Analysis Tools
mortality/aging
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• all double homozygous mutant pups die between P19 and P26; none survive beyond P26
• however, when fed a semisolid rodent chow softened in water for ~1 hr/d from P15 to P30, ~80-90% of ataxic pups survive to adulthood in the absence of competing littermates
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growth/size/body
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• by P7, double mutant pups are clearly smaller than control littermates
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• at P45 and P100, all surviving double mutants that have been fed a semisolid rodent chow reach only ~50% of the body weight of wild-type mice
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• double mutant pups gain less weight than control littermates and stop growing at ~P14
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weight loss
(
J:71179
)
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• at ~P14, double mutant pups start showing a gradual weight loss until they die
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behavior/neurological
| N |
• in spite of severe motor deficits, double mutant mice exhibit no obvious learning or memory deficit in the simple task of avoiding a foot shock relative to wild-type and double-heterozygous mice
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• all double mutant mice are highly sensitive to low doses of ethanol (0.5 mg/gm of body weight), showing an increase in sideways falls that reaches a plateau at 1.0 mg/gm; in contrast, wild-type and double-heterozygous mice show no sideways falls at 1.0 mg/gm but begin to show a few sideways falls at 2.0 mg/gm at a frequency similar to that of double homozygous mutants in the absence of ethanol
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• unlike wild-type mice which spend for most of their time in the corners of an open field with only brief excursions to the center, all double mutant mice spend more time along the edges and show a significant increase in center-field occupancy
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• all double mutant pups display tremulous movements
• all adult double mutants exhibit intermittent tremor-like episodes
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• all double mutant pups display some form of ataxia that prevents them from feeding properly
• all adult double mutants are severely ataxic
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• all double mutant pups exhibit poor balance when moving
• adult double mutants appear unbalanced when moving around the cage
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• all double mutant pups show motor incoordination
• adult double mutants appear uncoordinated when moving around the cage
• double mutants are unable to stay on either the rotating or stationary rod for more than a few seconds
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• when at rest, double mutant pups display sudden, brief muscle jerks
• adult double mutants exhibit whole-body jerks every few seconds; some are strong enough to lift the mouse off the cage floor
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• in spite severe motor deficits, double mutant mice show a significant increase in spontaneous locomotor activity in the open field relative to wild-type controls
• increased spontaneous locomotion is directly related to a significant increase in ambulatory time during the 60 min test period; however, the traveling speed, remains unchanged
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• all adult double mutants display spontaneous, brief and involuntary muscle contractions (myoclonus)
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nervous system
| N |
• strikingly, adult double mutants display normal gross brain anatomy, with no obvious alterations in cerebellar and hippocampal cytoarchitecture or in cerebellar foliation relative to wild-type controls
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• all adult double mutants display spontaneous, brief and involuntary muscle contractions (myoclonus)
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muscle
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• all adult double mutants display spontaneous, brief and involuntary muscle contractions (myoclonus)
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homeostasis/metabolism
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• at 2-3 days prior to death, the body temperature of double mutant pups begins to drop from ~38 degrees C to as low as ~25-28 degress C on the day of death
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