TY - JOUR
T1 - ER stress signaling and neurodegeneration
T2 - At the intersection between Alzheimer's disease and Prion-related disorders
AU - Torres, Mauricio
AU - Matamala, José Manuel
AU - Duran-Aniotz, Claudia
AU - Cornejo, Victor Hugo
AU - Foley, Andrew
AU - Hetz, Claudio
N1 - Publisher Copyright:
© 2014 Elsevier B.V.
PY - 2015/9/2
Y1 - 2015/9/2
N2 - Alzheimer's and Prion diseases are two neurodegenerative conditions sharing different pathophysiological characteristics. Disease symptoms are associated with the abnormal accumulation of protein aggregates, which are generated by the misfolding and oligomerization of specific proteins. Recent functional studies uncovered a key role of endoplasmic reticulum (ER) stress and the unfolded protein response (UPR) in the occurrence of synaptic dysfunction and neurodegeneration in Prion-related disorders and Alzheimer's disease. Here we review common pathological features of both diseases, emphasizing the link between amyloid formation, its pathogenesis and alterations in ER proteostasis. The potential benefits of targeting the UPR as a therapeutic strategy is also discussed.
AB - Alzheimer's and Prion diseases are two neurodegenerative conditions sharing different pathophysiological characteristics. Disease symptoms are associated with the abnormal accumulation of protein aggregates, which are generated by the misfolding and oligomerization of specific proteins. Recent functional studies uncovered a key role of endoplasmic reticulum (ER) stress and the unfolded protein response (UPR) in the occurrence of synaptic dysfunction and neurodegeneration in Prion-related disorders and Alzheimer's disease. Here we review common pathological features of both diseases, emphasizing the link between amyloid formation, its pathogenesis and alterations in ER proteostasis. The potential benefits of targeting the UPR as a therapeutic strategy is also discussed.
KW - Amyloid formation
KW - ER-stress
KW - Prion-like diseases
KW - Sustained translational repression
KW - Targeting ER-stress
KW - Unfolded protein response (UPR)
UR - http://www.scopus.com/inward/record.url?scp=84938744930&partnerID=8YFLogxK
U2 - 10.1016/j.virusres.2014.12.018
DO - 10.1016/j.virusres.2014.12.018
M3 - Article
C2 - 25556124
AN - SCOPUS:84938744930
SN - 0168-1702
VL - 207
SP - 69
EP - 75
JO - Virus Research
JF - Virus Research
ER -