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 …

GBA Variants and Parkinson Disease: Mechanisms and Treatments

L Smith, AHV Schapira - Cells, 2022 - mdpi.com
The GBA gene encodes for the lysosomal enzyme glucocerebrosidase (GCase), which
maintains glycosphingolipid homeostasis. Approximately 5–15% of PD patients have …

Protein quality control in the secretory pathway

Z Sun, JL Brodsky - Journal of Cell Biology, 2019 - rupress.org
Protein folding is inherently error prone, especially in the endoplasmic reticulum (ER). Even
with an elaborate network of molecular chaperones and protein folding facilitators …

Proteotoxic stress and the ubiquitin proteasome system

R Kandel, J Jung, S Neal - Seminars in cell & developmental biology, 2024 - Elsevier
The ubiquitin proteasome system maintains protein homeostasis by regulating the
breakdown of misfolded proteins, thereby preventing misfolded protein aggregates. The …

Severe influenza pneumonitis in children with inherited TLR3 deficiency

HK Lim, SXL Huang, J Chen, G Kerner… - Journal of Experimental …, 2019 - rupress.org
Autosomal recessive IRF7 and IRF9 deficiencies impair type I and III IFN immunity and
underlie severe influenza pneumonitis. We report three unrelated children with influenza A …

The interplay between endoplasmic reticulum stress and inflammation

SZ Hasnain, R Lourie, I Das, ACH Chen… - Immunology and cell …, 2012 - Wiley Online Library
Endoplasmic reticulum (ER) stress may be both a trigger and consequence of chronic
inflammation. Chronic inflammation is often associated with diseases that arise because of …

Protein kinase R is an innate immune sensor of proteotoxic stress via accumulation of cytoplasmic IL-24

S Davidson, CH Yu, A Steiner, F Ebstein, PJ Baker… - Science …, 2022 - science.org
Proteasome dysfunction can lead to autoinflammatory disease associated with elevated type
I interferon (IFN-αβ) and NF-κB signaling; however, the innate immune pathway driving this …

TFEB regulates lysosomal proteostasis

W Song, F Wang, M Savini, A Ake… - Human molecular …, 2013 - academic.oup.com
Loss-of-function diseases are often caused by destabilizing mutations that lead to protein
misfolding and degradation. Modulating the innate protein homeostasis (proteostasis) …

2-hydroxypropyl-β-cyclodextrin promotes transcription factor EB-mediated activation of autophagy

W Song, F Wang, P Lotfi, M Sardiello… - Journal of Biological …, 2014 - ASBMB
2-Hydroxypropyl-β-cyclodextrin (HPβCD) is a Food and Drug Administration-approved
excipient used to improve the stability and bioavailability of drugs. Despite its wide use as a …

Ceria nanoparticles stabilized by organic surface coatings activate the lysosome-autophagy system and enhance autophagic clearance

W Song, S Soo Lee, M Savini, L Popp, VL Colvin… - ACS …, 2014 - ACS Publications
Cerium oxide nanoparticles (nanoceria) are widely used in a variety of industrial
applications including UV filters and catalysts. The expanding commercial scale production …