Analysis Tools
mortality/aging
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• incomplete penetrance; 70% of homozygous mice die within 5 days of birth and die within 20-30 hours after the onset of weakness symptoms
• the remaining mice appeared normal, were viable and fertile and survived greater than 6 months in a pathogen free environment
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homeostasis/metabolism
cardiovascular system
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• marked congestion is shown by histology
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• marked congestion is shown by histology
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• atrial and ventricular walls are thin
• heart abnormalities are seen in 70% of mice at 1 day and appear to correlate with mice exhibiting postnatal lethality, suggesting that the lethality is due to heart defects
• cardiomyocytes proliferate and differentiate normally during embryogenesis
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• myocardium shows decreased cellularity without signs of inflammation
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• reduced atrial and ventricular trabeculae
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• described as extended
• reduced trabeculation
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• septal walls are thin
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• in 70% of mice at 1 day of age
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• described as extended
• reduced trabeculation
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immune system
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• cultured thymocytes from homozygous mice that are challenged with etoposide or UV irradiation show a 3-4 fold increase in caspase-3 activity, an upstream activator of DNA fragmentation enzymes that are active during apoptosis
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• widespread pyknotic nuclei are present in the cortex and medulla
• TUNEL staining indicates that these nuclei are undergoing apoptosis
• profiles of remaining CD4 and CD8-positive cell populations indicate normal ratios
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small thymus
(
J:62244
)
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• total thymocyte numbers are reduced 5-10 fold
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nervous system
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• increased number of apoptotic cells compared to controls at 12.5 dpc
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• increased number of apoptotic cells compared to controls at 12.5 dpc
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• increased number of apoptotic cells compared to controls at 12.5 dpc
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• increased number of apoptotic cells compared to controls at 12.5 dpc
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liver/biliary system
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• marked congestion is shown by histology
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respiratory system
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• marked congestion is shown by histology
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hematopoietic system
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• cultured thymocytes from homozygous mice that are challenged with etoposide or UV irradiation show a 3-4 fold increase in caspase-3 activity, an upstream activator of DNA fragmentation enzymes that are active during apoptosis
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• widespread pyknotic nuclei are present in the cortex and medulla
• TUNEL staining indicates that these nuclei are undergoing apoptosis
• profiles of remaining CD4 and CD8-positive cell populations indicate normal ratios
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small thymus
(
J:62244
)
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• total thymocyte numbers are reduced 5-10 fold
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muscle
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• myocardium shows decreased cellularity without signs of inflammation
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• reduced atrial and ventricular trabeculae
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• massive cell death of cardiomyocytes is seen by TUNEL staining 6 hours after birth
• apoptotic changes are prominent in the left ventricle, although also seen in the right ventricles in both mutant and control mice
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behavior/neurological
integument
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• in 70% of mice at 1 day of age
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cellular
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• cultured thymocytes from homozygous mice that are challenged with etoposide or UV irradiation show a 3-4 fold increase in caspase-3 activity, an upstream activator of DNA fragmentation enzymes that are active during apoptosis
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• massive cell death of cardiomyocytes is seen by TUNEL staining 6 hours after birth
• apoptotic changes are prominent in the left ventricle, although also seen in the right ventricles in both mutant and control mice
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• increased number of apoptotic cells compared to controls at 12.5 dpc
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endocrine/exocrine glands
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• cultured thymocytes from homozygous mice that are challenged with etoposide or UV irradiation show a 3-4 fold increase in caspase-3 activity, an upstream activator of DNA fragmentation enzymes that are active during apoptosis
|
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• widespread pyknotic nuclei are present in the cortex and medulla
• TUNEL staining indicates that these nuclei are undergoing apoptosis
• profiles of remaining CD4 and CD8-positive cell populations indicate normal ratios
|
small thymus
(
J:62244
)
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• total thymocyte numbers are reduced 5-10 fold
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growth/size/body
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• in 70% of mice at 1 day of age
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