Endoplasmic reticulum and the unfolded protein response: dynamics and metabolic integration

R Bravo, V Parra, D Gatica, AE Rodriguez… - International review of …, 2013 - Elsevier
The endoplasmic reticulum (ER) is a dynamic intracellular organelle with multiple functions
essential for cellular homeostasis, development, and stress responsiveness. In response to …

The unfolded protein response in neurodegenerative disorders–therapeutic modulation of the PERK pathway

D Hughes, GR Mallucci - The FEBS journal, 2019 - Wiley Online Library
The unfolded protein response (UPR) is a highly conserved protein quality control
mechanism, activated in response to Endoplasmic Reticulum (ER) stress. Signalling is …

Oral treatment targeting the unfolded protein response prevents neurodegeneration and clinical disease in prion-infected mice

JA Moreno, M Halliday, C Molloy, H Radford… - Science translational …, 2013 - science.org
During prion disease, an increase in misfolded prion protein (PrP) generated by prion
replication leads to sustained overactivation of the branch of the unfolded protein response …

Protein folding stress in neurodegenerative diseases: a glimpse into the ER

S Matus, LH Glimcher, C Hetz - Current opinion in cell biology, 2011 - Elsevier
Several neurodegenerative diseases share common neuropathology, primarily featuring the
presence in the brain of abnormal protein inclusions containing specific misfolded proteins …

A systems approach to prion disease

D Hwang, IY Lee, H Yoo, N Gehlenborg… - Molecular systems …, 2009 - embopress.org
Prions cause transmissible neurodegenerative diseases and replicate by conformational
conversion of normal benign forms of prion protein (PrPC) to disease‐causing PrPSc …

Endoplasmic reticulum stress features are prominent in Alzheimer disease but not in prion diseases in vivo

U Unterberger, R Höftberger, E Gelpi… - … of Neuropathology & …, 2006 - academic.oup.com
Prion diseases and Alzheimer disease (AD) share a variety of clinical and neuropathologic
features (eg progressive dementia, accumulation of abnormally folded proteins in diseased …

Unfolded protein response transcription factor XBP-1 does not influence prion replication or pathogenesis

C Hetz, AH Lee, D Gonzalez-Romero… - Proceedings of the …, 2008 - National Acad Sciences
The unfolded protein response (UPR) is a conserved adaptive reaction that increases cell
survival under endoplasmic reticulum (ER) stress conditions. X-box-binding protein-1 (XBP …

Aging and the UPR (ER)

RC Taylor - Brain research, 2016 - Elsevier
The aging process is characterized by tissue decline and the onset of age-associated
disease. It is not, however, immutable, and aging can be modulated by various genetic and …

The stress rheostat: an interplay between the unfolded protein response (UPR) and autophagy in neurodegeneration

S Matus, F Lisbona, M Torres, C Leon… - Current molecular …, 2008 - ingentaconnect.com
The unfolded protein response (UPR) is a conserved adaptive reaction that increases cell
survival under conditions of endoplasmic reticulum (ER) stress. The UPR controls diverse …

Early mechanisms of pathobiology are revealed by transcriptional temporal dynamics in hippocampal CA1 neurons of prion infected mice

A Majer, SJ Medina, Y Niu, B Abrenica… - PLoS …, 2012 - journals.plos.org
Prion diseases typically have long pre-clinical incubation periods during which time the
infectious prion particle and infectivity steadily propagate in the brain. Abnormal neuritic …