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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.
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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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Vesicular Tubular Clusters

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Protein Translocation Machinery on the ER Membrane01:28

Protein Translocation Machinery on the ER Membrane

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Related Experiment Video

Updated: May 18, 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

Development of CFTR Structure.

Anna E Patrick1, Philip J Thomas

  • 1Department of Physiology, University of Texas Southwestern Medical Center Dallas, TX, USA.

Frontiers in Pharmacology
|September 14, 2012
PubMed
Summary
This summary is machine-generated.

Cystic fibrosis (CF) arises from non-functional CFTR proteins due to mutations like ΔF508, which disrupt protein folding. Therapeutics can target these folding defects to treat CF.

Keywords:
ABC transporterCFTRcystic fibrosismembrane protein structuremultidomain protein folding

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Last Updated: May 18, 2026

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

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In Vitro Analysis of PDZ-dependent CFTR Macromolecular Signaling Complexes
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Expression and Purification of the Cystic Fibrosis Transmembrane Conductance Regulator Protein in Saccharomyces cerevisiae
14:56

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

Published on: March 10, 2012

Area of Science:

  • Biochemistry
  • Genetics
  • Structural Biology

Background:

  • Cystic fibrosis (CF) is a fatal genetic disorder caused by defective cystic fibrosis transmembrane conductance regulator (CFTR) protein.
  • CFTR protein dysfunction results from mutations, with ΔF508 being the most common, leading to improper protein folding.

Purpose of the Study:

  • To review the structural relationships between CFTR and ABC transporters.
  • To elucidate the folding process of CFTR and how mutations disrupt it.
  • To highlight therapeutic targets for correcting CFTR folding defects.

Main Methods:

  • Analysis of homologous ABC transporter structures.
  • Molecular modeling of CFTR.
  • Examination of high-resolution structures of individual CFTR domains.

Main Results:

  • The ΔF508 mutation impairs the folding of the first nucleotide-binding domain (NBD1) and its association with the second transmembrane domain (TMD2).
  • Understanding CFTR's hierarchical folding process is crucial for developing targeted therapies.
  • No full-length CFTR structures are available, but insights are derived from related proteins and domains.

Conclusions:

  • CFTR folding defects, particularly those caused by ΔF508, present viable therapeutic targets.
  • Targeting specific steps in CFTR's complex folding pathway offers a strategy for treating cystic fibrosis.
  • Further research into CFTR structure and folding is essential for advancing CF therapeutics.