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 …

[HTML][HTML] Upstream and downstream regulators of Klotho expression in chronic kidney disease

SS Li, M Sheng, ZY Sun, Y Liang, LX Yu, QF Liu - Metabolism, 2023 - Elsevier
Klotho is a critical protein that protects the kidney. Klotho is severely downregulated in
chronic kidney disease (CKD), and its deficiency is implicated in the pathogenesis and …

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 …

Proteomic screens of SEL1L-HRD1 ER-associated degradation substrates reveal its role in glycosylphosphatidylinositol-anchored protein biogenesis

X Wei, Y Lu, LL Lin, C Zhang, X Chen, S Wang… - Nature …, 2024 - nature.com
Endoplasmic reticulum-associated degradation (ERAD) plays indispensable roles in many
physiological processes; however, the nature of endogenous substrates remains largely …

[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 …

SEL1L-HRD1 interaction is required to form a functional HRD1 ERAD complex

LL Lin, HH Wang, B Pederson, X Wei, M Torres… - nature …, 2024 - nature.com
Abstract The SEL1L-HRD1 protein complex represents the most conserved branch of
endoplasmic reticulum (ER)-associated degradation (ERAD). Despite recent advances in …

Dihydromyricetin attenuates cisplatin-induced acute kidney injury by reducing oxidative stress, inflammation and ferroptosis

Z Xu, M Zhang, W Wang, S Zhou, M Yu, X Qiu… - Toxicology and Applied …, 2023 - Elsevier
Background Cisplatin is effective against various types of cancers. However, its clinical
application is limited owing to its adverse effects, especially acute kidney injury (AKI) …

Hepatic SEL1L-HRD1 ER-associated degradation regulates systemic iron homeostasis via ceruloplasmin

P Thepsuwan, A Bhattacharya… - Proceedings of the …, 2023 - National Acad Sciences
Iron homeostasis is critical for cellular and organismal function and is tightly regulated to
prevent toxicity or anemia due to iron excess or deficiency, respectively. However …