Browsing by Author "Woehlbier, Ute"

Now showing items 1-7 of 7

    • 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 ...
    • 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 ...
    • Functional Role of the Disulfide Isomerase ERp57 in Axonal Regeneration 
      Castillo, Valentina; Oñate, Maritza G.; Woehlbier, Ute; Rozas, Pablo; Andreu, Catherine; Medinas Bilches, Danilo; Valdés, Pamela; Osorio, Fabiola; Mercado, Gabriela; Vidal, René L.; Kerr, Bredford; Court, Felipe A.; Hetz Flores, Claudio (Public Library Science, 2015)
      ERp57 (also known as grp58 and PDIA3) is a protein disulfide isomerase that catalyzes disulfide bonds formation of glycoproteins as part of the calnexin and calreticulin cycle. ERp57 is markedly upregulated in most common ...
    • Identification of rare protein disulfide isomerase gene variants in amyotrophic lateral sclerosis patients 
      González Pérez, Paloma; Woehlbier, Ute; Chian, Ru-Ju; Sapp, Peter; Rouleau, Guy A.; Leblond, Claire S.; Daoud, Hussein; Dion, Patrick A.; Landers, John E.; Hetz Flores, Claudio; Brown, Robert H. (Elsevier, 2015)
      Disruption of endoplasmic reticulum (ER) proteostasis is a salient feature of amyotrophic lateral sclerosis (ALS). Upregulation of ER foldases of the protein disulfide isomerase (PDI) family has been reported in ALS mouse ...
    • Neuronal rubicon represses extracellular APP/amyloid beta deposition in Alzheimer's disease 
      Espinoza, Sandra; Grunenwald, Felipe; Gomez, Wileidy; García, Felipe; Abarzúa Catalán, Lorena; Oyarce Pezoa, Sebastián; Hernández, María Fernanda; Cortés, Bastián I.; Uhrig, Markus; Ponce de la Vega, Daniela Paz; Durán Aniotz, Claudia; Hetz Flores, Claudio Andres; San Martín Rovirosa, Carol Dazil; Cornejo Corona, Víctor Hugo; Ezquer, Fernando; Parra Ortiz, Valentina María; Behrens Pellegrino, María Isabel Ofelia; Manque, Patricio A.; Rojas Rivera, Diego; Vidal, René L.; Woehlbier, Ute; Nassif, Melissa (MDPI, 2022)
      Alzheimer's disease (AD) is the most prevalent age-associated neurodegenerative disease. A decrease in autophagy during aging contributes to brain disorders by accumulating potentially toxic substrates in neurons. Rubicon ...
    • 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. ...
    • The autophagy protein pacer positively regulates the therapeutic potential of mesenchymal stem cells in a ... 
      Bergmann, Cristian A.; Beltrán, Sebastián; Vega Letter, Ana María; Murgas, Paola; Hernández, María Fernanda; Gómez, Laura; Labrador, Luis; Cortés, Bastián I.; Poblete, Cristian; Quijada Cruz, Cristóbal Emilio; Carrión, Flavio; Woehlbier, Ute; Manque, Patricio A. (MDPI, 2022)
      Mesenchymal stem cells (MSC) have emerged as a promising tool to treat inflammatory diseases, such as inflammatory bowel disease (IBD), due to their immunoregulatory properties. Frequently, IBD is modeled in mice by using ...