Browsing by Author "Medinas Bilches, Danilo"

Now showing items 1-6 of 6

    • 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 ...
    • ERp57 as a novel cellular factor controlling prion protein biosynthesis: Therapeutic potential of protein ... 
      Sepúlveda, Martín; Rozas, Pablo; Hetz Flores, Claudio; Medinas Bilches, Danilo (TAYLOR & FRANCIS, 2016)
      Disturbance of endoplasmic reticulum (ER) proteostasis is observed in Prion-related disorders (PrDs). The protein disulfide isomerase ERp57 is a stress-responsive ER chaperone up-regulated in the brain of Creutzfeldt-Jakob ...
    • 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 ...
    • 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 transcription factor CHOP, a central component of the transcriptional regulatory network induced upon ... 
      Campos, Gisela; Schmidt Heck, Wolfgang; Ghallab, Ahmed; Rochlitz, Katharina; Putter, Larissa; Medinas Bilches, Danilo; Hetz Flores, Claudio; Widera, Agata; Cadenas, Cristina; Begher-Tibbe, Brigitte; Reif, Raymond; Gunther, Georgia; Sachinidis, Agapios; Hengstler, Jan G.; Godoy, Patricio (Springer, 2014)
      Since xenobiotics enter the organism via the liver, hepatocytes must cope with numerous perturbations, including modifications of proteins leading to endoplasmic reticulum stress (ER-stress). This triggers a signaling pathway ...