Browsing by Author "Hetz Flores, Claudio"

Now showing items 41-60 of 119

    • Endoplasmic reticulum proteostasis impairment in aging 
      Martínez Bravo, Gabriela; Durán Aniotz, Claudia; Cabral Miranda, Felipe; Vivar, Juan P.; Hetz Flores, Claudio (Blackwell, 2017)
      Perturbed neuronal proteostasis is a salient feature shared by both aging and protein misfolding disorders. The proteostasis network controls the health of the proteome by integrating pathways involved in protein synthesis, ...
    • Endoplasmic reticulum proteostasis in glioblastoma - From molecular mechanisms to therapeutic perspectives 
      Obacz, Joanna; Avril, Tony; Le Reste, Pierre Jean; Urra, Hery; Quillien, Véronique; Hetz Flores, Claudio; Chevet, Eric (American Association for the Advancement of Science, 2017)
      © 2017 The Authors, some rights reserved.Cellular stress induced by the accumulation of misfolded proteins at the endoplasmic reticulum (ER) is a central feature of secretory cells and is observed in many tissues in various ...
    • Endoplasmic Reticulum Stress and the Hallmarks of Cancer 
      Urra, Hery; Dufey, Estefanie; Avril, Tony; Chevet, Eric; Hetz Flores, Claudio (Cell Press, 2016)
      © 2016 Elsevier Inc. Tumor cells are often exposed to intrinsic and external factors that alter protein homeostasis, thus producing endoplasmic reticulum (ER) stress. To cope with this, cells evoke an adaptive mechanism ...
    • Endoplasmic reticulum stress leads to accumulation of wild-type SOD1 aggregates associated with sporadic ... 
      Medinas Bilches, Danilo; Rozas, Pablo; Martínez Traub, Francisca; Woehlbier, Ute; Brown, Robert H.; Bosco, Daryl A.; Hetz Flores, Claudio (National Academy of Sciences, 2018)
      Abnormal modifications to mutant superoxide dismutase 1 (SOD1) are linked to familial amyotrophic lateral sclerosis (fALS). Misfolding of wild-type SOD1 (SOD1(WT)) is also observed in postmortem tissue of a subset of ...
    • Endoplasmic reticulum stress signalling and the pathogenesis of non-alcoholic fatty liver disease 
      Lebeaupin, Cynthia; Vallée, Deborah; Hazari, Younis; Hetz Flores, Claudio; Chevet, Eric; Bailly-Maitre, Béatrice (Elsevier B.V., 2018)
      © 2018 The global epidemic of obesity has been accompanied by a rising burden of non-alcoholic fatty liver disease (NAFLD), with manifestations ranging from simple steatosis to non-alcoholic steatohepatitis, potentially ...
    • ER proteostasis addiction in cancer biology: Novel concepts 
      Dufey, Estefanie; Urra, Hery; Hetz Flores, Claudio (Academic Press, 2015)
      © 2015 Elsevier Ltd. Endoplasmic reticulum (ER) stress is generated by various physiological and pathological conditions that induce an accumulation of misfolded proteins in its lumen. ER stress activates the unfolded ...
    • ER Proteostasis Control of Neuronal Physiology and Synaptic Function 
      Martínez, Gabriela; Khatiwada, Sanjeev; Costa-Mattioli, Mauro; Hetz Flores, Claudio (Elsevier Ltd, 2018)
      © 2018 Elsevier Ltd Neuronal proteostasis is maintained by the dynamic integration of different processes that regulate the synthesis, folding, quality control, and localization of proteins. The endoplasmic reticulum (ER) ...
    • ER stress and the unfolded protein response in neurodegeneration 
      Hetz Flores, Claudio; Saxena, Smita (Nature Publishing Group, 2017)
      The clinical manifestation of neurodegenerative diseases is initiated by the selective alteration in the functionality of distinct neuronal populations. The pathology of many neurodegenerative diseases includes accumulation ...
    • ER stress links aging to sporadic ALS 
      Medinas Bilches, Danilo; Cabral Miranda, Felipe; Hetz Flores, Claudio (Impact Journals LLC, 2019)
    • ER stress sensing mechanism: Putting off the brake on UPR transducers 
      Rojas Rivera, Diego; Rodríguez, Diego A.; Sepúlveda, Denisse; Hetz Flores, Claudio (Impact Journals LLC, 2018)
    • ER stress signaling and neurodegeneration: At the intersection between Alzheimer's disease and Prion-related ... 
