growth/size/body
• pups exhibit a small growth deficit
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homeostasis/metabolism
• 3 month old females exhibit lower triglyceride levels
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• in everted gut sac experiments, the transileal taurocholate (TC) transport is reduced by about 95% and 80% in male and female mutants, respectively, indicating impaired intestinal bile acid transport
• efflux of taurocholate from tissue to the serosal side (amount of TC in serosal fluid/TC associated with tissue) is decreased
• the bile acid FTR (daily fecal bile acid excretion/pool size) is elevated 5-fold in males and 3-fold in females
• mutants fed exogenous bile acids (0.2% cholic acid diet) exhibit a 5-fold elevation in the rate of fecal bile acid excretion by day 2 compared to wild-type and remains constant and higher
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• bile acid pool is decreased by 70% and 65% in males and females, respectively
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• fecal cholesterol excretion is elevated about 4-fold in both males and females
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• fecal cholesterol excretion is elevated about 4-fold in both males and females
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• 3 month old females exhibit lower plasma cholesterol levels
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digestive/alimentary system
• fecal cholesterol excretion is elevated about 4-fold in both males and females
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• 3 month old adult males exhibit longer small intestines than wild-type
• small intestinal hypertrophy
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