Endoplasmic reticulum–associated protein degradation

L Krshnan, ML van de Weijer… - Cold Spring Harbor …, 2022 - cshperspectives.cshlp.org
Misfolded, potentially toxic proteins in the lumen and membrane of the endoplasmic
reticulum (ER) are eliminated by proteasomes in the cytosol through ER-associated …

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 …

SEL1L–HRD1 endoplasmic reticulum-associated degradation controls STING-mediated innate immunity by limiting the size of the activable STING pool

Y Ji, Y Luo, Y Wu, Y Sun, L Zhao, Z Xue, M Sun… - Nature Cell …, 2023 - nature.com
Stimulator of interferon genes (STING) orchestrates the production of proinflammatory
cytokines in response to cytosolic double-stranded DNA; however, the pathophysiological …

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 …

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 …

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 …

Endoplasmic reticulum–associated degradation regulates mitochondrial dynamics in brown adipocytes

Z Zhou, M Torres, H Sha, CJ Halbrook… - Science, 2020 - science.org
The endoplasmic reticulum (ER) engages mitochondria at specialized ER domains known
as mitochondria-associated membranes (MAMs). Here, we used three-dimensional high …

Proteolysis targeting chimeras (PROTACs) are emerging therapeutics for hematologic malignancies

Y He, S Khan, Z Huo, D Lv, X Zhang, X Liu… - Journal of Hematology & …, 2020 - Springer
Proteolysis targeting chimeras (PROTACs) are heterobifunctional small molecules that
utilize the ubiquitin proteasome system (UPS) to degrade proteins of interest (POI) …

[HTML][HTML] Muscle-specific ER-associated degradation maintains postnatal muscle hypertrophy and systemic energy metabolism

B Abdon, Y Liang, D da Luz Scheffer, M Torres… - JCI insight, 2023 - ncbi.nlm.nih.gov
The growth of skeletal muscle relies on a delicate equilibrium between protein synthesis and
degradation; however, how proteostasis is managed in the endoplasmic reticulum (ER) is …

ER-associated degradation in health and disease–from substrate to organism

A Bhattacharya, L Qi - Journal of Cell Science, 2019 - journals.biologists.com
The recent literature has revolutionized our view on the vital importance of endoplasmic
reticulum (ER)-associated degradation (ERAD) in health and disease. Suppressor/enhancer …