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 …

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 …

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 …

Intestinal dysbiosis featuring abundance of Ruminococcus gnavus associates with allergic diseases in infants

HH Chua, HC Chou, YL Tung, BL Chiang, CC Liao… - Gastroenterology, 2018 - Elsevier
Background & Aims Dysbiosis of the intestinal microbiota has been associated with
development of allergies in infants. However, it is not clear what microbes might contribute to …

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 …

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 …

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 …

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 …