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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 1, 2026

Functional Reconstitution and Channel Activity Measurements of Purified Wildtype and Mutant CFTR Protein
09:59

Functional Reconstitution and Channel Activity Measurements of Purified Wildtype and Mutant CFTR Protein

Published on: March 9, 2015

Introduction to section V: assessment of CFTR function.

Karl Kunzelmann1

  • 1Department of Physiology, University of Regensburg, 93053, Regensburg, Germany. karl.kunzelmann@vkl.uni-regensburg.de

Methods in Molecular Biology (Clifton, N.J.)
|May 20, 2011
PubMed
Summary

This chapter details methods for assessing cystic fibrosis transmembrane conductance regulator (CFTR) function, from cellular interactions to protein analysis. Understanding CFTR function requires a multi-level approach for comprehensive insights.

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Area of Science:

  • Biophysics
  • Cell Biology
  • Physiology

Background:

  • Cystic fibrosis transmembrane conductance regulator (CFTR) plays a crucial role in cellular functions and epithelial properties.
  • Understanding CFTR's interactions and affected cellular properties is key to studying its function.

Discussion:

  • Patch clamping and double electrode voltage clamp in Xenopus oocytes are vital for CFTR expression analysis.
  • The Ussing chamber technique is essential for analyzing CFTR function in polarized epithelia.

Key Insights:

  • CFTR function assessment should encompass multiple biological levels.
  • A comprehensive understanding requires examining CFTR from intact epithelia down to isolated proteins.

Outlook:

  • Further research into CFTR function at various levels will elucidate its role in health and disease.
  • Developing and refining these techniques will advance cystic fibrosis research and therapeutic strategies.