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10.1101/cshperspect.a009787

http://scihub22266oqcxt.onion/10.1101/cshperspect.a009787
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C3662351!3662351!23732851
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suck abstract from ncbi


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pmid23732851      Cold+Spring+Harb+Perspect+Med 2013 ; 3 (6): ä
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  • New Pulmonary Therapies Directed at Targets Other than CFTR #MMPMID23732851
  • Donaldson SH; Galietta L
  • Cold Spring Harb Perspect Med 2013[Jun]; 3 (6): ä PMID23732851show ga
  • Our current understanding of the pathogenesis of cystic fibrosis (CF) lung disease stresses the importance of the physical and chemical properties of the airway surface liquid (ASL). In particular, the loss of cystic fibrosis transmembrane conductance regulator (CFTR) chloride channel function in CF reduces the volume and fluidity of the ASL, thus impairing mucociliary clearance and innate antimicrobial mechanisms. Besides direct approaches to restoring mutant CFTR function, alternative therapeutic strategies may also be considered to correct the basic defect of impaired salt and water transport. Such alternative strategies are focused on the restoration of mucociliary transport by (1) reducing sodium and fluid absorption by inhibiting the ENaC channel; (2) activating alternative chloride channels; and (3) increasing airway surface hydration with osmotic agents. Therapeutic approaches directed at targets other than CFTR are attractive because they are potentially useful to all patients irrespective of their genotype. Clinical trials are underway to test the efficacy of these approaches.
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