Cystic fibrosis heterozygote resistance to cholera toxin in the cystic fibrosis mouse model

SE Gabriel, KN Brigman, BH Koller, RC Boucher… - Science, 1994 - science.org
SE Gabriel, KN Brigman, BH Koller, RC Boucher, MJ Stutts
Science, 1994science.org
The effect of the number of cystic fibrosis (CF) alleles on cholera toxin (CT)-induced
intestinal secretion was examined in the CF mouse model. CF mice that expressed no CF
transmembrane conductance regulator (CFTR) protein did not secrete fluid in response to
CT. Heterozygotes expressed 50 percent of the normal amount of CFTR protein in the
intestinal epithelium and secreted 50 percent of the normal fluid and chloride ion in
response to CT. This correlation between CFTR protein and CT-induced chloride ion and …
The effect of the number of cystic fibrosis (CF) alleles on cholera toxin (CT)-induced intestinal secretion was examined in the CF mouse model. CF mice that expressed no CF transmembrane conductance regulator (CFTR) protein did not secrete fluid in response to CT. Heterozygotes expressed 50 percent of the normal amount of CFTR protein in the intestinal epithelium and secreted 50 percent of the normal fluid and chloride ion in response to CT. This correlation between CFTR protein and CT-induced chloride ion and fluid secretion suggests that CF heterozygotes might possess a selective advantage of resistance to cholera.
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