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 …

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 …

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 …

Maintenance of organellar protein homeostasis by ER-associated degradation and related mechanisms

MK Lemberg, K Strisovsky - Molecular Cell, 2021 - cell.com
Protein homeostasis mechanisms are fundamentally important to match cellular needs and
to counteract stress conditions. A fundamental challenge is to understand how defective …

HBV genotype: a significant risk factor in determining which patients with chronic HBV infection should undergo surveillance for HCC: the hepatitis B Alaska study

BJ McMahon, LD Nolen, M Snowball, C Homan… - Hepatology, 2021 - journals.lww.com
Conclusions These data indicate that RNF5 inhibits NASH progression by targeting HRD1 in
the ubiquitin‐mediated proteasomal pathway. Targeting the RNF5–HRD1 axis may provide …

Accumulated precursors of specific GPI-anchored proteins upregulate GPI biosynthesis with ARV1

YS Liu, Y Wang, X Zhou, L Zhang, G Yang… - Journal of Cell …, 2023 - rupress.org
We previously reported that glycosylphosphatidylinositol (GPI) biosynthesis is upregulated
when endoplasmic reticulum–associated degradation (ERAD) is defective; however, the …

Highly efficient expression and secretion of human lysozyme using multiple strategies in Pichia pastoris

Y Wang, B Wang, Y Gao, H Nakanishi… - Biotechnology …, 2023 - Wiley Online Library
Background Human lysozyme (hLYZ), an emerging antibacterial agent, has extensive
application in the food and pharmaceutical industries. However, the source of hLYZ is …

Hrd1 forms the retrotranslocation pore regulated by auto-ubiquitination and binding of misfolded proteins

V Vasic, N Denkert, CC Schmidt, D Riedel, A Stein… - Nature Cell …, 2020 - nature.com
During endoplasmic-reticulum-associated protein degradation (ERAD), misfolded proteins
are polyubiquitinated, extracted from the ER membrane and degraded by the proteasome …

The ERAD system is restricted by elevated ceramides

J Hwang, BG Peterson, J Knupp, RD Baldridge - Science Advances, 2023 - science.org
Misfolded proteins in the endoplasmic reticulum (ER) are removed through a process known
as ER-associated degradation (ERAD). ERAD occurs through an integral membrane protein …