Congenital tracheal malformation in cystic fibrosis transmembrane conductance regulator-deficient mice.

@article{Bonvin2008CongenitalTM,
  title={Congenital tracheal malformation in cystic fibrosis transmembrane conductance regulator-deficient mice.},
  author={Elise Bonvin and Philippe le Rouzic and J. -F. Bernaudin and Charles-Henry Cottart and C. Vandebrouck and Antoine Cri{\'e} and Teresinha Leal and Annick Cl{\'e}ment and Monique Bonora},
  journal={The Journal of physiology},
  year={2008},
  volume={586 13},
  pages={3231-43}
}
In cystic fibrosis (CF) patients, the major alteration in pulmonary function is due to peripheral airway obstruction. In the present study, we investigated the possibility that alterations in the extrathoracic airways, particularly in the trachea that expresses high levels of CFTR (CF transmembrane conductance regulator), may contribute to respiratory dysfunction. We performed morphological analyses of the trachea and airway functional studies in adult Cftr knockout (Cftr(-/-)) and F508del-CFTR… CONTINUE READING

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