Browsing by Subject "Niemann-Pick type C"

Now showing items 1-2 of 2

    • Lack of activation of the unfolded protein response in mouse and cellular models of Niemann-Pick type C disease 
      Klein, Andres; Mosqueira, Matías; Martínez, Gabriela; Robledo, Fermín; González, Marcela; Caballero, Benjamín; Cancino, Gonzalo I.; Alvarez, Alejandra R.; Hetz Flores, Claudio; Zanlungo, Silvana (2011)
      Background: Niemann-Pick type C (NPC) disease is a fatal lysosomal storage disease related to progressive neurodegeneration secondary to abnormal intracellular accumulation of cholesterol. Signs of endoplasmic reticulum ...
    • Oxidative stress activates the c-Abl/p73 proapoptotic pathway in Niemann-Pick type C neurons 
      Klein, Andres; Maldonado, Carola; Vargas, Lina M.; Gonzalez, Marcela; Robledo, Fermín; Perez de Arce, Karen; Muñoz, Francisco J.; Hetz Flores, Claudio; Alvarez, Alejandra R.; Zanlungo, Silvana (2011)
      Niemann-Pick type C (NPC) is a neurodegenerative disease characterized by the intralysosomal accumulation of cholesterol leading to neuronal apoptosis. We have previously reported the activation of the c-Abl/p73 proapoptotic ...