The future of cystic fibrosis care: a global perspective

SC Bell, MA Mall, H Gutierrez, M Macek… - The Lancet …, 2020 - thelancet.com
Executive summary The past six decades have seen remarkable improvements in health
outcomes for people with cystic fibrosis, which was once a fatal disease of infants and young …

Advances in anion transport and supramolecular medicinal chemistry

JT Davis, PA Gale, R Quesada - Chemical Society Reviews, 2020 - pubs.rsc.org
Advances in anion transport by synthetic supramolecular systems are discussed in this
article. Developments in the design of discrete molecular carriers for anions and …

Pathophysiology and management of pulmonary infections in cystic fibrosis

RL Gibson, JL Burns, BW Ramsey - American journal of respiratory …, 2003 - atsjournals.org
This comprehensive State of the Art review summarizes the current published knowledge
base regarding the pathophysiology and microbiology of pulmonary disease in cystic …

∆ F508 CFTR interactome remodelling promotes rescue of cystic fibrosis

S Pankow, C Bamberger, D Calzolari… - Nature, 2015 - nature.com
Deletion of phenylalanine 508 of the cystic fibrosis transmembrane conductance regulator (∆
F508 CFTR) is the major cause of cystic fibrosis, one of the most common inherited …

CFTR: cystic fibrosis and beyond

MA Mall, D Hartl - 2014 - Eur Respiratory Soc
Cystic fibrosis (CF) remains the most common fatal hereditary lung disease. The discovery of
the cystic fibrosis transmembrane conductance regulator (CFTR) gene 25 years ago set the …

CFTR function and prospects for therapy

JR Riordan - Annu. Rev. Biochem., 2008 - annualreviews.org
Mutations in the gene coding for the cystic fibrosis transmembrane conductance regulator
(CFTR) epithelial anion channel cause cystic fibrosis (CF). The multidomain integral …

Thiazolidinone CFTR inhibitor identified by high-throughput screening blocks cholera toxin–induced intestinal fluid secretion

T Ma, JR Thiagarajah, H Yang… - The Journal of …, 2002 - Am Soc Clin Investig
Secretory diarrhea is the leading cause of infant death in developing countries and a major
cause of morbidity in adults. The cystic fibrosis transmembrane conductance regulator …

Chloride channels as drug targets

AS Verkman, LJV Galietta - Nature reviews Drug discovery, 2009 - nature.com
Chloride channels represent a relatively under-explored target class for drug discovery as
elucidation of their identity and physiological roles has lagged behind that of many other …

[HTML][HTML] Animal and cell culture models for cystic fibrosis: which model is right for your application?

A McCarron, D Parsons, M Donnelley - The American journal of pathology, 2021 - Elsevier
Over the past 30 years, a range of cystic fibrosis (CF) animal models have been generated
for research purposes. Different species, including mice, rats, ferrets, rabbits, pigs, sheep …

Cystic fibrosis transmembrane regulator correctors and potentiators

SM Rowe, AS Verkman - Cold Spring …, 2013 - perspectivesinmedicine.cshlp.org
Cystic fibrosis (CF) is caused by loss-of-function mutations in the CF transmembrane
conductance regulator (CFTR) protein, a cAMP-regulated anion channel expressed …