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 …

Mechanisms and regulation of cholesterol homeostasis

J Luo, H Yang, BL Song - Nature reviews Molecular cell biology, 2020 - nature.com
Cholesterol homeostasis is vital for proper cellular and systemic functions. Disturbed
cholesterol balance underlies not only cardiovascular disease but also an increasing …

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 …

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 …

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 …

Characterisation of molecular motions in cryo-EM single-particle data by multi-body refinement in RELION

T Nakane, D Kimanius, E Lindahl, SHW Scheres - elife, 2018 - elifesciences.org
Macromolecular complexes that exhibit continuous forms of structural flexibility pose a
challenge for many existing tools in cryo-EM single-particle analysis. We describe a new …

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 …

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 …

Dynamic regulation of the 26S proteasome: from synthesis to degradation

RS Marshall, RD Vierstra - Frontiers in Molecular Biosciences, 2019 - frontiersin.org
All eukaryotes rely on selective proteolysis to control the abundance of key regulatory
proteins and maintain a healthy and properly functioning proteome. Most of this turnover is …