Nucleotide signalling during inflammation

M Idzko, D Ferrari, HK Eltzschig - Nature, 2014 - nature.com
Inflammatory conditions are associated with the extracellular release of nucleotides,
particularly ATP. In the extracellular compartment, ATP predominantly functions as a …

The future of cystic fibrosis treatment: from disease mechanisms to novel therapeutic approaches

SY Graeber, MA Mall - The Lancet, 2023 - thelancet.com
With the 2019 breakthrough in the development of highly effective modulator therapy
providing unprecedented clinical benefits for over 90% of patients with cystic fibrosis who …

Ion channel modulators in cystic fibrosis

M Gentzsch, MA Mall - Chest, 2018 - Elsevier
Cystic fibrosis (CF) is caused by mutations in the cystic fibrosis transmembrane conductance
regulator (CFTR) gene and remains one of the most common life-shortening genetic …

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 …

Mucus, mucins, and cystic fibrosis

CB Morrison, MR Markovetz, C Ehre - Pediatric pulmonology, 2019 - Wiley Online Library
Cystic fibrosis (CF) is both the most common and most lethal genetic disease in the
Caucasian population. CF is caused by mutations in the cystic fibrosis transmembrane …

New pharmacological approaches for cystic fibrosis: promises, progress, pitfalls

SC Bell, K De Boeck, MD Amaral - Pharmacology & therapeutics, 2015 - Elsevier
With the discovery of the CFTR gene in 1989, the search for therapies to improve the basic
defects of cystic fibrosis (CF) commenced. Pharmacological manipulation provides the …

[HTML][HTML] Targeting ion channels in cystic fibrosis

MA Mall, LJV Galietta - Journal of Cystic Fibrosis, 2015 - Elsevier
Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene cause a
characteristic defect in epithelial ion transport that plays a central role in the pathogenesis of …

Lung function is abnormal in 3-month-old infants with cystic fibrosis diagnosed by newborn screening

AF Hoo, LP Thia, A Bush, J Chudleigh, S Lum… - Thorax, 2012 - thorax.bmj.com
Background Long-term benefits of newborn screening (NBS) for cystic fibrosis (CF) have
been established with respect to nutritional status, but effects on pulmonary health remain …

Small-molecule drugs for cystic fibrosis: Where are we now?

O Laselva, L Guerra, S Castellani, M Favia… - Pulmonary …, 2022 - Elsevier
The cystic fibrosis (CF) lung disease is due to the lack/dysfunction of the CF Transmembrane
Conductance Regulator (CFTR), a chloride channel expressed by epithelial cells as the …

TMEM16A in cystic fibrosis: activating or inhibiting?

K Kunzelmann, J Ousingsawat, I Cabrita… - Frontiers in …, 2019 - frontiersin.org
The inflammatory airway disease cystic fibrosis (CF) is characterized by airway obstruction
due to mucus hypersecretion, airway plugging, and bronchoconstriction. The cystic fibrosis …