This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Cystic fibrosis (CF) is the most common lethal genetic disease of Caucasians. CF is caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) which forms a chloride channel in the apical membrane of affected epithelia. As a result of the defective function of the CFTR, affected epithelia have abnormal transepithelial electrolyte transport. In airway epithelia, including the nasal epithelium, the cystic fibrosis defect is measured as an abnormally increased voltage and an abnormal response to agents that modulate chloride transport. The lack of chloride transport is a diagnostic criteria for CF. In addition, an increase in basal Vt and the change in Vt after perfusion with amiloride have been implicated by others in the pathogenesis of CF. However, previous data suggest this may not be of significance for the pathogenesis of the disease. Thus, these investigators have developed three hypotheses: a) nasal Vt measurement can be used as an end point for evaluating CFTR function and CFTR gene transfer to airway epithelia in vivo; b) people wihout CF but with bronchiectasis will have an abnormal sodium or chloride transport as measured by the nasal Vt protocol; and c) interventions that increase the VtAmil will not result in cystic fibrosis lung disease.
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