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 …

CFTR: folding, misfolding and correcting the ΔF508 conformational defect

GL Lukacs, AS Verkman - Trends in molecular medicine, 2012 - cell.com
Cystic fibrosis (CF), the most common lethal genetic disease in the Caucasian population, is
caused by loss-of-function mutations of the CF transmembrane conductance regulator …

Identification of Downstream Components of Ubiquitin-Conjugating Enzyme PHOSPHATE2 by Quantitative Membrane Proteomics in Arabidopsis Roots

TK Huang, CL Han, SI Lin, YJ Chen, YC Tsai… - The Plant …, 2013 - academic.oup.com
MicroRNA399-mediated regulation of the ubiquitin-conjugating enzyme
UBC24/PHOSPHATE2 (PHO2) is crucial for Pi acquisition and translocation in plants …

Ubiquitin-dependent sorting in endocytosis

RC Piper, I Dikic, GL Lukacs - Cold Spring Harbor …, 2014 - cshperspectives.cshlp.org
When ubiquitin (Ub) is attached to membrane proteins on the plasma membrane, it directs
them through a series of sorting steps that culminate in their delivery to the lumen of the …

Structure-guided combination therapy to potently improve the function of mutant CFTRs

G Veit, H Xu, E Dreano, RG Avramescu, M Bagdany… - Nature medicine, 2018 - nature.com
Available corrector drugs are unable to effectively rescue the folding defects of CFTR-ΔF508
(or CFTR-F508del), the most common disease-causing mutation of the cystic fibrosis …

Cell surface thermal proteome profiling tracks perturbations and drug targets on the plasma membrane

M Kalxdorf, I Günthner, I Becher, N Kurzawa… - Nature …, 2021 - nature.com
Numerous drugs and endogenous ligands bind to cell surface receptors leading to
modulation of downstream signaling cascades and frequently to adaptation of the plasma …

CFTR: a new horizon in the pathomechanism and treatment of pancreatitis

P Hegyi, M Wilschanski, S Muallem, GL Lukacs… - Reviews of Physiology …, 2016 - Springer
Cystic fibrosis transmembrane conductance regulator (CFTR) is an ion channel that
conducts chloride and bicarbonate ions across epithelial cell membranes. Mutations in the …

[HTML][HTML] Quality control and fate determination of Hsp90 client proteins

MA Theodoraki, AJ Caplan - … et Biophysica Acta (BBA)-Molecular Cell …, 2012 - Elsevier
Quality control processes regulate the proteome by determining whether a protein is to be
folded or degraded. Hsp90 is a hub in the network of molecular chaperones that maintain …

Chaperone-independent peripheral quality control of CFTR by RFFL E3 ligase

T Okiyoneda, G Veit, R Sakai, M Aki, T Fujihara… - Developmental cell, 2018 - cell.com
The peripheral protein quality control (QC) system removes non-native membrane proteins,
including ΔF508-CFTR, the most common CFTR mutant in cystic fibrosis (CF), from the …

Targeting autophagy as a novel strategy for facilitating the therapeutic action of potentiators on ΔF508 cystic fibrosis transmembrane conductance regulator

A Luciani, VR Villella, S Esposito, M Gavina, I Russo… - Autophagy, 2012 - Taylor & Francis
Channel activators (potentiators) of cystic fibrosis (CF) transmembrane conductance
regulator (CFTR), can be used for the treatment of the small subset of CF patients that carry …