Targeting the mitochondria in chronic respiratory diseases

DK Chellappan, KR Paudel, NW Tan, KS Cheong… - Mitochondrion, 2022 - Elsevier
Mitochondria are one of the basic essential components for eukaryotic life survival. It is also
the source of respiratory ATP. Recently published studies have demonstrated that …

Discovery of CFTR modulators for the treatment of cystic fibrosis

M Lopes-Pacheco, N Pedemonte… - Expert Opinion on Drug …, 2021 - Taylor & Francis
Introduction Cystic fibrosis (CF) is a life-threatening inherited disease caused by mutations
in the gene encoding the CF transmembrane conductance regulator (CFTR) protein, an …

Impact of cystic fibrosis transmembrane conductance regulator therapy on chronic rhinosinusitis and health status: deep learning CT analysis and patient-reported …

DM Beswick, SM Humphries… - Annals of the …, 2022 - atsjournals.org
Rationale: Elexacaftor, tezacaftor, and ivacaftor (ETI) in triple combination improves
pulmonary health for people with cystic fibrosis (PwCF). However, its impact on objective …

[HTML][HTML] Mutation-specific dual potentiators maximize rescue of CFTR gating mutants

G Veit, DF Da Fonte, RG Avramescu… - Journal of Cystic …, 2020 - Elsevier
Background The potentiator ivacaftor (VX-770) has been approved for therapy of 38 cystic
fibrosis (CF) mutations (∼ 10% of the patient population) associated with a gating defect of …

Loureirin C extracted from Dracaena cochinchinensis SC Chen prevents rotaviral diarrhea in mice by inhibiting the intestinal Ca2+-activated Cl− channels

SC Guo, B Yu, Q Jia, HY Yan, LQ Wang, FF Sun… - Journal of …, 2024 - Elsevier
Abstract Ethnopharmacological relevance Resina Draconis (RD) is the red resin of
Dracaena cochinchinensis (Lour.) SC Chen and most used as a hemostatic drug in …

Mitochondrial stress responses and “Mito-Inflammation” in cystic fibrosis

S Patergnani, VAM Vitto, P Pinton… - Frontiers in …, 2020 - frontiersin.org
Cystic fibrosis (CF) is a genetic disease associated to mutations in the cystic fibrosis
transmembrane conductance regulator gene, which results in the alteration of biological …

Hypoxia‐induced cystic fibrosis transmembrane conductance regulator dysfunction is a universal mechanism underlying reduced mucociliary transport in sinusitis

DY Cho, S Zhang, TG Norwood… - … Forum of Allergy & …, 2024 - Wiley Online Library
Introduction Hypoxia due to sinus obstruction is a major pathogenic mechanism leading to
sinusitis. The objective of the current study is to define the electrophysiologic characteristics …

Role of inflammation and oxidative stress in tissue damage associated with cystic fibrosis: CAPE as a future therapeutic strategy

VEM Soares, TIT do Carmo, F Dos Anjos… - Molecular and Cellular …, 2022 - Springer
Cystic fibrosis (CF) is an autosomal recessive disease caused by mutations in the cystic
fibrosis transmembrane conductance regulator (CFTR) gene, responsible for the synthesis …

[HTML][HTML] LPS decreases CFTR open probability and mucociliary transport through generation of reactive oxygen species

S Zhang, A Lazrak, D Skinner, HM Thompson… - Redox Biology, 2021 - Elsevier
Lipopolysaccharide (LPS) serves as the interface between gram-negative bacteria (GNB)
and the innate immune response in respiratory epithelial cells (REC). Herein, we describe a …

Ivacaftor restores delayed mucociliary transport caused by Pseudomonas aeruginosa–induced acquired cystic fibrosis transmembrane conductance regulator …

DY Cho, S Zhang, DF Skinner, DJ Lim… - International forum of …, 2022 - Wiley Online Library
Background Abnormal chloride (Cl–) transport dehydrates airway surface liquid (ASL) in
sinonasal epithelium leading to mucus stasis and chronic rhinosinusitis. As an experimental …