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Related Concept Videos

Cystic Fibrosis: Pathogenesis01:23

Cystic Fibrosis: Pathogenesis

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, but...
Cystic Fibrosis: Management01:24

Cystic Fibrosis: Management

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 sinusitis...

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Related Experiment Video

Updated: Jun 27, 2026

Functional Reconstitution and Channel Activity Measurements of Purified Wildtype and Mutant CFTR Protein
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Published on: March 9, 2015

Cellular Models and Functional Assays for Assessing CFTR Function: A Comprehensive Review.

Margarita Lopatina1, Anna Demchenko1, Svetlana Smirnikhina1

  • 1Research Centre for Medical Genetics, Moskvorechye, 1, 115522 Moscow, Russia.

International Journal of Molecular Sciences
|June 26, 2026
PubMed
Summary

This review details advanced cellular models and functional assays for cystic fibrosis (CF) research. It covers patient-specific stem cells and organoids, aiding CFTR protein studies and therapeutic development.

Keywords:
CFTRcellular modelcystic fibrosisfunctional assay

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Area of Science:

  • Biomedical research
  • Cell biology
  • Genetic disorders

Background:

  • Cystic fibrosis (CF) is a genetic disorder stemming from CFTR chloride channel dysfunction.
  • Advancing CF research requires sophisticated cellular models and reliable assays for CFTR function.
  • Current research relies on evolving in vitro models to study CF pathology.

Purpose of the Study:

  • To review the evolution of in vitro models for cystic fibrosis research.
  • To compare various assays for assessing CFTR function.
  • To provide a framework for selecting CFTR functional assays.

Main Methods:

  • Review of literature on in vitro models for CF research.
  • Comparison of traditional and high-throughput CFTR functional assays.
  • Discussion of advanced models: iPSCs, ALI cultures, organoids, and organ-on-a-chip platforms.

Main Results:

  • Advanced models like iPSCs, organoids, and ALI cultures better mimic CF tissue architecture and pathology.
  • High-throughput assays offer scalable CFTR function screening compared to traditional methods.
  • A framework is presented for assay selection based on research objectives.

Conclusions:

  • Patient-specific iPSCs and complex 3D models significantly enhance CF research capabilities.
  • The choice of CFTR functional assay is crucial and depends on specific research goals.
  • Continued development of models and assays is vital for CF therapy advancement.