Browsing by Author "XX609648"

Now showing items 41-60 of 63

    • NF kappa B is a central regulator of protein quality control in response to protein aggregation stresses ... 
      Nivon, Mathieu; Fort, Loïc; Muller, Pascale; Richet, Emma; Simon, Stephanie; Guey, Baptiste; Fournier, Maelenn; Arrigo, André-Patrick; Hetz Flores, Claudio; Atkin, Julie D.; Kretz-Remy, Carole (Amer Soc Cell Biology, 2016)
      During cell life, proteins often misfold, depending on particular mutations or environmental changes, which may lead to protein aggregates that are toxic for the cell. Such protein aggregates are the root cause of numerous ...
    • PERK regulated miR-424(322)-503 cluster fine-tunes activation of IRE1 and ATF6 during Unfolded Protein Response 
      Gupta, Ananya; Mosaraf Hossain, Muhammad; Read, Danielle E.; Hetz Flores, Claudio; Samali, Afshin; Gupta, Sanjeev (Nature, 2015)
      The endoplasmic reticulum (ER) responds to changes in intracellular homeostasis through activation of the unfolded protein response (UPR). UPR can facilitate the restoration of cellular homeostasis, via the concerted ...
    • Peroxisomes Get Loud: A Redox Antidote to Hearing Loss 
      Mardones, Pablo; Hetz Flores, Claudio (Cell Press, 2015)
      Pejvakin (PJVK), a protein originally identified in Persian families with sensorineural hearing loss, regulates peroxisomal dynamics and the antioxidant defense triggered by noise exposure in hair cells and auditory neurons ...
    • Perturbation of endoplasmic reticulum homeostasis facilitates prion replication 
      Hetz Flores, Claudio; Castilla, Joaquín; Soto, Claudio (2007)
      Prion diseases are fatal and infectious neurodegenerative disorders characterized by the accumulation of an abnormally folded form of the prion protein ( PrP), termed PrPSc. Prion replication triggers endoplasmic reticulum ...
    • Prion replication alters the distribution of synaptophysin and caveolin 1 in neuronal lipid rafts 
      Russelakis Carneiro, Milene; Hetz Flores, Claudio; Maundrell, Kinsey; Soto, Claudio (AMER SOC INVESTIGATIVE PATHOLOGY, 2004-11)
      The main event in the pathogenesis of prion diseases is the conversion of the cellular prion protein (PrPC) into the abnormal, protease-resistant prion protein (PrPres). PrPC is a GPI-anchored protein located in lipid rafts ...
    • Protein folding stress in neurodegenerative diseases: a glimpse into the ER 
      Matus, Soledad; Glimcher, Laurie H.; Hetz Flores, Claudio (CURRENT BIOLOGY LTD, 2011-03)
      Several neurodegenerative diseases share common neuropathology, primarily featuring the presence in the brain of abnormal protein inclusions containing specific misfolded proteins. Recent evidence indicates that alteration ...
    • Protein homeostasis networks in physiology and disease 
      Hetz Flores, Claudio; Glimcher, Laurie H. (CURRENT BIOLOGY LTD, 2011-04)
    • The Protein-disulfide Isomerase ERp57 Regulates the Steady-state Levels of the Prion Protein 
      Torres, Mauricio; Medinas Bilches, Danilo; Matamala, José Manuel; Woehlbier, Ute; Cornejo, Víctor Hugo; Solda, Tatiana; Andreu, Catherine; Rozas, Pablo; Matus, Soledad; Muñoz, Natalia; Vergara, Carmen; Cartier Rovirosa, Luis; Soto, Claudio; Molinari, Maurizio; Hetz Flores, Claudio (Amer Soc Biochemistry Molecular Biology, 2015)
      Although the accumulation of a misfolded and protease-resistant form of the prion protein (PrP) is a key event in prion pathogenesis, the cellular factors involved in its folding and quality control are poorly understood. ...
    • Proteostasis control by the unfolded protein response 
      Hetz Flores, Claudio; Chevet, Eric; Oakes, Scott A. (Macmillan, 2015)
      Stress induced by accumulation of misfolded proteins in the endoplasmic reticulum is observed in many physiological and pathological conditions. To cope with endoplasmic reticulum stress, cells activate the unfolded protein ...
    • 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, ...
    • Regulation of the endolysosomal pathway by beclin-1 and its role in the proteolytic processing of the amyloid ... 
      González, A.; Cornejo, V.; Hetz Flores, Claudio; González, A.; Mardones, G.; Burgos, P. (Wiley & Sons, 2015)
    • RNA metabolism: putting the brake on the UPR 
      Carreras Sureda, Amado; Hetz Flores, Claudio (Wiley, 2015)
      The unfolded protein response (UPR) is a major signaling cascade that determines cell fate under conditions of endoplasmic reticulum (ER) stress. The kinetics and amplitude of UPR responses are tightly controlled by ...
    • Stressing out the ER in aminoglycoside-induced hearing loss 
      García Huerta, P.; Rivas, A.; Hetz Flores, Claudio (Macmillan Publishers Limited, 2015)
    • Targeting autophagy in ALS A complex mission 
      Nassif, Melissa; Hetz Flores, Claudio (LANDES BIOSCIENCE, 2011-04)
    • Targeting the unfolded protein response for disease intervention 
      Rivas, Alexis; Vidal, René; Hetz Flores, Claudio (Taylor & Francis, 2015)
      Introduction: The accumulation of misfolded proteins in the endoplasmic reticulum (ER) generates a stress condition that engages the unfolded protein response (UPR). The UPR is an adaptive reaction that aims to reestablish ...
    • Targeting the unfolded protein response in disease 
      Hetz Flores, Claudio; Chevet, Eric; Harding, Heather P. (Macmillan, 2013)
      Stress induced by the accumulation of unfolded proteins in the endoplasmic reticulum (ER) is a feature of specialized secretory cells and is also observed in many diseases, including cancer, diabetes, autoimmune conditions, ...
    • The intersection between growth factors, autophagy and ER stress: A new target to treat neurodegenerative diseases? 
      García Huerta, Paula; Troncoso Escudero, Paulina; Jerez, Carolina; Hetz Flores, Claudio; Vidal, René L. (Elsevier Science, 2016-10-15)
      One of the salient features of most neurodegenerative diseases is the aggregation of specific proteins in the brain. This proteostasis imbalance is proposed as a key event triggering the neurodegenerative cascade. The ...
    • The unfolded protein response and cell fate control 
      Hetz Flores, Claudio; Papa, Feroz R. (Cell Press, 2018)
      The secretory capacity of a cell is constantly challenged by physiological demands and pathological perturbations. To adjust and match the protein-folding capacity of the endoplasmic reticulum (ER) to changing secretory ...
    • TMBIM protein family: ancestral regulators of cell death 
      Rojas Rivera, D.; Hetz Flores, Claudio (Nature Publishing Group, 2015)
      The control of apoptosis in mammals has been historically associated with the activity of the BCL-2 family of proteins at the mitochondria. In the past years, a novel group of cell death regulators have emerged, known as ...
    • TMBIM3/GRINA: un gen regulado por la respuesta a proteínas mal plegadas (UPR) que inhibe la apoptosis ... 
      Rojas Rivera, Diego (Universidad de Chile, 2012)
      La familia de proteínas TMBIM (del inglés TransMembrane Bax-Inhibitor 1 Motive) es altamente conservada y ha sido pobremente caracterizada. Los genes de la familia TMBIM están presente en mamíferos, insectos, plantas y ...