Hyperacidification in cystic fibrosis: links with lung disease and new prospects for treatment.

@article{Poschet2002HyperacidificationIC,
  title={Hyperacidification in cystic fibrosis: links with lung disease and new prospects for treatment.},
  author={Jens Fredrich Poschet and Elizabeth A. Perkett and Vojo Deretic},
  journal={Trends in molecular medicine},
  year={2002},
  volume={8 11},
  pages={
          512-9
        }
}
A new link between the genetic defect and lung pathology in cystic fibrosis (CF) has been established by the recent discovery of an abnormally acidic pH in the organelles of CF respiratory epithelial cells, along with an increased acidity of the CF airway surface liquid. The defect in cystic fibrosis transmembrane resistance regulator (CFTR) results in hyperacidification of the trans-Golgi network, an organelle responsible for glycosylation, and protein- and membrane-sorting in mammalian cells… CONTINUE READING
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