      Torres, Mauricio; Matamala, José Manuel; Durán Aniotz, Claudia; Cornejo, Víctor Hugo; Foley, Andrew; Hetz Flores, Claudio (Elsevier, 2015)
      © 2014 Elsevier B.V. Alzheimer's and Prion diseases are two neurodegenerative conditions sharing different pathophysiological characteristics. Disease symptoms are associated with the abnormal accumulation of protein ...
    • ER stress signaling and the BCL-2 family of proteins: From adaptation to irreversible cellular damage 
      Hetz Flores, Claudio (2007)
      Programmed cell death is essential for the development and maintenance of cellular homeostasis, and its deregulation results in a variety of pathologic conditions. The BCL-2 family of proteins is a group of evolutionarily ...
    • Fine tuning PERK signaling to control cell fate under stress 
      Urra, Hery; Hetz Flores, Claudio (Nature, 2017)
      PERK is a major sensor of the unfolded protein response controlling cell fate under endoplasmic reticulum (ER) stress. A new study reveals an additional step for optimal PERK signaling, involving the binding of CNPY2 to ...
    • Fine-tuning ER stress signal transducers to treat amyotrophic lateral sclerosis 
      Medinas Bilches, Danilo; González, José V.; Falcon, Paulina; Hetz Flores, Claudio (Frontiers Media SA, 2017)
      Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by the progressive loss of motoneurons and paralysis. The mechanisms underlying neuronal degeneration in ALS are starting to be elucidated, ...
    • Fine-Tuning of the Unfolded Protein Response: Assembling the IRE1α Interactome 
      Hetz Flores, Claudio; Glimcher, Laurie H. (2009)
      Endoplasmic reticulum (ER) stress is a hallmark feature of secretory cells and many diseases, including cancer, neurodegeneration, and diabetes. Adaptation to protein-folding stress is mediated by the activation of an ...
    • Function in the development of neurodegenerative diseases 
      García González, Paulina; Cabral Miranda, Felipe; Hetz Flores, Claudio; Osorio Olivares, Fabiola (Frontiers Media S.A., 2018)
      Copyright © 2018 García-González, Cabral-Miranda, Hetz and Osorio. Emerging evidence suggests that the immune and nervous systems are in close interaction in health and disease conditions. Protein aggregation and proteostasis ...
    • Gene therapy in Parkinson’s disease: Targeting the endplasmic reticulum proteostasis network 
      Castillo, Valentina; Mercado, Gabriela; Hetz Flores, Claudio (Editorial Board of Neural Regeneration Research, 2015)
    • Gene Therapy Strategies to Restore ER Proteostasis in Disease 
      Valenzuela, Vicente; Jackson, Kasey L.; Sardi, Sergio P.; Hetz Flores, Claudio (Cell Press, 2018)
      © 2018 The American Society of Gene and Cell Therapy Proteostasis alterations are proposed as a transversal hallmark of several pathological conditions, including metabolic disorders, mechanical injury, cardiac malfunction, ...
    • Gene therapy to target ER stress in brain diseases 
      Valenzuela, Vicente; Martínez, Gabriela; Durán Aniotz, Claudia; Hetz Flores, Claudio (Elsevier B.V., 2016)
      © 2016 Elsevier B.V. Gene therapy based on the use of Adeno-associated viruses (AAVs) is emerging as a safe and stable strategy to target molecular pathways involved in a variety of brain diseases. Endoplasmic reticulum ...
    • Genome-wide circulating microRNA expression profiling reveals potential biomarkers for amyotrophic lateral ... 
      Matamala, José Manuel; Arias Carrasco, Raul; Sánchez, Carolina; Uhrig, Markus; Bargsted, Leslie; Matus, Soledad; Maracaja Coutinho, Vinicius; Abarzua, Sebastián; Van Zundert, Brigitte; Verdugo Latorre, Renato; Manque, Patricio; Hetz Flores, Claudio (Elsevier, 2018)
      The occurrence of mutations of TDP-43, FUS, and C9ORF72 in amyotrophic lateral sclerosis (ALS) suggests pathogenic alterations to RNA metabolism and specifically to microRNA (miRNA) biology. Moreover, several ALS-related ...