Quality control in the endoplasmic reticulum: crosstalk between ERAD and UPR pathways

J Hwang, L Qi - Trends in biochemical sciences, 2018 - cell.com
Endoplasmic reticulum (ER)-associated degradation (ERAD) and the unfolded protein
response (UPR) are two key quality-control machineries in the cell. ERAD is responsible for …

ER-associated degradation: Protein quality control and beyond

A Ruggiano, O Foresti, P Carvalho - Journal of Cell Biology, 2014 - rupress.org
Even with the assistance of many cellular factors, a significant fraction of newly synthesized
proteins ends up misfolded. Cells evolved protein quality control systems to ensure that …

Hrd1 suppresses Nrf2-mediated cellular protection during liver cirrhosis

T Wu, F Zhao, B Gao, C Tan, N Yagishita… - Genes & …, 2014 - genesdev.cshlp.org
Increased endoplasmic reticulum (ER) stress and reactive oxygen species (ROS) are the
salient features of end-stage liver diseases. Using liver tissues from liver cirrhosis patients …

New insights into the physiological role of endoplasmic reticulum-associated degradation

L Qi, B Tsai, P Arvan - Trends in cell biology, 2017 - cell.com
Many human diseases are associated with mutations causing protein misfolding and
aggregation in the endoplasmic reticulum (ER). ER-associated degradation (ERAD) is a …

[HTML][HTML] Biallelic Cys141Tyr variant of SEL1L is associated with neurodevelopmental disorders, agammaglobulinemia, and premature death

D Weis, LL Lin, HH Wang, ZJ Li… - The Journal of …, 2024 - Am Soc Clin Investig
Suppressor of lin-12-like–HMG-CoA reductase degradation 1 (SEL1L-HRD1) ER-
associated degradation (ERAD) plays a critical role in many physiological processes in …

[HTML][HTML] Hypomorphic variants of SEL1L-HRD1 ER-associated degradation are associated with neurodevelopmental disorders

HH Wang, LL Lin, ZJ Li, X Wei… - The Journal of …, 2024 - Am Soc Clin Investig
Recent studies using cell type–specific knockout mouse models have improved our
understanding of the pathophysiological relevance of suppressor of lin-12-like–HMG-CoA …

IRE1α is an endogenous substrate of endoplasmic-reticulum-associated degradation

S Sun, G Shi, H Sha, Y Ji, X Han, X Shu, H Ma… - Nature cell …, 2015 - nature.com
Endoplasmic reticulum (ER)-associated degradation (ERAD) represents a principle quality
control mechanism to clear misfolded proteins in the ER; however, its physiological …

The unfolded protein response, degradation from the endoplasmic reticulum, and cancer

YC Tsai, AM Weissman - Genes & cancer, 2010 - journals.sagepub.com
The endoplasmic reticulum (ER) is an essential organelle involved in many cellular
functions including protein folding and secretion, lipid biosynthesis, and calcium …

Interaction mapping of endoplasmic reticulum ubiquitin ligases identifies modulators of innate immune signalling

EJ Fenech, F Lari, PD Charles, R Fischer… - Elife, 2020 - elifesciences.org
Ubiquitin ligases (E3s) embedded in the endoplasmic reticulum (ER) membrane regulate
essential cellular activities including protein quality control, calcium flux, and sterol …

Sel1L is indispensable for mammalian endoplasmic reticulum-associated degradation, endoplasmic reticulum homeostasis, and survival

S Sun, G Shi, X Han, AB Francisco… - Proceedings of the …, 2014 - National Acad Sciences
Suppressor/Enhancer of Lin-12-like (Sel1L) is an adaptor protein for the E3 ligase
hydroxymethylglutaryl reductase degradation protein 1 (Hrd1) involved in endoplasmic …