CFTR and sphingolipids mediate hypoxic pulmonary vasoconstriction

    Proceedings of the National Academy of Sciences of the United States of America, Volume 112, Issue 13, 2015, Pages E1614-23.

    Cited by: 9|Bibtex|Views1|Links
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    Keywords:
    ceramideneutral sphingomyelinasepulmonary hypertensiontransient receptor potential canonical 6

    Abstract:

    Hypoxic pulmonary vasoconstriction (HPV) optimizes pulmonary ventilation-perfusion matching in regional hypoxia, but promotes pulmonary hypertension in global hypoxia. Ventilation-perfusion mismatch is a major cause of hypoxemia in cystic fibrosis. We hypothesized that cystic fibrosis transmembrane conductance regulator (CFTR) may be crit...More

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