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Updated: Jul 5, 2026
15:12
Purification of the Cystic Fibrosis Transmembrane Conductance Regulator Protein Expressed in Saccharomyces cerevisiae
Published on: May 10, 2014
Role of oxygen availability in CFTR expression and function.
Jennifer S Guimbellot1, James A Fortenberry, Gene P Siegal
1Department of Genetics, Fleming James Cystic Fibrosis Research Center, University of Alabama at Birmingham, Birmingham, AL 35294-0005, USA.
Summary
Hypoxia, or low oxygen, significantly alters cystic fibrosis transmembrane conductance regulator (CFTR) mRNA levels and function in human cells and tissues. This discovery offers insights into chronic hypoxic lung disease pathogenesis.
Area of Science:
- Cell Biology
- Physiology
- Molecular Biology
Background:
- The cystic fibrosis transmembrane conductance regulator (CFTR) is crucial for epithelial homeostasis.
- CFTR dysfunction causes exocrine tissue destruction, particularly in the lungs and gastrointestinal tract.
- While CFTR protein regulation is understood, mRNA level control remains less clear.
Purpose of the Study:
- To investigate CFTR mRNA regulation under oxygen restriction (hypoxia).
- To examine the impact of hypoxic signaling on epithelial chloride transport.
- To explore the role of these findings in chronic hypoxic lung disease.
Main Methods:
- Studied human cells and mouse models under hypoxic conditions.
- Assessed CFTR mRNA, protein levels, and function.
- Analyzed pulmonary tissues from hypoxemic lung transplant recipients.
Main Results:
- Hypoxia robustly and reversibly altered CFTR mRNA, protein, and function in human cells.
- In vivo, low oxygen repressed CFTR mRNA expression in mouse airways, GI tract, and liver.
- Reduced CFTR mRNA was observed in human pulmonary tissues from hypoxemic patients.
Conclusions:
- Environmental factors inducing hypoxic signaling regulate CFTR mRNA levels.
- Hypoxia impacts epithelial chloride transport both in vitro and in vivo.
- Findings suggest a role for CFTR mRNA regulation in hypoxic lung disease pathogenesis.
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