mortality/aging
• mice die between 18 and 28 days of age exhibiting severe neurological defects
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growth/size/body
• do not gain weight starting at 2 weeks after birth
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behavior/neurological
• while mice show no overt abnormalities prior to 2 weeks of age, they later exhibit severe tremors
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• slack posture
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• flaccid tail
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abnormal gait
(
J:85759
)
• waddling gait first observed at P14
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• infrequent mobility
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• while mice show no overt abnormalities prior to 2 weeks of age, they later exhibit spontaneous seizures
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nervous system
• while mice show no overt abnormalities prior to 2 weeks of age, they later exhibit spontaneous seizures
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• lack of proliferation of precursor cells in the hippocampus and cerebellum
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• reduced white matter tracts in the subcortical white matter and in the cortex
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• reduced number of neuronal cells in regions of the hippocampus
• aberrant lamination of the hippocampus
• reduced synapse formation in the hippocampus
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• aberrant lamination of the cerebellum
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• the external granule layer and internal granular layer are still present at P10, a time in which this layer has disappeared in wild-type mice
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• disorganized
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• reduced numbers of neurons in the cortex
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• reduced numbers of neurons in the Purkinje cell layer
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• smaller in size
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• reduced numbers of neurons in the cerebellar granule layer
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• molecular layer is not organized into a typically more compacted layer and appears broader
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• synapse formation in the hippocampus is reduced by about 69%
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• reduced white matter tracts in the subcortical white matter and in the cortex
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• reduction in the number of neurons in the cerebellum, cortex, and hippocampus and in the percentage of cells which express neurofilaments
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cellular
• lack of proliferation of precursor cells in the hippocampus and cerebellum
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• decrease in cell proliferation in the cerebellum and hippocampus during development, in the thymus, in the white and red pulp of the spleen, and bronchiolar epithelium of the lungs
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• decrease in cells undergoing DNA replication in multiple organs, including the brain, thymus, spleen, colon and lungs
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hematopoietic system
• decrease in cellular proliferation in the thymus, particularly in the thymic cortex
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• decrease in cellular proliferation in the white and red pulp of the spleen
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small spleen
(
J:85759
)
homeostasis/metabolism
• decrease in cells undergoing DNA replication in multiple organs, including the brain, thymus, spleen, colon and lungs
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immune system
• decrease in cellular proliferation in the thymus, particularly in the thymic cortex
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• decrease in cellular proliferation in the white and red pulp of the spleen
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small spleen
(
J:85759
)
respiratory system
• bronchiolar epithelium shows reduced cellular proliferation
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endocrine/exocrine glands
• decrease in cellular proliferation in the thymus, particularly in the thymic cortex
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