Structure, gating, and regulation of the CFTR anion channel
The cystic fibrosis transmembrane conductance regulator (CFTR) belongs to the ATP
binding cassette (ABC) transporter superfamily but functions as an anion channel crucial for …
binding cassette (ABC) transporter superfamily but functions as an anion channel crucial for …
Small-molecule drugs for cystic fibrosis: Where are we now?
The cystic fibrosis (CF) lung disease is due to the lack/dysfunction of the CF Transmembrane
Conductance Regulator (CFTR), a chloride channel expressed by epithelial cells as the …
Conductance Regulator (CFTR), a chloride channel expressed by epithelial cells as the …
CFTR and lung homeostasis
JF Collawn, S Matalon - American Journal of Physiology …, 2014 - journals.physiology.org
CFTR is a cAMP-activated chloride and bicarbonate channel that is critical for lung
homeostasis. Decreases in CFTR expression have dire consequences in cystic fibrosis (CF) …
homeostasis. Decreases in CFTR expression have dire consequences in cystic fibrosis (CF) …
[HTML][HTML] Molecular basis of cystic fibrosis: from bench to bedside
MC Dechecchi, A Tamanini… - Annals of translational …, 2018 - ncbi.nlm.nih.gov
Cystic fibrosis (CF), is an autosomal recessive disease affecting different organs. The lung
disease, characterized by recurrent and chronic bacterial infection and inflammation since …
disease, characterized by recurrent and chronic bacterial infection and inflammation since …
[HTML][HTML] Correcting CFTR folding defects by small-molecule correctors to cure cystic fibrosis
M Mijnders, B Kleizen, I Braakman - Current opinion in pharmacology, 2017 - Elsevier
Highlights•Combining correctors that correct different folding defects improves
efficacy.•Complete F508del CFTR rescue likely requires full correction of NBD1.•All classes …
efficacy.•Complete F508del CFTR rescue likely requires full correction of NBD1.•All classes …
[HTML][HTML] Role of cystic fibrosis bronchial epithelium in neutrophil chemotaxis
A hallmark of cystic fibrosis (CF) chronic respiratory disease is an extensive neutrophil
infiltrate in the mucosa filling the bronchial lumen, starting early in life for CF infants. The …
infiltrate in the mucosa filling the bronchial lumen, starting early in life for CF infants. The …
Corrector VX-809 promotes interactions between cytoplasmic loop one and the first nucleotide-binding domain of CFTR
TW Loo, DM Clarke - Biochemical Pharmacology, 2017 - Elsevier
A large number of correctors have been identified that can partially repair defects in folding,
stability and trafficking of CFTR processing mutants that cause cystic fibrosis (CF). The best …
stability and trafficking of CFTR processing mutants that cause cystic fibrosis (CF). The best …
[HTML][HTML] Dysfunctional inflammation in cystic fibrosis airways: from mechanisms to novel therapeutic approaches
A Ghigo, G Prono, E Riccardi, V De Rose - International Journal of …, 2021 - mdpi.com
Cystic fibrosis (CF) is an inherited disorder caused by mutations in the gene encoding for the
cystic fibrosis transmembrane conductance regulator (CFTR) protein, an ATP-gated chloride …
cystic fibrosis transmembrane conductance regulator (CFTR) protein, an ATP-gated chloride …
[HTML][HTML] Phenotyping Rare CFTR Mutations Reveal Functional Expression Defects Restored by TRIKAFTATM
The rare Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) mutations, c.
1826A> G (H609R) and c. 3067_3072delATAGTG (I1023_V1024del), are associated with …
1826A> G (H609R) and c. 3067_3072delATAGTG (I1023_V1024del), are associated with …
Correctors of the major cystic fibrosis mutant interact through membrane-spanning domains
The most common cystic fibrosis causing mutation is deletion of phenylalanine at position
508 (F508del), a mutation that leads to protein misassembly with defective processing …
508 (F508del), a mutation that leads to protein misassembly with defective processing …