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


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