Analysis Tools
mortality/aging
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• mice have a median survival of ~18 weeks of age
• NOTE: recombination in brain is only ~30% when transgene is inherited maternally, compared to ~95% with paternal transmission, as result of paternal imprinting of transgene; thus, all mice analyzed inherited transgene from male parent
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growth/size/body
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• 8- to 12-week old males are underweight compared to wild-type littermates, whereas females show normal weights
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behavior/neurological
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• when placed in new cage, mutants are only ~40% as active as controls
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• in a hanging wire test, mutants hold on to cage top only ~half as long as controls at 8-12 weeks of age
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• at 12 weeks of age, average step distance is ~40% less than heterozygous or wild-type mice; gait widths are variable but average width is not significantly different from controls
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• in an activity test, mutants exhibit a baseline activity only ~54% of heterozygous and wild-type controls at 8- to 12-weeks of age
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nervous system
| N |
• no major structural abnormalities of the central nervous system are seen between mutants and controls in motor cortex, hippocampus, olfactory bulb, striatum and thalamic nuclei with respect to myelination and gross distribution and size of neuronal cell bodies
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• average number of hypoglossal motor neurons in brainstem is reduced compared to littermate controls
• however, motor neuron pools are not depleted in spinal cord or hypoglossal nucleus
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• in diaphragm muscle, 50% of endplates at neuromuscular synapse are fradmented compared to ~18% in control mice, while innervation of endplates is similar to control muscles
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muscle
| N |
• no gross abnormalities are seen in muscle tissue; no signs of muscle degeneration are observed
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homeostasis/metabolism
| N |
• mice show no abnormalities of serum markers used as indicators of liver and kidney function, such as serum glutamic pyruvic transaminase, glutamic oxaloacetic transaminase, and total protein levels, relative to wild-type; blood urea nitrogen (BUN) and creatinine levels, and BUN/creatinine ratios are normal compared to wild-type
• no gross structural abnormalities or serum clinical parameters indicative of organ (liver, kidney, heart) damage are seen in mutant animals
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