[HTML][HTML] Highlighting membrane protein structure and function: A celebration of the Protein Data Bank

F Li, PF Egea, AJ Vecchio, I Asial, M Gupta… - Journal of Biological …, 2021 - ASBMB
Biological membranes define the boundaries of cells and compartmentalize the chemical
and physical processes required for life. Many biological processes are carried out by …

[HTML][HTML] Towards next generation therapies for cystic fibrosis: folding, function and pharmacology of CFTR

SJ Bose, G Krainer, DRS Ng, M Schenkel… - Journal of Cystic …, 2020 - Elsevier
The treatment of cystic fibrosis (CF) has been transformed by orally-bioavailable small
molecule modulators of the cystic fibrosis transmembrane conductance regulator (CFTR) …

Identifying the molecular target sites for CFTR potentiators GLPG1837 and VX-770

HI Yeh, L Qiu, Y Sohma, K Conrath, X Zou… - Journal of General …, 2019 - rupress.org
The past two decades have witnessed major breakthroughs in developing compounds that
target the chloride channel CFTR for the treatment of patients with cystic fibrosis. However …

Molecular pathology of the R117H cystic fibrosis mutation is explained by loss of a hydrogen bond

MA Simon, L Csanády - Elife, 2021 - elifesciences.org
The phosphorylation-activated anion channel cystic fibrosis transmembrane conductance
regulator (CFTR) is gated by an ATP hydrolysis cycle at its two cytosolic nucleotide-binding …

ABCG: a new fold of ABC exporters and a whole new bag of riddles!

A Banerjee, A Moreno, J Pata, P Falson… - Advances in Protein …, 2021 - Elsevier
ATP-binding cassette (ABC) superfamily comprises membrane transporters that power the
active transport of substrates across biological membranes. These proteins harness the …

[HTML][HTML] Discovering the chloride pathway in the CFTR channel

B Farkas, H Tordai, R Padányi, A Tordai, J Gera… - Cellular and Molecular …, 2020 - Springer
Cystic fibrosis (CF), a lethal monogenic disease, is caused by pathogenic variants of the
CFTR chloride channel. The majority of CF mutations affect protein folding and stability …

[HTML][HTML] Activity of lumacaftor is not conserved in zebrafish Cftr bearing the major cystic fibrosis‐causing mutation

O Laselva, S Erwood, K Du, Z Ivakine… - FASEB …, 2019 - ncbi.nlm.nih.gov
F508del‐cystic fibrosis transmembrane conductance regulator (CFTR) is the major mutant
responsible for cystic fibrosis (CF). ORKAMBI®, approved for patients bearing this mutant …

[HTML][HTML] Challenges and opportunities in the development of novel antimicrobial therapeutics for cystic fibrosis

TE Barton, F Frost, JL Fothergill… - Journal of Medical …, 2022 - microbiologyresearch.org
Chronic respiratory infection is the primary driver of mortality in individuals with cystic fibrosis
(CF). Existing drug screening models utilised in preclinical antimicrobial development are …

Optimization of CFTR gating through the evolution of its extracellular loops

MA Simon, L Csanády - Journal of General Physiology, 2023 - rupress.org
CFTR chloride channel mutations cause the lethal and incurable disease cystic fibrosis (CF).
CFTR is activated by phosphorylation, and phosphorylated channels exhibit “bursting” …

CFTR regulation of aquaporin-mediated water transport

DF Carrageta, RL Bernardino, MG Alves… - Vitamins and …, 2020 - Elsevier
The cystic fibrosis transmembrane conductance regulator (CFTR) is an anion channel
responsible for the direct transport of bicarbonate and chloride. CFTR-dependent ionic …