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Related Concept Videos

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.
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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.
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Pharmacogenomics: Identification of New Drug Targets

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P-glycoprotein modulates the fluidity gradient of the plasma membrane of multidrug resistant CHO cells.

FEBS letters·2025
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Domain-interface dynamics of CFTR revealed by stabilizing nanobodies.

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Cryo-EM Visualization of an Active High Open Probability CFTR Anion Channel.

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R-Domain Phosphorylation by Protein Kinase A Stimulates Dissociation of Unhydrolyzed ATP from the First Nucleotide-Binding Site of the Cystic Fibrosis Transmembrane Conductance Regulator.

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Ligand binding to a remote site thermodynamically corrects the F508del mutation in the human cystic fibrosis transmembrane conductance regulator.

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Structural stability of purified human CFTR is systematically improved by mutations in nucleotide binding domain 1.

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Related Experiment Video

Updated: Jul 7, 2026

Purification of the Cystic Fibrosis Transmembrane Conductance Regulator Protein Expressed in Saccharomyces cerevisiae
15:12

Purification of the Cystic Fibrosis Transmembrane Conductance Regulator Protein Expressed in Saccharomyces cerevisiae

Published on: May 10, 2014

CFTR function and prospects for therapy.

John R Riordan1

  • 1Department of Biochemistry and Biophysics, Cystic Fibrosis Treatment and Research Center, School of Medicine, University of North Carolina at Chapel Hill, NC 27599, USA. jack_riordan@med.unc.edu

Annual Review of Biochemistry
|February 29, 2008
PubMed
Summary
This summary is machine-generated.

Cystic fibrosis (CF) is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. This review explores therapeutic strategies based on CFTR

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

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Related Experiment Videos

Last Updated: Jul 7, 2026

Purification of the Cystic Fibrosis Transmembrane Conductance Regulator Protein Expressed in Saccharomyces cerevisiae
15:12

Purification of the Cystic Fibrosis Transmembrane Conductance Regulator Protein Expressed in Saccharomyces cerevisiae

Published on: May 10, 2014

Forskolin-induced Swelling in Intestinal Organoids: An In Vitro Assay for Assessing Drug Response in Cystic Fibrosis Patients
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Forskolin-induced Swelling in Intestinal Organoids: An In Vitro Assay for Assessing Drug Response in Cystic Fibrosis Patients

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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

Area of Science:

  • Biochemistry
  • Molecular Biology
  • Physiology

Background:

  • Cystic fibrosis (CF) is a genetic disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
  • CFTR is an epithelial anion channel essential for ion and fluid homeostasis.
  • It belongs to the adenine nucleotide-binding cassette (ABC) transporter family.

Purpose of the Study:

  • To review therapeutic strategies for CF.
  • To discuss CFTR structure, function, and processing.
  • To explore insights from CFTR's biosynthetic pathway, trafficking, and turnover.

Main Methods:

  • Literature review of CFTR structure and function.
  • Analysis of CFTR's role in ion and fluid transport.
  • Examination of CFTR's biosynthetic processing, trafficking, and turnover.

Main Results:

  • Knowledge of CFTR structure and function informs therapeutic development.
  • Understanding CFTR's cellular journey is crucial for effective treatment.
  • Various therapeutic strategies are emerging based on these insights.

Conclusions:

  • Targeting CFTR offers promising therapeutic avenues for cystic fibrosis.
  • Further research into CFTR's complex biology will drive innovation.
  • Comprehensive understanding of CFTR is key to managing CF.