mortality/aging
• survival is limited to 3 to 4 days
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digestive/alimentary system
• examined at E18.5 and P0
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embryo
• as examined at E13.5
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growth/size/body
• as examined at E13.5
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• 30% body weight reduction at birth compared to littermates
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liver/biliary system
• abnormal mitochondrial structure, autophagic vacuoles, accumulation of lipids and increased number of macrophages; absence of peroxisomes in hepatocytes
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muscle
• hypotonic, unable to support their weight
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renal/urinary system
• autophagic vacuoles, and increased number of macrophages; absence of peroxisomes in renal epithelial cells
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• examined at E18.5 and P0
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respiratory system
• noted in some neonates, variable onset and severity
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nervous system
• reduced thickness of the neocortical plate examined at P0.5
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• reduced thickness of cortex
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