Structural mechanisms of CFTR function and dysfunction

TC Hwang, JT Yeh, J Zhang, YC Yu, HI Yeh… - Journal of General …, 2018 - rupress.org
Cystic fibrosis (CF) transmembrane conductance regulator (CFTR) chloride channel plays a
critical role in regulating transepithelial movement of water and electrolyte in exocrine …

CFTR structure and cystic fibrosis

N Cant, N Pollock, RC Ford - The international journal of biochemistry & …, 2014 - Elsevier
CFTR (cystic fibrosis transmembrane conductance regulator) is a member of the ATP-
binding cassette family of membrane proteins. Although almost all members of this family …

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 …

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 …

Tetrameric Orai1 is a teardrop-shaped molecule with a long, tapered cytoplasmic domain

Y Maruyama, T Ogura, K Mio, K Kato, T Kaneko… - Journal of Biological …, 2009 - ASBMB
The Ca 2+ release-activated Ca 2+ channel is a principal regulator of intracellular Ca 2+
rise, which conducts various biological functions, including immune responses. This …

Cystic fibrosis transmembrane conductance regulator (ABCC7) structure

JF Hunt, C Wang, RC Ford - Cold Spring …, 2013 - perspectivesinmedicine.cshlp.org
Structural studies of the cystic fibrosis transmembrane conductance regulator (CFTR) are
reviewed. Like many membrane proteins, full-length CFTR has proven to be difficult to …

Molecular modelling and molecular dynamics of CFTR

I Callebaut, B Hoffmann, P Lehn, JP Mornon - Cellular and Molecular Life …, 2017 - Springer
The cystic fibrosis transmembrane conductance regulator (CFTR) protein is a member of the
ATP-binding cassette (ABC) transporter superfamily that functions as an ATP-gated channel …

Functional architecture of the CFTR chloride channel

P Linsdell - Molecular membrane biology, 2014 - Taylor & Francis
Cystic fibrosis is caused by mutations in the cystic fibrosis transmembrane conductance
regulator (CFTR), a member of the ATP-binding cassette (ABC) family of membrane …

On the mechanism of gating defects caused by the R117H mutation in cystic fibrosis transmembrane conductance regulator

YC Yu, Y Sohma, TC Hwang - The Journal of physiology, 2016 - Wiley Online Library
Key points Two functional abnormalities of cystic fibrosis transmembrane conductance
regulator (CFTR), a 25% reduction of the single‐channel conductance (g) and a∼ 13‐fold …

Regulation of conductance by the number of fixed positive charges in the intracellular vestibule of the CFTR chloride channel pore

JJ Zhou, MS Li, J Qi, P Linsdell - Journal of General Physiology, 2010 - rupress.org
Rapid chloride permeation through the cystic fibrosis transmembrane conductance regulator
(CFTR) Cl− channel is dependent on the presence of fixed positive charges in the …