title: ER stress signaling and neurodegeneration: At the intersection between Alzheimer's disease and Prion-related disorders.
abstract: 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.
authors.affiliation: Biomedical Neuroscience Institute, Faculty of Medicine, University of Chile, Santiago, Chile; Center for Molecular Studies of the Cell, Institute of Biomedical Sciences, University of Chile, Santiago, Chile; Center for Biological Research in Patagonia, University of Magallanes, Coyhaique, Chile.
authors.forename: Mauricio
authors.initials: M
authors.lastname: Torres
authors.affiliation: Biomedical Neuroscience Institute, Faculty of Medicine, University of Chile, Santiago, Chile; Center for Molecular Studies of the Cell, Institute of Biomedical Sciences, University of Chile, Santiago, Chile; Department of Neurological Sciences, Faculty of Medicine, University of Chile, Chile.
authors.forename: José Manuel
authors.initials: JM
authors.lastname: Matamala
authors.affiliation: Biomedical Neuroscience Institute, Faculty of Medicine, University of Chile, Santiago, Chile; Center for Molecular Studies of the Cell, Institute of Biomedical Sciences, University of Chile, Santiago, Chile.
authors.forename: Claudia
authors.initials: C
authors.lastname: Duran-Aniotz
authors.affiliation: Biomedical Neuroscience Institute, Faculty of Medicine, University of Chile, Santiago, Chile; Center for Molecular Studies of the Cell, Institute of Biomedical Sciences, University of Chile, Santiago, Chile.
authors.forename: Victor Hugo
authors.initials: VH
authors.lastname: Cornejo
authors.affiliation: Biomedical Neuroscience Institute, Faculty of Medicine, University of Chile, Santiago, Chile; Center for Molecular Studies of the Cell, Institute of Biomedical Sciences, University of Chile, Santiago, Chile.
authors.forename: Andrew
authors.initials: A
authors.lastname: Foley
authors.affiliation: Biomedical Neuroscience Institute, Faculty of Medicine, University of Chile, Santiago, Chile; Center for Molecular Studies of the Cell, Institute of Biomedical Sciences, University of Chile, Santiago, Chile; Neurounion Biomedical Foundation, Santiago, Chile; Harvard School of Public Health, Boston, USA. Electronic address: chetz@med.uchile.cl.
authors.forename: Claudio
authors.initials: C
authors.lastname: Hetz
mesh_terms: D000544:Alzheimer Disease / Q000378:metabolism / Q000503:physiopathology*; D000818:Animals; D059865:Endoplasmic Reticulum Stress; D006801:Humans; D009410:Nerve Degeneration / Q000378:metabolism / Q000503:physiopathology*; D011328:Prions / Q000737:chemistry / Q000378:metabolism; D017510:Protein Folding; D011506:Proteins / Q000737:chemistry* / Q000378:metabolism
chemical_list: D011328:Prions; D011506:Proteins
keywords: Amyloid formation; ER-stress; Prion-like diseases; Sustained translational repression; Targeting ER-stress; Unfolded protein response (UPR)