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 …

[HTML][HTML] Stress response silencing by an E3 ligase mutated in neurodegeneration

DL Haakonsen, M Heider, AJ Ingersoll, K Vodehnal… - Nature, 2024 - nature.com
Stress response pathways detect and alleviate adverse conditions to safeguard cell and
tissue homeostasis, yet their prolonged activation induces apoptosis and disrupts …

[HTML][HTML] Broad transcriptomic dysregulation occurs across the cerebral cortex in ASD

MJ Gandal, JR Haney, B Wamsley, CX Yap, S Parhami… - Nature, 2022 - nature.com
Neuropsychiatric disorders classically lack defining brain pathologies, but recent work has
demonstrated dysregulation at the molecular level, characterized by transcriptomic and …

Maternal immune activation in mice disrupts proteostasis in the fetal brain

BT Kalish, E Kim, B Finander, EE Duffy, H Kim… - Nature …, 2021 - nature.com
Maternal infection and inflammation during pregnancy are associated with
neurodevelopmental disorders in offspring, but little is understood about the molecular …

[HTML][HTML] The science of puromycin: From studies of ribosome function to applications in biotechnology

R Aviner - Computational and Structural Biotechnology Journal, 2020 - Elsevier
Puromycin is a naturally occurring aminonucleoside antibiotic that inhibits protein synthesis
by ribosome-catalyzed incorporation into the C-terminus of elongating nascent chains …

Correction of eIF2-dependent defects in brain protein synthesis, synaptic plasticity, and memory in mouse models of Alzheimer's disease

MM Oliveira, MV Lourenco, F Longo, NP Kasica… - Science …, 2021 - science.org
Neuronal protein synthesis is essential for long-term memory consolidation, and its
dysregulation is implicated in various neurodegenerative disorders, including Alzheimer's …

[HTML][HTML] Adapting the proteostasis capacity to sustain brain healthspan

C Hetz - Cell, 2021 - cell.com
Sustaining neuronal proteostasis during the course of our life is a central aspect required for
brain function. The dynamic nature of synaptic composition and abundance is a requisite to …

RNA-binding proteins balance brain function in health and disease

R Schieweck, J Ninkovic… - Physiological reviews, 2021 - journals.physiology.org
Posttranscriptional gene expression including splicing, RNA transport, translation, and RNA
decay provides an important regulatory layer in many if not all molecular pathways …

[HTML][HTML] ISRIB blunts the integrated stress response by allosterically antagonising the inhibitory effect of phosphorylated eIF2 on eIF2B

AF Zyryanova, K Kashiwagi, C Rato, HP Harding… - Molecular cell, 2021 - cell.com
The small molecule ISRIB antagonizes the activation of the integrated stress response (ISR)
by phosphorylated translation initiation factor 2, eIF2 (αP). ISRIB and eIF2 (αP) bind distinct …