Related Experiment Video
Updated: Jul 15, 2025

08:00
Generation of Human Nasal Epithelial Cell Spheroids for Individualized Cystic Fibrosis Transmembrane Conductance Regulator Study
Published on: April 11, 2018
10.7K
The gut-lung axis in the CFTR modulator era
Florian Lussac-Sorton1,2, Éléna Charpentier1,2, Sébastien Imbert1,2,3
1Univ. Bordeaux, Centre de Recherche Cardio-Thoracique de Bordeaux, INSERM U1045, Pessac, France.
Frontiers in Cellular and Infection Microbiology
|October 2, 2023
Summary
CFTR modulators transform cystic fibrosis (CF) care by enhancing mucosal hydration. This review explores how these therapies impact lung and gut microbiomes, including the gut-lung axis, to predict disease progression.
Area of Science:
- Microbiology
- Pulmonology
- Gastroenterology
Background:
- Cystic Fibrosis (CF) is characterized by bacterial and fungal dysbiosis.
- CFTR modulators have revolutionized CF management by improving mucosal hydration.
- The precise impact of CFTR modulators on microbial communities remains incompletely understood.
Purpose of the Study:
- To review the current knowledge on CFTR modulator effects on pulmonary and digestive microbiomes.
- To examine the gut-lung axis in CF pathophysiology.
- To understand how these modulators influence the gut-lung axis in CF.
Main Methods:
- Literature review of studies investigating CFTR modulators and microbiome changes.
- Analysis of research on pulmonary and gut microbial communities in CF patients.
- Synthesis of data on inter-organ microbial connections and the gut-lung axis.
Main Results:
- CFTR modulators significantly alter airway and digestive tract microbiomes.
- Evidence suggests a modulation of the gut-lung axis by CFTR modulators.
- Understanding these shifts is crucial for predicting CF progression.
Conclusions:
- CFTR modulators represent a paradigm shift in CF treatment, impacting microbial ecosystems.
- Further research is needed to fully elucidate the mechanisms and long-term consequences of these microbial changes.
- Targeting the gut-lung axis may offer novel therapeutic strategies in the era of CFTR modulators.
Related Concept Videos
Cystic Fibrosis: Management
185
Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
Sinus disease and chronic...
Sinus disease and chronic...
185
Cystic Fibrosis: Pathogenesis
269
Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation,...
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation,...
269
GPCRs Regulate Adenylyl Cylase Activity
5.7K
Some GPCRs transmit signals through adenylyl cyclase (AC), a transmembrane enzyme. AC helps synthesize second messenger cyclic adenosine monophosphate (cAMP). AC catalyzes cyclization reaction and converts ATP to cAMP by releasing a pyrophosphate. The pyrophosphate is further hydrolyzed to phosphate by the enzyme pyrophosphatase, which drives cAMP synthesis to completion. However, cAMP is rapidly degraded to 5′ AMP by the enzymes phosphodiesterase (PDE), preventing overstimulation of...
5.7K

