Physiology and pathophysiology of human airway mucus
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 …
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
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 …
The ABC protein turned chloride channel whose failure causes cystic fibrosis
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 …
These channels belong to the superfamily of ABC transporter ATPases. ATP-driven …
Dynamic protein interaction networks and new structural paradigms in signaling
Understanding signaling and other complex biological processes requires elucidating the
critical roles of intrinsically disordered proteins (IDPs) and regions (IDRs), which represent∼ …
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 …
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 …
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 …
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 …
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 …
(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 …
monogenic disease worldwide. These mutations alter the synthesis, processing, function, or …