Search research articles

ABOUT JoVE

Overview Leadership Blog JoVE Help Center

AUTHORS

Publishing Process Editorial Board Scope & Policies Peer Review FAQ Submit

LIBRARIANS

Testimonials Subscriptions Access Resources Library Advisory Board FAQ

RESEARCH

JoVE Journal Methods Collections JoVE Encyclopedia of Experiments Archive

EDUCATION

JoVE Core JoVE Business JoVE Science Education JoVE Lab Manual Faculty Resource Center Faculty Site

Terms & Conditions of Use

Search research articles

Related Experiment Videos

Cystic fibrosis: basic science.

D F McAuley¹, J S Elborn

¹Adult Cystic Fibrosis Centre, Belfast City Hospital, Belfast, UK.

Paediatric Respiratory Reviews

|January 18, 2003

Summary

This summary is machine-generated.

Related Concept Videos

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by

Same author

A user evaluation of speech/phrase recognition software in critically ill patients: a DECIDE-AI feasibility study.

Critical care (London, England)·2023

Same author

Modulation, microbiota and inflammation in the adult CF gut: A prospective study.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society·2022

Same author

Sugar or salt ("SOS"): A protocol for a UK multicentre randomised trial of mannitol and hypertonic saline in severe traumatic brain injury and intracranial hypertension.

Journal of the Intensive Care Society·2022

Same author

Guiding the rational design of patient-centred drug trials in Cystic Fibrosis: A Delphi study.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society·2021

Same author

Cystic fibrosis drug trial design in the era of CFTR modulators associated with substantial clinical benefit: stakeholders' consensus view.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society·2020

Same author

Infection with Prevotella nigrescens induces TLR2 signalling and low levels of p65 mediated inflammation in Cystic Fibrosis bronchial epithelial cells.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society·2019

Same journal

Image-guided pediatric airway endoscopy: A review.

Paediatric respiratory reviews·2026

Same journal

Bronchoscopic and interventional management of tracheobronchomalacia in children with bronchopulmonary dysplasia: a review of evidence.

Paediatric respiratory reviews·2026

Same journal

Hand in glove: New imaging techniques and airway endoscopy.

Paediatric respiratory reviews·2026

Same journal

Bronchopulmonary dysplasia associated pulmonary hypertension: implications across the lifespan.

Paediatric respiratory reviews·2026

Same journal

How to monitor response to biologics in children with severe asthma.

Paediatric respiratory reviews·2026

Same journal

Moving the goalposts from symptom control to preventing airway remodeling in preschool asthma.

Paediatric respiratory reviews·2026

See all related articles

Understanding cystic fibrosis transmembrane regulator (CFTR) function reveals how CF gene defects cause disease. CFTR dysfunction impacts airway liquids, defense, and inflammation, leading to novel therapeutic strategies.

Area of Science:

Molecular Biology
Genetics
Physiology

Background:

Cystic Fibrosis (CF) is a genetic disorder caused by mutations in the CFTR gene.
The CFTR protein is crucial for regulating ion transport, particularly chloride ions, in epithelial cells.
Understanding CFTR's diverse functions is key to comprehending CF pathogenesis.

Purpose of the Study:

To elucidate the multifaceted roles of CFTR in cellular functions beyond chloride transport.
To explore how CFTR mutations and dysfunction contribute to the clinical manifestations of cystic fibrosis.
To identify potential targets for novel therapeutic interventions based on CFTR function.

Main Methods:

Review of current literature on CFTR function and its implications in cystic fibrosis.

Related Experiment Videos

Analysis of the relationship between CFTR mutation classes and clinical phenotypes.

Examination of CFTR's role in regulating airway surface liquid, mucociliary clearance, and inflammatory responses.

Main Results:

CFTR regulates airway surface liquid volume and composition by controlling chloride transport.
CFTR influences mucociliary clearance and innate defense mechanisms through transport of other molecules.
CFTR dysfunction modulates inflammatory responses in respiratory and other inflammatory cells.

Conclusions:

CFTR dysfunction leads to a spectrum of effects contributing to the cystic fibrosis clinical phenotype.
These effects manifest at various stages of the disease's natural history.
Enhanced understanding of CFTR defect mechanisms promises new therapeutic avenues for cystic fibrosis.