Browsing by Subject "Unfolded protein response"

Now showing items 1-20 of 25

    • A decay of the adaptive capacity of the unfolded protein response exacerbates Alzheimer's disease 
      Gerakis, Yannis; Hetz Flores, Claudio (Elsevier, 2018)
      Alterations in the buffering capacity of the proteostasis network are a salient feature of Alzheimer's disease, associated with the occurrence of chronic endoplasmic reticulum (ER) stress. To cope with ER stress, cells ...
    • AAV-mediated delivery of the transcription factor XBP1s into the striatum reduces mutant Huntingtin aggregation ... 
      Zuleta, Amparo; Vidal, Rene L.; Armentano, Donna; Parsons, Geoffrey; Hetz Flores, Claudio (2012)
      Huntington's disease (HD) is caused by mutations that expand a polyglutamine region in the amino-terminal domain of Huntingtin (Htt), leading to the accumulation of intracellular inclusions and progressive neurodegeneration. ...
    • Addicted to secrete - novel concepts and targets in cancer therapy 
      Dejeans, Nicolas; Manié, Serge; Hetz Flores, Claudio; Bard, Frédéric; Hupp, Ted; Agostinis, Patrizia; Samali, Afshin; Chevet, Eric (Elsevier Ltd, 2014)
      The unfolded protein response (UPR) mediates the adaptation of the secretory pathway (SP) to fluctuations in cellular protein demand or to environmental variations. Recently, drug screenings have confirmed the therapeutic ...
    • An ERcentric view of Parkinson's disease 
      Mercado, Gabriela; Valdés, Pamela; Hetz Flores, Claudio (2013)
      Parkinson's disease (PD) is the second most common neurodegenerative disease and is characterized by the selective loss of dopaminergic neurons of the substantia nigra pars compacta and the accumulation of intracellular ...
    • Autophagy in hepatic adaptation to stress 
      Hazari, Younis M.; Bravo San Pedro, José Manuel; Hetz Flores, Claudio; Galluzzi, Lorenzo; Kroemer, Guido (Elsevier, 2020)
      Autophagy is an evolutionarily ancient process whereby eukaryotic cells eliminate disposable or potentially dangerous cytoplasmic material, to support bioenergetic metabolism and adapt to stress. Accumulating evidence ...
    • Calcium signaling at the endoplasmic reticulum: fine-tuning stress responses 
      Carreras Sureda, Amado; Pihan, Philippe; Hetz Flores, Claudio (Elsevier, 2018)
      Endoplasmic reticulum (ER) calcium signaling is implicated in a myriad of coordinated cellular processes. The ER calcium content is tightly regulated as it allows a favorable environment for protein folding, in addition ...
    • Causal role of oxidative stress in unfolded protein response development in the hyperthyroid state 
      Videla Cabrera, Luis; Fernández Arancibia, Virginia; Cornejo, Pamela; Vargas, Romina; Carrasco, Juan; Fernández, Javier; Varela Figueroa, Nelson (Elsevier, 2015)
      L-3,3',5-Triiodothyronine (T-3) induced liver oxidative stress underlies significant protein oxidation, which may trigger the unfolded protein response (UPR). Administration of daily doses of 0.1 mg T-3 for three consecutive ...
    • Control of lysosomal-mediated cell death by the pH-dependent calcium channel RECS1 
      Pihan, Philippe; Lisbona, Fernanda; Borgonovo, Janina Edith; Edwards Jorquera, Sandra Sofía; Nunes Hasler, Paula; Castillo, Karen; Kepp, Oliver; Urra, Hery; Saarnio, Suvi; Vihinen, Helena; Carreras Sureda, Amado; Forveille, Sabrina; Sauvat, Allan; De Giorgis, Daniela; Pupo, Amaury; Rodríguez, Diego A.; Quarato, Giovanni; Sagredo, Alfredo; Lourido Araneda, María Fernanda; Letai, Anthony; Latorre, Ramón; Kroemer, Guido; Demaurex, Nicolás; Jokitalo, Eija; Concha Nordemann, Miguel Luis Angel; Glavic Maurer, Alvaro Alberto; Green, Douglas R.; Hetz Flores, Claudio Andrés (Amer Assoc Advancement Science, 2021)
      Programmed cell death is regulated by the balance between activating and inhibitory signals. Here, we have identified RECS1 (responsive to centrifugal force and shear stress 1) [also known as TMBIM1 (transmembrane BAX ...
    • Converging pathways in the occurrence of endoplasmic reticulum (er) stress in Huntington's disease 
      Vidal, R.; Caballero, B.; Couve Correa, Andrés; Hetz Flores, Claudio (2011)
      A variety of neurological diseases including Huntington's disease (HD), Alzheimer's disease and Parkinson's disease share common neuropathology, primarily featuring the presence of abnormal protein inclusions containing ...
