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 …

Role of epithelial sodium channels in the regulation of lung fluid homeostasis

S Matalon, R Bartoszewski… - American Journal of …, 2015 - journals.physiology.org
In utero, fetal lung epithelial cells actively secrete Cl− ions into the lung air spaces while Na+
ions follow passively to maintain electroneutrality. This process, driven by an …

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 …

[HTML][HTML] Niclosamide repurposed for the treatment of inflammatory airway disease

I Cabrita, R Benedetto, R Schreiber, K Kunzelmann - JCI insight, 2019 - ncbi.nlm.nih.gov
Inflammatory airway diseases, such as asthma, cystic fibrosis (CF), and chronic obstructive
pulmonary disease (COPD), are characterized by mucus hypersecretion and airway …

CFTR and Anoctamin 1 (ANO1) contribute to cAMP amplified exocytosis and insulin secretion in human and murine pancreatic beta-cells

A Edlund, JLS Esguerra, A Wendt… - BMC medicine, 2014 - Springer
Background Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR)
gene lead to the disease cystic fibrosis (CF). Although patients with CF often have …

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 …

TMEM16A mediates mucus production in human airway epithelial cells

I Cabrita, R Benedetto, P Wanitchakool… - American journal of …, 2021 - atsjournals.org
TMEM16A is a Ca2+-activated chloride channel that was shown to enhance production and
secretion of mucus in inflamed airways. It is, however, not clear whether TMEM16A directly …

Ano1, a Ca2+‐activated Cl channel, coordinates contractility in mouse intestine by Ca2+ transient coordination between interstitial cells of Cajal

RD Singh, SJ Gibbons… - The Journal of …, 2014 - Wiley Online Library
Key points Ano1, a Ca2+‐activated Cl− channel, is expressed in interstitial cells of Cajal
(ICC) throughout the gut. We report here that it is required to maintain coordinated Ca2+ …

Molecular functions of anoctamin 6 (TMEM16F): a chloride channel, cation channel, or phospholipid scramblase?

K Kunzelmann, B Nilius, G Owsianik… - … -European Journal of …, 2014 - Springer
Abstract Anoctamin 6 (Ano6; TMEM16F gene) is a ubiquitous protein; the expression of
which is defective in patients with Scott syndrome, an inherited bleeding disorder based on …

TMEM16A/F support exocytosis but do not inhibit Notch-mediated goblet cell metaplasia of BCi-NS1. 1 human airway epithelium

R Centeio, I Cabrita, R Schreiber… - Frontiers in …, 2023 - frontiersin.org
Cl− channels such as the Ca2+ activated Cl− channel TMEM16A and the Cl− permeable
phospholipid scramblase TMEM16F may affect the intracellular Cl− concentration ([Cl−] i) …