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 …

The critical role of GRP78 in physiologic and pathologic stress

KT Pfaffenbach, AS Lee - Current opinion in cell biology, 2011 - Elsevier
GRP78 is a major endoplasmic reticulum chaperone as well as a master regulator of the
unfolded protein response. In addition to playing an essential role in early embryonic …

[HTML][HTML] 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 …

The metabolic ER stress sensor IRE1α suppresses alternative activation of macrophages and impairs energy expenditure in obesity

B Shan, X Wang, Y Wu, C Xu, Z Xia, J Dai, M Shao… - Nature …, 2017 - nature.com
Obesity is associated with metabolic inflammation and endoplasmic reticulum (ER) stress,
both of which promote metabolic disease progression. Adipose tissue macrophages (ATMs) …

[HTML][HTML] Thbs1 induces lethal cardiac atrophy through PERK-ATF4 regulated autophagy

D Vanhoutte, TG Schips, A Vo, KM Grimes… - Nature …, 2021 - nature.com
The thrombospondin (Thbs) family of secreted matricellular proteins are stress-and injury-
induced mediators of cellular attachment dynamics and extracellular matrix protein …

[HTML][HTML] A J-protein co-chaperone recruits BiP to monomerize IRE1 and repress the unfolded protein response

N Amin-Wetzel, RA Saunders, MJ Kamphuis, C Rato… - Cell, 2017 - cell.com
When unfolded proteins accumulate in the endoplasmic reticulum (ER), the unfolded protein
response (UPR) increases ER-protein-folding capacity to restore protein-folding …

[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 …

[HTML][HTML] Pharmacological targeting of MYC-regulated IRE1/XBP1 pathway suppresses MYC-driven breast cancer

NA Zhao, J Cao, L Xu, Q Tang… - The Journal of …, 2018 - Am Soc Clin Investig
The unfolded protein response (UPR) is a cellular homeostatic mechanism that is activated
in many human cancers and plays pivotal roles in tumor progression and therapy resistance …

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 …