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Related Experiment Video

Updated: Apr 15, 2026

Videomorphometric Analysis of Hypoxic Pulmonary Vasoconstriction of Intra-pulmonary Arteries Using Murine Precision Cut Lung Slices
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CFTR and sphingolipids mediate hypoxic pulmonary vasoconstriction.

Christoph Tabeling1, Hanpo Yu2, Liming Wang2

  • 1Departments of Infectious Diseases and Pulmonary Medicine and.

Proceedings of the National Academy of Sciences of the United States of America
|April 2, 2015
PubMed
Summary

Cystic fibrosis transmembrane conductance regulator (CFTR) plays a key role in hypoxic pulmonary vasoconstriction (HPV) by interacting with TRPC6 channels and sphingolipids, impacting pulmonary hypertension and ventilation-perfusion mismatch.

Keywords:
ceramideneutral sphingomyelinasepulmonary hypertensiontransient receptor potential canonical 6

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Area of Science:

  • Cardiovascular Physiology
  • Respiratory Medicine
  • Molecular Biology

Background:

  • Hypoxic pulmonary vasoconstriction (HPV) is crucial for matching ventilation and perfusion in the lungs.
  • Ventilation-perfusion mismatch contributes to hypoxemia in cystic fibrosis.
  • The role of cystic fibrosis transmembrane conductance regulator (CFTR) in HPV is not well understood.

Purpose of the Study:

  • To investigate the role of CFTR in HPV.
  • To determine if CFTR modulates sphingolipid mediators of HPV.
  • To elucidate the mechanisms underlying CFTR's involvement in HPV and ventilation-perfusion mismatch.

Main Methods:

  • Analysis of HPV and ventilation-perfusion mismatch in isolated mouse lungs and in vivo.
  • Study of Ca(2+) mobilization and TRPC6 translocation in human pulmonary and coronary artery smooth muscle cells.
  • Investigation of the effects of CFTR inhibition, nSMase inhibition, SphK1 inhibition, and S1P receptor antagonism.

Main Results:

  • CFTR inhibition or deficiency reduced HPV and worsened ventilation-perfusion mismatch.
  • Hypoxia induced CFTR-TRPC6 interaction, essential for TRPC6 translocation and Ca(2+) mobilization in pulmonary artery smooth muscle cells.
  • CFTR modulated S1P-induced Ca(2+) mobilization differently in pulmonary versus coronary artery smooth muscle cells.
  • nSMase and S1P synergistically induced pulmonary vasoconstriction via TRPC6, phospholipase C, and rho kinase, with CFTR and SphK1 playing critical roles.

Conclusions:

  • CFTR is central to HPV, interacting with TRPC6 channels and sphingolipids.
  • CFTR's interaction with TRPC6 is required for hypoxia-induced vasoconstriction.
  • Sphingolipid signaling, involving nSMase, SphK1, and S1P receptors, is critical for HPV and is modulated by CFTR.