Lack of Activation of the Unfolded Protein Response in Mouse and Cellular Models of Niemann-Pick Type C Disease

Andrés D. Klein,Matías Mosqueira,Gabriela Martínez,Fermín Robledo,Marcela González,Benjamin Caballero,Gonzalo I. Cancino,Alejandra R. Alvarez,Claudio Hetz,Silvana Zanlungo

Lack of Activation of the Unfolded Protein Response in Mouse and Cellular Models of Niemann-Pick Type C Disease

2011

Andrés D. Klein
Matías Mosqueira
Gabriela Martínez
Fermín Robledo
Marcela González
Benjamin Caballero
Gonzalo I. Cancino
Alejandra R. Alvarez
Claudio Hetz
Silvana Zanlungo

Background: Niemann-Pick type C (NPC) disease is a fatal lysosomal storage disease related to progressive neurodegeneration secondary to abnormal intracellular accumulation of chole

Keywords:

Unfolded protein response
Intracellular
Cholesterol
Cellular model
Signal transduction
Apoptosis
Cell biology
Lysosomal storage disease
Biology
Endoplasmic reticulum
Niemann–Pick disease, type C

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