Browsing by Author "Matus, Soledad"

Now showing items 1-8 of 8

    • ALS-linked protein disulfide isomerase variants cause motor dysfunction 
      Woehlbier, Ute; Colombo Flores, Alicia; Saaranen, Mirva J.; Pérez, Viviana; Ojeda, Jorge; Bustos, Fernando J.; Andreu, Catherine; Torres, Mauricio; Valenzuela, Vicente; Medinas Bilches, Danilo; Rozas, Pablo; Vidal, René L.; López González, Rodrigo; Salameh, Johnny; Fernández Collemann, Sara; Muñoz, Natalia; Matus, Soledad; Armisen Yáñez, Ricardo; Sagredo, Alfredo; Palma, Karina; Irrázabal, Thergiory; Almeida, Sandra; González Pérez, Paloma; Campero, Mario; Gao, Fen-Biao; Henny, Pablo; Van Zundert, Brigitte; Ruddock, Lloyd W.; Concha, Miguel L.; Henríquez, Juan P.; Brown, Robert H.; Hetz Flores, Claudio (Wiley-Blackwell, 2016)
      Disturbance of endoplasmic reticulum (ER) proteostasis is a common feature of amyotrophic lateral sclerosis (ALS). Protein disulfide isomerases (PDIs) are ER foldases identified as possible ALS biomarkers, as well as ...
    • Autophagy meets fused in sarcoma-positive stress granules 
      Matus, Soledad; Bosco, Daryl A.; Hetz Flores, Claudio (Elsevier, 2014)
      Mutations in fused in sarcoma and/or translocated in liposarcoma (FUS, TLS or FUS) are linked to familial cases of amyotrophic lateral sclerosis (ALS). Mutant FUS selectively accumulates into discrete cytosolic structures ...
    • ERp57 in neurodegeneration and regeneration 
      Bargsted, Leslie; Hetz Flores, Claudio; Matus, Soledad (MEDKNOW PUBLICATIONS & MEDIA, 2016)
    • Functional Contribution of the Transcription Factor ATF4 to the Pathogenesis of Amyotrophic Lateral Sclerosis 
      Matus, Soledad; López, Estefanía; Valenzuela, Vicente; Nassif, Melissa; Hetz Flores, Claudio (PUBLIC LIBRARY SCIENCE, 2013-07-18)
      Endoplasmic reticulum (ER) stress represents an early pathological event in amyotrophic lateral sclerosis (ALS). ATF4 is a key ER stress transcription factor that plays a role in both adaptation to stress and the activation ...
    • 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 ...
    • 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. ...
    • Rol de la respuesta de proteínas mal plegadas (UPR) en la patología mediada por TDP-43 en la esclerosis ... 
      Bargsted Elgueda, Leslie Ann (Universidad de Chile, 2016)
    • The potential role of protein kinase R as a regulator of age-related neurodegeneration 
      Martínez, Nicolás W.; Gómez, Felipe E.; Matus, Soledad (Frontiers Media, 2021)
      There is a growing evidence describing a decline in adaptive homeostasis in agingrelated diseases affecting the central nervous system (CNS), many of which are characterized by the appearance of non-native protein ...