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 …

The unfolded protein response in neurodegenerative diseases: a neuropathological perspective

W Scheper, JJM Hoozemans - Acta neuropathologica, 2015 - Springer
The unfolded protein response (UPR) is a stress response of the endoplasmic reticulum
(ER) to a disturbance in protein folding. The so-called ER stress sensors PERK, IRE1 and …

ER stress and the unfolded protein response in neurodegeneration

C Hetz, S Saxena - Nature Reviews Neurology, 2017 - nature.com
The clinical manifestation of neurodegenerative diseases is initiated by the selective
alteration in the functionality of distinct neuronal populations. The pathology of many …

Fine‐tuning PERK signaling for neuroprotection

M Halliday, D Hughes, GR Mallucci - Journal of neurochemistry, 2017 - Wiley Online Library
Protein translation and folding are tightly controlled processes in all cells, by proteostasis, an
important component of which is the unfolded protein response (UPR). During periods of …

PERK-opathies: an endoplasmic reticulum stress mechanism underlying neurodegeneration

MC Bell, SE Meier, AL Ingram… - Current Alzheimer …, 2016 - ingentaconnect.com
The unfolded protein response (UPR) plays a vital role in maintaining cell homeostasis as a
consequence of endoplasmic reticulum (ER) stress. However, prolonged UPR activity leads …

Disturbance of endoplasmic reticulum proteostasis in neurodegenerative diseases

C Hetz, B Mollereau - Nature Reviews Neuroscience, 2014 - nature.com
The unfolded protein response (UPR) is a homeostatic mechanism by which cells regulate
levels of misfolded proteins in the endoplasmic reticulum (ER). Although it is well …

The unfolded protein response: mechanisms and therapy of neurodegeneration

HL Smith, GR Mallucci - Brain, 2016 - academic.oup.com
Activation of the unfolded protein response is emerging as a common theme in protein-
misfolding neurodegenerative diseases, with relevant markers observed in patient tissue …

Targeting the unfolded protein response in neurodegeneration: a new approach to therapy

M Halliday, GR Mallucci - Neuropharmacology, 2014 - Elsevier
Neurodegenerative diseases such as Alzheimer's disease (AD), Parkinson's disease (PD),
Huntington's disease (HD), and the rarer prion diseases, share a number of key similarities …

[HTML][HTML] The endoplasmic reticulum unfolded protein response in neurodegenerative disorders and its potential therapeutic significance

P Remondelli, M Renna - Frontiers in molecular neuroscience, 2017 - frontiersin.org
In eukaryotic cells, the endoplasmic reticulum (ER) is the cell compartment involved in
secretory protein translocation and quality control of secretory protein folding. Different …

The UPR and synaptic dysfunction in neurodegeneration

OJ Freeman, GR Mallucci - Brain research, 2016 - Elsevier
Activation of the unfolded protein response (UPR) is emerging as a common theme in
neurodegenerative diseases, seen in both human brain tissue and mouse models. Genetic …