Abstract
Summary: Sera from 100 subjects were tested for the presence of the cystic fibrosis (CF) “factor” utilizing the short circuit current rat jejunum bioassay. Comparing the mean percent decrease in short circuit current, the presumed normal group differs from the homozygous CF group at P = 0.001 and from the heterozygous group at P = 0.05. The two CF genotypes are statistically different at P = 0.004. There is, however, a large overlap among the three groups, which limits the assay's utility as a guide for factor purification as well as clinical use.
Speculation: Serum and saliva from CF genotypes have been shown to contain a factor (or factors) which decreases the sodium ion transport across the cell membrane. Currently, a bioassay is being sought to guide isolation of the factor for development of a radioimmunoassay. Characterization of the factor may explain the underlying transport error in CF as well as enable heterozygote carrier identification.
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Tucker, R., Gibbs, G. & Christensen, M. Cystic Fibrosis Serum Effect on the Short Circuit Current of Rat Jejunum. Pediatr Res 13, 1371–1374 (1979). https://doi.org/10.1203/00006450-197912000-00014
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DOI: https://doi.org/10.1203/00006450-197912000-00014
