CFTR modulators: the changing face of cystic fibrosis in the era of precision medicine

M Lopes-Pacheco - Frontiers in pharmacology, 2020 - frontiersin.org
Cystic fibrosis (CF) is a lethal inherited disease caused by mutations in the CF
transmembrane conductance regulator (CFTR) gene, which result in impairment of CFTR …

Structure, gating, and regulation of the CFTR anion channel

L Csanády, P Vergani, DC Gadsby - Physiological reviews, 2019 - 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 …

CFTR modulators: shedding light on precision medicine for cystic fibrosis

M Lopes-Pacheco - Frontiers in pharmacology, 2016 - frontiersin.org
Cystic fibrosis (CF) is the most common life-threatening monogenic disease afflicting
Caucasian people. It affects the respiratory, gastrointestinal, glandular and reproductive …

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 …

PKC regulation of ion channels: The involvement of PIP2

KD Gada, DE Logothetis - Journal of Biological Chemistry, 2022 - ASBMB
Ion channels are integral membrane proteins whose gating has been increasingly shown to
depend on the presence of the low-abundance membrane phospholipid …

Pharmacological modulation of ion channels for the treatment of cystic fibrosis

MC Pinto, IAL Silva, MF Figueira… - Journal of …, 2021 - Taylor & Francis
Cystic fibrosis (CF) is a life-shortening monogenic disease caused by mutations in the gene
encoding the CF transmembrane conductance regulator (CFTR) protein, an anion channel …

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 …

Pathogenic relationships in cystic fibrosis and renal diseases: CFTR, SLC26A9 and anoctamins

K Kunzelmann, J Ousingsawat, A Kraus… - International journal of …, 2023 - mdpi.com
The Cl−-transporting proteins CFTR, SLC26A9, and anoctamin (ANO1; ANO6) appear to
have more in common than initially suspected, as they all participate in the pathogenic …

Anoctamins

K Kunzelmann, Y Tian, JR Martins, D Faria… - … -European Journal of …, 2011 - Springer
Abstract Endogenous Ca 2+-activated Cl− channels (CaCC) demonstrate biophysical and
pharmacological properties that are well represented in cells overexpressing anoctamin 1 …