Pharmacological targeting of endoplasmic reticulum stress in disease

SJ Marciniak, JE Chambers, D Ron - Nature reviews Drug discovery, 2022 - nature.com
The accumulation of misfolded proteins in the endoplasmic reticulum (ER) leads to ER
stress, resulting in activation of the unfolded protein response (UPR) that aims to restore …

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 …

Advances in progressive supranuclear palsy: new diagnostic criteria, biomarkers, and therapeutic approaches

AL Boxer, JT Yu, LI Golbe, I Litvan, AE Lang… - The Lancet …, 2017 - thelancet.com
Progressive supranuclear palsy (PSP), previously believed to be a common cause of
atypical parkinsonism, is now recognised as a range of motor and behavioural syndromes …

Four-repeat tauopathies

TW Roesler, AT Marvian, M Brendel… - Progress in …, 2019 - Elsevier
Tau is a microtubule-associated protein with versatile functions in the dynamic assembly of
the neuronal cytoskeleton. Four-repeat (4R-) tauopathies are a group of neurodegenerative …

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 integrated stress response and phosphorylated eukaryotic initiation factor 2α in neurodegeneration

S Bond, C Lopez-Lloreda, PJ Gannon… - … of Neuropathology & …, 2020 - academic.oup.com
The proposed molecular mechanisms underlying neurodegenerative pathogenesis are
varied, precluding the development of effective therapies for these increasingly prevalent …

PERK activation mitigates tau pathology in vitro and in vivo

J Bruch, H Xu, TW Rösler, A De Andrade… - EMBO molecular …, 2017 - embopress.org
The RNA‐like endoplasmic reticulum kinase (PERK) is genetically associated with the
tauopathy progressive supranuclear palsy (PSP). To elucidate the functional mechanisms …

Genetics of progressive supranuclear palsy: a review

Y Wen, Y Zhou, B Jiao, L Shen - Journal of Parkinson's …, 2021 - content.iospress.com
Progressive supranuclear palsy (PSP) is an atypical parkinsonism with prominent 4R-tau
neuropathology, and the classical clinical phenotype is characterized by vertical …

A novel specific PERK activator reduces toxicity and extends survival in Huntington's disease models

J Ganz, T Shacham, M Kramer, M Shenkman, H Eiger… - Scientific reports, 2020 - nature.com
One of the pathways of the unfolded protein response, initiated by PKR-like endoplasmic
reticulum kinase (PERK), is key to neuronal homeostasis in neurodegenerative diseases …

Neuronal regulation of eIF2α function in health and neurological disorders

SL Moon, N Sonenberg, R Parker - Trends in Molecular Medicine, 2018 - cell.com
A key site of translation control is the phosphorylation of the eukaryotic translation initiation
factor 2α (eIF2α), which reduces the rate of GDP to GTP exchange by eIF2B, leading to …