Results 81 to 90 of about 3,624 (150)

Structural basis of ER-associated protein degradation mediated by the Hrd1 ubiquitin ligase complex

, 2020
Misfolded luminal endoplasmic reticulum (ER) proteins undergo ER-associated degradation (ERAD-L): They are retrotranslocated into the cytosol, polyubiquitinated, and degraded by the proteasome.
Hummer, G. ; https://orcid.org/, Wu, X., Mi, W., Siggel, M., Ovchinnikov, S., Svetlov, V., Nudler, E., Liao, M., Rapoport, T.   +8 more
core   +1 more source

Regulation of hepatic inclusions and fibrinogen biogenesis by SEL1L-HRD1 ERAD

Nature Communications
Impaired secretion of an essential blood coagulation factor fibrinogen leads to hepatic fibrinogen storage disease (HFSD), characterized by the presence of fibrinogen-positive inclusion bodies and hypofibrinogenemia.
Zhenfeng Song, Pattaraporn Thepsuwan, Woosuk Steve Hur, Mauricio Torres, Shuangcheng Alivia Wu, Xiaoqiong Wei, Nusrat Jahan Tushi, Juncheng Wei, Francesca Ferraresso, Adrienne W. Paton, James C. Paton, Ze Zheng, Kezhong Zhang, Deyu Fang, Christian J. Kastrup, Sunil Jaiman, Matthew James Flick, Shengyi Sun   +17 more
doaj   +1 more source

Molecular mechanism of ER stress-induced pre-emptive quality control involving association of the translocon, Derlin-1, and HRD1

Scientific Reports, 2018
The maintenance of endoplasmic reticulum (ER) homeostasis is essential for cell function. ER stress-induced pre-emptive quality control (ERpQC) helps alleviate the burden to a stressed ER by limiting further protein loading.
Hisae Kadowaki, Pasjan Satrimafitrah, Yasunari Takami, Hideki Nishitoh   +3 more
doaj   +1 more source

Regulation of leptin signaling and diet-induced obesity by SEL1L-HRD1 ER-associated degradation in POMC expressing neurons

Nature Communications
Endoplasmic reticulum (ER) homeostasis in the hypothalamus has been implicated in the pathogenesis of diet-induced obesity (DIO) and type 2 diabetes; however, the underlying molecular mechanism remain vague and debatable.
Hancheng Mao, Geun Hyang Kim, Linxiu Pan, Ling Qi   +3 more
doaj   +1 more source

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

Nature Communications
Endoplasmic reticulum-associated degradation (ERAD) plays indispensable roles in many physiological processes; however, the nature of endogenous substrates remains largely elusive.
Xiaoqiong Wei, You Lu, Liangguang Leo Lin, Chengxin Zhang, Xinxin Chen, Siwen Wang, Shuangcheng Alivia Wu, Zexin Jason Li, Yujun Quan, Shengyi Sun, Ling Qi   +10 more
doaj   +1 more source

The HRD1-SEL1L ubiquitin ligase regulates stress granule homeostasis in couple with distinctive signaling branches of ER stress

iScience
Summary: Stress granules (SGs) are membrane-less cellular compartments which are dynamically assembled via biomolecular condensation mechanism when eukaryotic cells encounter environmental stresses.
Wenbo Shi, Ran Ding, Yilin Chen, Fubo Ji, Junfang Ji, Weirui Ma, Jianping Jin   +6 more
doaj   +1 more source

Endoplasmic reticulum associated degradation preserves neurons viability by maintaining endoplasmic reticulum homeostasis

Frontiers in Neuroscience
Endoplasmic reticulum-associated degradation (ERAD) is a principal quality-control mechanism responsible for targeting misfolded ER proteins for cytosolic degradation.
Shuangchan Wu, Shuangchan Wu, Pingting Liu, Pingting Liu, Marija Cvetanovic, Marija Cvetanovic, Wensheng Lin, Wensheng Lin   +7 more
doaj   +1 more source

HRD1 and UBE2J1 target misfolded MHC class I heavy chains for endoplasmic reticulum-associated degradation

, 2011
The assembly of MHC class I molecules is governed by stringent endoplasmic reticulum (ER) quality control mechanisms. MHC class I heavy chains that fail to achieve their native conformation in complex with β2-microglobulin (β2m) and peptide are targeted ...
Burr, Marian L., Florencia Cano, Jessica M. Boname, Paul J. Lehner, Louise H. Boyle, Marian L. Burr, Svobodova, Stanislava, Lehner, Paul J., Cano, Florencia, Boyle, Louise H., Stanislava Svobodova, Boname, Jessica M.   +11 more
core   +1 more source

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

The Journal of Clinical Investigation
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 mice, including immunity, water homeostasis, and energy metabolism; however, its relevance ...
Denisa Weis, Liangguang L. Lin, Huilun H. Wang, Zexin Jason Li, Katarina Kusikova, Peter Ciznar, Hermann M. Wolf, Alexander Leiss-Piller, Zhihong Wang, Xiaoqiong Wei, Serge Weis, Katarina Skalicka, Gabriela Hrckova, Lubos Danisovic, Andrea Soltysova, Tingxuan T. Yang, René Günther Feichtinger, Johannes A. Mayr, Ling Qi   +18 more
doaj   +1 more source

USP25 and HRD1 have opposing effects on CD3

, 2013
δ protein levels and ubiquitination. A) HEK-293 cells were transfected as indicated and harvested 48 hours later. Western blots are from whole cell lysates.
Gemma Marfany (342), Jessica R. Blount (143436), Amanda Denuc (263918), Aaron A. Burr (323611), Sokol V. Todi (46174)   +4 more
core   +1 more source