digestive/alimentary system
• jejuna obtained from class III mice (those surviving past three weeks of age) exhibit a negative basal potential difference across the intestinal epithelium, although it is less negative than that observed in jejuna from control animals
• transepithelial resistance in the jejunum of CIII mice (those surviving past three weeks of age) was higher than that seen in control animals
• the chloride channel blocker NPPB inhibited the jejunal PD measured in solid diet- and liquid diet-fed control animals to a similar extent, suggesting that the jejunal epithelium of class III mice can produce chloride secretion in the absence of Cftr
• the value of the negative potential difference across the jejunal intestinal epithelium correlates with survival in class II (survive only to weaning age) and class III (survive past 6 weeks of age) mice
• the chloride channel blocker NPPB effectively inhibits transepithelial PD in both class II and class III mice
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mortality/aging
• Background Sensitivity: most homozygotes die between birth and 25 days of age (class II mice); 31% survive longer than 6 weeks of age (class III mice)
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