Physiology and pathophysiology of human airway mucus

DB Hill, B Button, M Rubinstein… - Physiological …, 2022 - journals.physiology.org
The mucus clearance system is the dominant mechanical host defense system of the human
lung. Mucus is cleared from the lung by cilia and airflow, including both two-phase gas-liquid …

[HTML][HTML] Structure, gating, and regulation of the CFTR anion channel

L Csanády, P Vergani, DC Gadsby - Physiological reviews, 2018 - journals.physiology.org
The cystic fibrosis transmembrane conductance regulator (CFTR) belongs to the ATP
binding cassette (ABC) transporter superfamily but functions as an anion channel crucial for …

The ABC protein turned chloride channel whose failure causes cystic fibrosis

DC Gadsby, P Vergani, L Csanády - Nature, 2006 - nature.com
CFTR chloride channels are encoded by the gene mutated in patients with cystic fibrosis.
These channels belong to the superfamily of ABC transporter ATPases. ATP-driven …

Dynamic protein interaction networks and new structural paradigms in signaling

V Csizmok, AV Follis, RW Kriwacki… - Chemical …, 2016 - ACS Publications
Understanding signaling and other complex biological processes requires elucidating the
critical roles of intrinsically disordered proteins (IDPs) and regions (IDRs), which represent∼ …

[HTML][HTML] ABC transporter architecture and regulatory roles of accessory domains

E Biemans-Oldehinkel, MK Doeven, B Poolman - FEBS letters, 2006 - Elsevier
We present an overview of the architecture of ATP-binding cassette (ABC) transporters and
dissect the systems in core and accessory domains. The ABC transporter core is formed by …

Progression of cystic fibrosis lung disease from childhood to adulthood: neutrophils, neutrophil extracellular trap (NET) formation, and NET degradation

MA Khan, ZS Ali, N Sweezey, H Grasemann… - Genes, 2019 - mdpi.com
Genetic defects in cystic fibrosis (CF) transmembrane conductance regulator (CFTR) gene
cause CF. Infants with CFTR mutations show a peribronchial neutrophil infiltration prior to …

Regulatory R region of the CFTR chloride channel is a dynamic integrator of phospho-dependent intra-and intermolecular interactions

Z Bozoky, M Krzeminski… - Proceedings of the …, 2013 - National Acad Sciences
Intrinsically disordered proteins play crucial roles in regulatory processes and often function
as protein interaction hubs. Here, we present a detailed characterization of a full-length …

Cooperative assembly and misfolding of CFTR domains in vivo

K Du, GL Lukacs - Molecular biology of the cell, 2009 - Am Soc Cell Biol
The cystic fibrosis transmembrane conductance regulator (CFTR) architecture consists of
two membrane spanning domains (MSD1 and-2), two nucleotide binding domains (NBD1 …

Molecular models of the open and closed states of the whole human CFTR protein

JP Mornon, P Lehn, I Callebaut - Cellular and Molecular Life Sciences, 2009 - Springer
Cystic fibrosis transmembrane conductance regulator (CFTR), involved in cystic fibrosis
(CF), is a chloride channel belonging to the ATP-binding cassette (ABC) superfamily. Using …

Cystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis: current perspectives

BZ Schmidt, JB Haaf, T Leal, S Noel - … : advances and applications, 2016 - Taylor & Francis
Mutations of the CFTR gene cause cystic fibrosis (CF), the most common recessive
monogenic disease worldwide. These mutations alter the synthesis, processing, function, or …