Mechanisms of substrate processing during ER-associated protein degradation

JC Christianson, E Jarosch, T Sommer - Nature Reviews Molecular Cell …, 2023 - nature.com
Maintaining proteome integrity is essential for long-term viability of all organisms and is
overseen by intrinsic quality control mechanisms. The secretory pathway of eukaryotes …

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 …

Protein quality control in the secretory pathway

Z Sun, JL Brodsky - Journal of Cell Biology, 2019 - rupress.org
Protein folding is inherently error prone, especially in the endoplasmic reticulum (ER). Even
with an elaborate network of molecular chaperones and protein folding facilitators …

Ubiquitin ligases: structure, function, and regulation

N Zheng, N Shabek - Annual review of biochemistry, 2017 - annualreviews.org
Ubiquitin E3 ligases control every aspect of eukaryotic biology by promoting protein
ubiquitination and degradation. At the end of a three-enzyme cascade, ubiquitin ligases …

Order through destruction: how ER‐associated protein degradation contributes to organelle homeostasis

JC Christianson, P Carvalho - The EMBO journal, 2022 - embopress.org
The endoplasmic reticulum (ER) is a large, dynamic, and multifunctional organelle. ER
protein homeostasis is essential for the coordination of its diverse functions and depends on …

Mechanistic insights into ER-associated protein degradation

X Wu, TA Rapoport - Current opinion in cell biology, 2018 - Elsevier
Misfolded proteins of the endoplasmic reticulum (ER) are discarded by a conserved process,
called ER-associated protein degradation (ERAD). ERAD substrates are retro-translocated …

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

X Wu, M Siggel, S Ovchinnikov, W Mi, V Svetlov… - Science, 2020 - science.org
INTRODUCTION Protein homeostasis in the endoplasmic reticulum (ER) is maintained by a
quality control system. When a newly synthesized ER protein misfolds, it is ultimately …

Molecular mechanism of substrate processing by the Cdc48 ATPase complex

NO Bodnar, TA Rapoport - Cell, 2017 - cell.com
Summary The Cdc48 ATPase and its cofactors Ufd1/Npl4 (UN) extract polyubiquitinated
proteins from membranes or macromolecular complexes, but how they perform these …

Folding and misfolding of human membrane proteins in health and disease: from single molecules to cellular proteostasis

JT Marinko, H Huang, WD Penn, JA Capra… - Chemical …, 2019 - ACS Publications
Advances over the past 25 years have revealed much about how the structural properties of
membranes and associated proteins are linked to the thermodynamics and kinetics of …