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 …

Chaperoning endoplasmic reticulum–associated degradation (ERAD) and protein conformational diseases

PG Needham, CJ Guerriero… - Cold Spring Harbor …, 2019 - cshperspectives.cshlp.org
Misfolded proteins compromise cellular homeostasis. This is especially problematic in the
endoplasmic reticulum (ER), which is a high-capacity protein-folding compartment and …

Multiplexed measurement of variant abundance and activity reveals VKOR topology, active site and human variant impact

MA Chiasson, NJ Rollins, JJ Stephany, KA Sitko… - elife, 2020 - elifesciences.org
Vitamin K epoxide reductase (VKOR) drives the vitamin K cycle, activating vitamin K-
dependent blood clotting factors. VKOR is also the target of the widely used anticoagulant …

Doa10 is a membrane protein retrotranslocase in ER-associated protein degradation

CC Schmidt, V Vasic, A Stein - Elife, 2020 - elifesciences.org
In endoplasmic reticulum-associated protein degradation (ERAD), membrane proteins are
ubiquitinated, extracted from the membrane, and degraded by the proteasome. The cytosolic …

HERC3 facilitates ERAD of select membrane proteins by recognizing membrane-spanning domains

Y Kamada, Y Ohnishi, C Nakashima, A Fujii… - Journal of Cell …, 2024 - rupress.org
Aberrant proteins located in the endoplasmic reticulum (ER) undergo rapid ubiquitination by
multiple ubiquitin (Ub) E3 ligases and are retrotranslocated to the cytosol as part of the ER …

The Dsc ubiquitin ligase complex identifies transmembrane degrons to degrade orphaned proteins at the Golgi

Y Weyer, SI Schwabl, X Tang, A Purwar… - Nature …, 2024 - nature.com
The Golgi apparatus is essential for protein sorting, yet its quality control mechanisms are
poorly understood. Here we show that the Dsc ubiquitin ligase complex uses its rhomboid …

Co-expression of squalene epoxidases with triterpene cyclases boosts production of triterpenoids in plants and yeast

L Dong, J Pollier, JE Bassard, G Ntallas, A Almeida… - Metabolic …, 2018 - Elsevier
Triterpene cyclases catalyze the first committed step in triterpene biosynthesis, by forming
mono-to pentacyclic backbone structures from oxygenated C30 isoprenoid precursors …

The expression system influences stability, maturation efficiency, and oligomeric properties of the potassium-chloride co-transporter KCC2

M Kok, K Hartnett-Scott, CL Happe… - Neurochemistry …, 2024 - Elsevier
The neuron-specific K+/Cl− co-transporter 2, KCC2, which is critical for brain development,
regulates γ-aminobutyric acid-dependent inhibitory neurotransmission. Consistent with its …

Identification of polycystin 2 missense mutants targeted for endoplasmic reticulum-associated degradation

CJ Guerriero, MD Carattino, KG Sharp… - … of Physiology-Cell …, 2025 - journals.physiology.org
Autosomal dominant polycystic kidney disease (ADPKD) is a common genetic disorder
leading to end-stage renal disease. ADPKD arises from mutations in the PKD1 and PKD2 …

Lhs1 dependent ERAD is determined by transmembrane domain context

M Sukhoplyasova, AM Keith, EM Perrault… - Biochemical …, 2023 - portlandpress.com
Transmembrane proteins have unique requirements to fold and integrate into the
endoplasmic reticulum (ER) membrane. Most notably, transmembrane proteins must fold in …