    • 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, ...
    • ER stress and Parkinson's disease: Pathological inputs that converge into the secretory pathway 
      Mercado, Gabriela; Castillo, Valentina; Soto, Paulina; Sidhu, Anita (Elsevier, 2016)
      The major clinical feature of Parkinson's disease (PD) is impairment in motor control as a result of extensive dopaminergic neuron loss in the substantia nigra pars compacta. The central pathological hallmark of PD is the ...
    • 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 ...
    • Injury to the nervous system: A look into the ER 
      Valenzuela, Vicente; Oñate, Maritza; Hetz Flores, Claudio; Court, Felipe A. (Elsevier B.V., 2016)
      © 2016 Elsevier B.V.Injury to the central or peripheral nervous systems leads to the loss of cognitive and/or sensorimotor capabilities that still lack an effective treatment. Although injury to the nervous system involves ...
    • IRE1 alpha governs cytoskeleton remodelling and cell migration through a direct interaction with filamin A 
      Urra, Hery; Henríquez, Daniel R.; Cánovas, José; Villarroel Campos, David; Carreras Sureda, Amado; Pulgar, Eduardo; Molina, Emiliano; Hazari, Younis M.; Limia, Celia M.; Álvarez Rojas, Sebastián; Figueroa, Ricardo; Vidal, René L.; Rodríguez, Diego A.; Rivera, Claudia A.; Court, Felipe A.; Couve Correa, Andrés; Qi, Ling; Chevet, Eric; Akai, Ryoko; Iwawaki, Takao; Concha, Miguel L.; Glavic Maurer, Álvaro; González Billault, Christian; Hetz Flores, Claudio (Nature, 2018)
      Maintenance of endoplasmic reticulum (ER) proteostasis is controlled by a signalling network known as the unfolded protein response (UPR). Here, we identified filamin A as a major binding partner of the ER stress transducer ...
    • IRE1 signaling exacerbates Alzheimer's disease pathogenesis 
      Durán Aniotz, Claudia; Cornejo, Victor Hugo; Espinoza, Sandra; Ardiles, Alvaro O.; Medinas Bilches, Danilo; Salazar, Claudia; Foley, Andrew; Gajardo, Ivana; Thielen, Peter; Iwawaki, Takao; Scheper, Wiep; Soto, Claudio; Palacios, Adrian G.; Hoozemans, Jeroen J. M.; Hetz Flores, Claudio (Springer, 2017)
      Altered proteostasis is a salient feature of Alzheimer's disease (AD), highlighting the occurrence of endoplasmic reticulum (ER) stress and abnormal protein aggregation. ER stress triggers the activation of the unfolded ...
    • Lack of activation of the unfolded protein response in mouse and cellular models of Niemann-Pick type C disease 
      Klein, Andres; Mosqueira, Matías; Martínez, Gabriela; Robledo, Fermín; González, Marcela; Caballero, Benjamín; Cancino, Gonzalo I.; Alvarez, Alejandra R.; Hetz Flores, Claudio; Zanlungo, Silvana (2011)
      Background: Niemann-Pick type C (NPC) disease is a fatal lysosomal storage disease related to progressive neurodegeneration secondary to abnormal intracellular accumulation of cholesterol. Signs of endoplasmic reticulum ...
    • Modulation of endothelial cell migration by ER stress and insulin resistance: A role during maternal obesity? 
      Sáez, Pablo J.; Villalobos-Labra, Roberto; Westermeier, Francisco; Sobrevia, Luis; Farías-Jofré, Marcelo (Frontiers Research Foundation, 2014)
      Adverse microenvironmental stimuli can trigger the endoplasmic reticulum (ER) stress pathway, which initiates the unfolded protein response (UPR), to restore protein-folding homeostasis. Several studies show induction of ...
    • Proteostasis disturbance in amyotrophic lateral sclerosis 
      Medinas Bilches, Danilo; Valenzuela Paterakis, Vicente; Hetz Flores, Claudio (Oxford University Press, 2017)
      Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease affecting motoneurons in the brain and spinal cord leading to paralysis and death. Although the etiology of ALS remains poorly understood, ...
    • Role of the unfolded protein response in organ physiology: lessons from mouse models 
      Cornejo, Víctor Hugo; Pihán, Philippe; Vidal, René Luis; Hetz Flores, Claudio (2013)
      The endoplasmic reticulum (ER) is a key subcellular compartment involved in the folding and maturation of around one-third of the total proteome. Accumulation of misfolded proteins in the ER lumen engages a signal transduction ...
    • Stressing out the ER in aminoglycoside-induced hearing loss 
      García Huerta, P.; Rivas, A.; Hetz Flores, Claudio (Macmillan Publishers Limited, 2015)