Now showing items 1-12 of 12

    • Mollereau, B.; Rzechorzek, N. M.; Roussel, B. D.; Sedru, M.; Van den Brink, D. M.; Bailly-Maitre, Beatrice; Palladino, F.; Medinas Bilches, Danilo; Domingos, P. M.; Hunot, S.; Chandran, S.; Birman, S.; Baron, T.; Vivien, D.; Duarte, C. D.; Ryoo, H. D.; Steller, H.; Urano, F.; Chevet, E.; Kroemer, G.; Ciechanover, A.; Calabrese, E. J.; Kaufman, R. J.; Hetz Flores, Claudio (Elsevier, 2016)
      In neurological disorders, both acute and chronic neural stress can disrupt cellular proteostasis, resulting in the generation of pathological protein. However in most cases, neurons adapt to these proteostatic perturbations ...
    • Ramírez, Marcos; Gallardo, Andrés; Vidal, Aarón; Cornejo, Sebastián; Ramírez, Darío; Medinas Bilches, Danilo; Bustamante, Gonzalo; Pasquali, Renzo; Hetz Flores, Claudio (Sociedad Medica de Santiago, 2016)
      © 2016, Sociedad Medica de Santiago. All rights reserved. Creutzfeldt-Jakob disease has a higher incidence in Chile than in other countries. The post mortem pathological characterization of brain tissue is necessary to ...
    • Ramírez, Marcos; Gallardo, Andrés; Vidal, Aarón; Cornejo, Sebastián; Ramírez, Darío; Medinas Bilches, Danilo; Bustamante Fontecilla, Gonzalo; Pasquali, Renzo; Hetz Flores, Claudio (Soc Médica Santiago, 2016)
      Creutzfeldt-Jakob disease has a higher incidence in Chile than in other countries. The post mortem pathological characterization of brain tissue is necessary to reach a definitive diagnosis. We report a 73 years old man ...
    • Bargsted, Leslie; Medinas Bilches, Danilo; Martínez Traub, Francisca; Rozas, Pablo; Muñoz, Natalia; Nassif, Melissa; Jerez, Carolina; Catenaccio, Alejandra; Court, Felipe A.; Hetz Flores, Claudio; Matus, Soledad (Nature Publishing Group, 2017)
      Tar DNA binding protein 43 (TDP-43) is the principal component of ubiquitinated protein inclusions present in nervous tissue of most cases of both amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). ...
    • Medinas Bilches, Danilo; Rozas, Pablo; Martínez Traub, Francisca; Woehlbier, Ute; Brown, Robert H.; Bosco, Daryl A.; Hetz Flores, Claudio (National Academy of Sciences, 2018)
      © National Academy of Sciences. All rights reserved. Abnormal modifications to mutant superoxide dismutase 1 (SOD1) are linked to familial amyotrophic lateral sclerosis (fALS). Misfolding of wild-type SOD1 (SOD1WT) is also ...
    • Medinas Bilches, Danilo; Rozas, Pablo; Martínez Traub, Francisca; Woehlbier, Ute; Brown, Robert H.; Bosco, Daryl A.; Hetz Flores, Claudio (National Academy of Sciences, 2018-08-07)
      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 ...
    • Medinas Bilches, Danilo; Cabral Miranda, Felipe; Hetz Flores, Claudio (Impact Journals LLC, 2019)
    • 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, ...
    • 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 ...
    • Durán Aniotz, Claudia; Cornejo, Victor Hugo; Espinoza, Sandra; Ardiles, Álvaro 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 Verlag, 2017)
      © 2017, Springer-Verlag Berlin Heidelberg. 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 ...
    • 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, ...
    • Rozas, Pablo; Bargsted, Leslie; Martínez, Francisca; Hetz Flores, Claudio; Medinas Bilches, Danilo (Elsevier Ireland Ltd, 2017)
      © 2016 Elsevier Ireland Ltd Amyotrophic lateral sclerosis (ALS) is a fatal late-onset neurodegenerative disease characterized by the selective loss of motoneurons. The mechanisms underlying neuronal degeneration in ALS are ...