TRANSEPITHELIAL POTENTIAL DIFFERENCE IN PTS W/ VARIANTS OF CF

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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. Nasal Potenial Difference Measurements are a new diagnostic tool in CF. The hypothesis of this study is that Class IV or V mutations in CFTR will be associated with chloride transport in the NPD. The specific aims are 1) to correlate the magnitude of the change in NPD response to low chloride/isoproterenol/amiloride with CFTR genotype; 2) to expand the understanding of the relationship between potential surrogate markers and genotype in CF; and to substantiate the role of NPD measurement as a valuable research tool. Patients are referred by physicians who find some but not all features of CF, or on the basis of CFTR genotype.