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 …

The integrated stress response: From mechanism to disease

M Costa-Mattioli, P Walter - Science, 2020 - science.org
BACKGROUND The integrated stress response (ISR) is an evolutionarily conserved
intracellular signaling network that helps the cell, tissue, and organism to adapt to a variable …

Endoplasmic reticulum stress and unfolded protein response in neurodegenerative diseases

R Ghemrawi, M Khair - International journal of molecular sciences, 2020 - mdpi.com
The endoplasmic reticulum (ER) is an important organelle involved in protein quality control
and cellular homeostasis. The accumulation of unfolded proteins leads to an ER stress …

Endoplasmic reticulum stress signalling–from basic mechanisms to clinical applications

A Almanza, A Carlesso, C Chintha… - The FEBS …, 2019 - Wiley Online Library
The endoplasmic reticulum (ER) is a membranous intracellular organelle and the first
compartment of the secretory pathway. As such, the ER contributes to the production 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 …

Human ALS/FTD brain organoid slice cultures display distinct early astrocyte and targetable neuronal pathology

K Szebényi, LMD Wenger, Y Sun, AWE Dunn… - Nature …, 2021 - nature.com
Amyotrophic lateral sclerosis overlapping with frontotemporal dementia (ALS/FTD) is a fatal
and currently untreatable disease characterized by rapid cognitive decline and paralysis …

Protein misfolding in the endoplasmic reticulum as a conduit to human disease

M Wang, RJ Kaufman - Nature, 2016 - nature.com
In eukaryotic cells, the endoplasmic reticulum is essential for the folding and trafficking of
proteins that enter the secretory pathway. Environmental insults or increased protein …

[HTML][HTML] Stress granule assembly disrupts nucleocytoplasmic transport

K Zhang, JG Daigle, KM Cunningham, AN Coyne… - Cell, 2018 - cell.com
Defects in nucleocytoplasmic transport have been identified as a key pathogenic event in
amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) mediated by a …

Pharmacological targeting of the unfolded protein response for disease intervention

C Hetz, JM Axten, JB Patterson - Nature chemical biology, 2019 - nature.com
Accumulation of unfolded proteins at the endoplasmic reticulum (ER) is a salient attribute of
many human diseases including obesity, liver disorders, cancer, diabetes and …

Astrocyte unfolded protein response induces a specific reactivity state that causes non-cell-autonomous neuronal degeneration

HL Smith, OJ Freeman, AJ Butcher, S Holmqvist… - Neuron, 2020 - cell.com
Recent interest in astrocyte activation states has raised the fundamental question of how
these cells, normally essential for synapse and neuronal maintenance, become pathogenic …