Analysis Tools
mortality/aging
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• mean lifespan is 2-4 weeks
• some mice in early generations (less than 6) survive longer, up to 9 months of age; however, all mice in later generations die within the first 4 weeks after birth
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• about 50% of mice born alive die with 3 days of birth
• mean lifespan is 2-4 weeks
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• about 54% appear to die before birth
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cellular
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• increase in the frequency of micronuclei in PMEFs
• cells with micronuclei do not show wrinkling of the lamina
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• appears wrinkled in some fibroblasts
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• enhanced apoptosis in the central nervous system
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• more granulocytes lacking mature ring forms are found in the bone marrow
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immune system
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• hyposegmentation of the nuclei
• however, no defects in granulocyte function are detected
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• more granulocytes lacking mature ring forms are found in the bone marrow
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• lack nuclear segmentation
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• splenic lymphocytes display clumping of the chromatin in the nuclei
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• develop in the few mice that survive up to 9 months of age and result in sores
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adipose tissue
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• very little subcutaneous fat
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nervous system
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• enhanced apoptosis in the central nervous system
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• increase in GFAP positive astrocytes at the periphery of the brain and extending out from the ventricles
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reproductive system
vision/eye
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• the nasolacrimal ducts are clogged with undefined matter
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skeleton
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• compression of the rib cage resulting in 7 lumbar vertebrae between the rib cage and the pelvis instead of the normal 6
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homeostasis/metabolism
| N |
• unlike in human patients with mutations in LBR, no significant difference in cholesterol or desmosterol levels are detected
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hematopoietic system
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• hyposegmentation of the nuclei
• however, no defects in granulocyte function are detected
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• more granulocytes lacking mature ring forms are found in the bone marrow
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• lack nuclear segmentation
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• splenic lymphocytes display clumping of the chromatin in the nuclei
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integument
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• very little subcutaneous fat
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