Endoplasmic reticulum and the unfolded protein response: dynamics and metabolic integration
The endoplasmic reticulum (ER) is a dynamic intracellular organelle with multiple functions
essential for cellular homeostasis, development, and stress responsiveness. In response to …
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 …
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 …
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 …
presence in the brain of abnormal protein inclusions containing specific misfolded proteins …
A systems approach to prion disease
Prions cause transmissible neurodegenerative diseases and replicate by conformational
conversion of normal benign forms of prion protein (PrPC) to disease‐causing PrPSc …
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 …
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
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 …
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 …
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 …
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 …
infectious prion particle and infectivity steadily propagate in the brain. Abnormal neuritic …