growth/size/body
• body weight is about 20% lower than in wild-type at weaning and lower weight is seen throughout life
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• liver is enlarged to 10.1% of the body weight
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homeostasis/metabolism
• decrease in liver glycogen content
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• total concentration of biliary bile salts is reduced more than 4-fold
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• total bile salt concentrations in the liver and plasma are elevated 4- and 5.8-fold, respectively
• mutants exhibit increased amounts of tetra-hydroxylated bile acids
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• 7-fold increase in biliary cholesterol concentration
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• 2.5-fold increase in biliary phospholipid concentration
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liver/biliary system
• liver is enlarged to 10.1% of the body weight
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• decrease in liver glycogen content
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• dilation of canalicular lumens, partial or complete loss of microvilli, and retention of biliary material
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• increase in peroxisomes, lysosomes, and lipid droplets
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• impaired secretion of major hydrophobic bile salts, such as taurine and glycine-conjugated cholic acid, however secretion of the more hydrophilic muricholic acids is unaffected
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• nonprogressive but persistent intrahepatic cholestasis
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