclease EndoG is involved in mitochondrial DNA replication. Previous results suggested that, in addition to regulate cardiomyocyte hypertrophy, EndoG could be involved in cell proliferation. Here, by using ...
    • Loss of SIRT2 leads to axonal degeneration and locomotor disability associated with redox and energy imbalance 

      Fourcade, Stéphane; Morató, Laia; Parameswaran, Janani; Ruiz, Montserrat; Ruiz-Cortés, Tatiana; Jové Font, Mariona; Naudí i Farré, Alba; Martínez, Paloma; Dierssen, Mara; Ferrer, Isidre; Villarroya, Francesc; Pamplona Gras, Reinald; Vaquero, Alejandro; Portero Otín, Manuel; Pujol, Aurora (Wiley, 2017)
      Sirtuin 2 (SIRT2) is a member of a family of NAD+-dependent histone deacetylases (HDAC) that play diverse roles in cellular metabolism and especially for aging process. SIRT2 is located in the nucleus, cytoplasm, and ...
    • Low abundance of NDUFV2 and NDUFS4 subunits of the hydrophilic complex I domain and VDAC1 predicts mammalian longevity 

      Mota Martorell, Natàlia; Jové Font, Mariona; Pradas Barriga, Irene; Sanchez, Isabel; Gómez, José; Naudí i Farré, Alba; Barja, Gustavo; Pamplona Gras, Reinald (Elsevier, 2020-07-01)
      Mitochondrial reactive oxygen species (ROS) production, specifically at complex I (Cx I), has been widely suggested to be one of the determinants of species longevity. The present study follows a comparative approach to ...
    • Mitochondrial localization of the yeast forkhead factor Hcm1 

      Rodríguez Colman, Maria José; Ros Salvador, Joaquim; Cabiscol Català, Elisa (MDPI, 2020-12)
      Hcm1 is a member of the forkhead transcription factor family involved in segregation, spindle pole dynamics, and budding in Saccharomyces cerevisiae. Our group described the role of Hcm1 in mitochondrial biogenesis and ...
    • Prokaryotic and eukaryotic monothiol glutaredoxins are able to perform the functions of Grx5 in the biogenesis of Fe/S clusters in yeast mitochondria 

      Molina Navarro, Maria Micaela; Casas Herranz, Celia; Piedrafita Llorens, Lídia; Bellí i Martínez, Gemma; Herrero Perpiñán, Enrique (Elsevier, 2003)
      The Saccharomyces cerevisiae monothiol glutare- doxin Grx5 participates in the mitochondrial biogenesis of iron–sulfur clusters. Grx5 homologues exist in organisms from bacteria to humans. Chicken (cGRX5) and human ...
    • Target of rapamycin activation predicts lifespan in fruit flies 

      Scialò, Filippo; Sriram, Ashwin; Naudí i Farré, Alba; Ayala Jové, Ma. Victoria (Maria Victoria); Jové Font, Mariona; Pamplona Gras, Reinald; Sanz, Alberto (Taylor & Francis, 2015-09)
      Aging and age-related diseases are one of the most important health issues that the world will confront during the 21st century. Only by understanding the proximal causes will we be able to find treatments to reduce or ...
    • Tor1, Sch9 and PKA downregulation in quiescence rely on Mtl1 to preserve mitochondrial integrity and cell survival 

      Sundaram, Venkatraghavan; Ivanova Petkova, Mima; Pujol Carrión, Núria; Boada Pallàs, Jordi; Torre Ruiz, M. A. de la (John Wiley & Sons, 2015-07-22)
      Here we show that Mtl1, member of the cell wall integrity pathway of Saccharomyces cerevisiae, plays a positive role in chronological life span (CLS). The absence of Mtl1 shortens CLS and causes impairment in the mitochondrial ...