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 …

Contribution of anoctamins to cell survival and cell death

K Kunzelmann, J Ousingsawat, R Benedetto, I Cabrita… - Cancers, 2019 - mdpi.com
Before anoctamins (TMEM16 proteins) were identified as a family of Ca2+-activated chloride
channels and phospholipid scramblases, the founding member anoctamin 1 (ANO1 …

Cyst growth in ADPKD is prevented by pharmacological and genetic inhibition of TMEM16A in vivo

I Cabrita, A Kraus, JK Scholz, K Skoczynski… - Nature …, 2020 - nature.com
In autosomal dominant polycystic kidney disease (ADPKD) multiple bilateral renal cysts
gradually enlarge, leading to a decline in renal function. Transepithelial chloride secretion …

Broadening the clinical spectrum: molecular mechanisms and new phenotypes of ANO3-dystonia

J Ousingsawat, K Talbi, H Gómez-Martín, A Koy… - Brain, 2024 - academic.oup.com
Abstract Anoctamin 3 (ANO3) belongs to a family of transmembrane proteins that form
phospholipid scramblases and ion channels. A large number of ANO3 variants were …

[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 …

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 …

Pharmacological Inhibition and Activation of the Ca2+ Activated Cl Channel TMEM16A

R Centeio, I Cabrita, R Benedetto, K Talbi… - International journal of …, 2020 - mdpi.com
TMEM16A is a Ca2+ activated Cl− channel with important functions in airways, intestine,
and other epithelial organs. Activation of TMEM16A is proposed as a therapy in cystic …

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 …

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) …

Novel anti-inflammatory approaches for cystic fibrosis lung disease: Identification of molecular targets and design of innovative therapies

C Mitri, Z Xu, P Bardin, H Corvol, L Touqui… - Frontiers in …, 2020 - frontiersin.org
Cystic fibrosis (CF) is the most common genetic disorder among Caucasians, estimated to
affect more than 70,000 people in the world. Severe and persistent bronchial inflammation …