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 …
caused by loss-of-function mutations of the CF transmembrane conductance regulator …
NEDD4-2 (NEDD4L): the ubiquitin ligase for multiple membrane proteins
P Goel, JA Manning, S Kumar - Gene, 2015 - Elsevier
Abstract NEDD4-2 (also known as NEDD4L, neural precursor cell expressed
developmentally down-regulated 4-like) is a ubiquitin protein ligase of the Nedd4 family …
developmentally down-regulated 4-like) is a ubiquitin protein ligase of the Nedd4 family …
Calcium/calmodulin-dependent protein kinase kinase 2 regulates macrophage-mediated inflammatory responses
L Racioppi, PK Noeldner, F Lin, S Arvai… - Journal of Biological …, 2012 - ASBMB
Calcium/calmodulin-dependent kinase kinase 2 (CaMKK2) plays a key role in regulating
food intake and energy expenditure at least in part by its actions in hypothalamic neurons …
food intake and energy expenditure at least in part by its actions in hypothalamic neurons …
Chaperone-independent peripheral quality control of CFTR by RFFL E3 ligase
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 …
including ΔF508-CFTR, the most common CFTR mutant in cystic fibrosis (CF), from the …
Endocytosis: a turnover mechanism controlling ion channel function
I Estadella, O Pedrós-Gámez, M Colomer-Molera… - Cells, 2020 - mdpi.com
Ion channels (IChs) are transmembrane proteins that selectively drive ions across
membranes. The function of IChs partially relies on their abundance and proper location in …
membranes. The function of IChs partially relies on their abundance and proper location in …
Tgf-β1 inhibits Cftr biogenesis and prevents functional rescue of ΔF508-Cftr in primary differentiated human bronchial epithelial cells
SM Snodgrass, KM Cihil, PK Cornuet, MM Myerburg… - PloS one, 2013 - journals.plos.org
CFTR is an integral transmembrane glycoprotein and a cAMP-activated Cl− channel.
Mutations in the CFTR gene lead to Cystic Fibrosis (CF)–an autosomal recessive disease …
Mutations in the CFTR gene lead to Cystic Fibrosis (CF)–an autosomal recessive disease …
Ubiquitination and degradation of CFTR by the E3 ubiquitin ligase MARCH2 through its association with adaptor proteins CAL and STX6
J Cheng, W Guggino - PLoS One, 2013 - journals.plos.org
Golgi-localized cystic fibrosis transmembrane conductance regulator (CFTR)-associated
ligand (CAL) and syntaxin 6 (STX6) regulate the abundance of mature, post-ER CFTR by …
ligand (CAL) and syntaxin 6 (STX6) regulate the abundance of mature, post-ER CFTR by …
Peripheral protein quality control as a novel drug target for CFTR stabilizer
R Fukuda, T Okiyoneda - Frontiers in Pharmacology, 2018 - frontiersin.org
Conformationally defective cystic fibrosis transmembrane conductance regulator (CFTR)
including rescued ΔF508-CFTR is rapidly eliminated from the plasma membrane (PM) even …
including rescued ΔF508-CFTR is rapidly eliminated from the plasma membrane (PM) even …
Cigarette smoke exposure reveals a novel role for the MEK/ERK1/2 MAPK pathway in regulation of CFTR
Background Cystic fibrosis transmembrane conductance regulator plays a key role in
maintenance of lung fluid homeostasis. Cigarette smoke decreases CFTR expression in the …
maintenance of lung fluid homeostasis. Cigarette smoke decreases CFTR expression in the …
Effects of Pseudomonas aeruginosa on CFTR chloride secretion and the host immune response
BA Stanton - American Journal of Physiology-Cell …, 2017 - journals.physiology.org
In the healthy lung the opportunistic pathogen, Pseudomonas aeruginosa, is rapidly
eliminated by mucociliary clearance, a process that is dependent on the activity of the CFTR …
eliminated by mucociliary clearance, a process that is dependent on the activity of the CFTR …