mortality/aging
• mutants die within 12 hours of birth
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homeostasis/metabolism
hypoglycemia
(
J:103919
)
• blood glucose is less than 10 mg/dl shortly after birth compared to 58 mg/dl in controls; however mutants are able to feed as milk is found in the digestive tract
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• total glucose production in the liver is reduced about 3-fold with gluconeogenesis reduced about 2-fold
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• total glucose production in the liver is reduced about 3-fold with glycogenolysis reduced about 3-fold
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• at E17.5, E18.5, and P0, glycogen levels in the liver are severely reduced
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liver/biliary system
• proliferation in parenchymal cells is increased 1.6-fold and 4- to 5-fold at E17.5 and E18.5, respectively and a 40% increase in parenchymal cells is seen
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• at E17.5, E18.5, and P0, glycogen levels in the liver are severely reduced
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• the cytoplasm contains fewer organelles and mitochondria suggesting a defect in terminal hepatocyte differentiation
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• intermembrane spaces are enlarged and cell-cell junctions are disrupted
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cellular
• proliferation in parenchymal cells is increased 1.6-fold and 4- to 5-fold at E17.5 and E18.5, respectively and a 40% increase in parenchymal cells is seen
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