Analysis Tools
mortality/aging
| N |
• mice exhibit a normal lifespan
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behavior/neurological
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• mice exhibit episodic periods of extended freezing behavior, piloerection, and a hunched posture unlike wild-type mice
• however, mice exhibit normal gait, limb strength, and vestibular-dependent behaviors
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• anesthetized mice exhibit dystonic postures and a longer recovery time compared with wild-type mice
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• at 5 to 6 months, mice exhibit increased latency to enter the light half of a light dark chamber compared with wild-type mice
• at 6 weeks, mice exhibit increase in the frequency of freezing in a clickbox test compared with wild-type mice
• at 10 weeks, mice exhibit increase in duration of freezing in a clickbox test compared with wild-type mice
• however, behavior in an open field is normal
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• when suspended by their tails, mice exhibit trunk circling behavior unlike similarly treated wild-type mice
• beginning at 10 weeks, toe-pinch reflex is impaired compared to in wild-type mice
• however, mice exhibit normal corneal, pinna, and reaching reflexes
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• mice exhibit decreased startle reflex compared with wild-type mice at greater penetrance than in homozygous mice that worsens with age
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• as early as P8, mice exhibit continuous tremors that increase in severity with age and a dominant episodic tremor with behavioral arrest unlike wild-type mice
• tremors during freezing period are increased in severity and amplitude
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• in a wire maneuver test
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hearing/vestibular/ear
| N |
• outer, middle, and inner ears are morphologically normal
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• the waveform of ABR is abnormal compared to controls
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• between 8 and 26 kHz, mice exhibit increased auditory brainstem response (ABR) threshold compared with wild-type mice
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• mild semi-dominant
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nervous system
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• slightly
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• slightly
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growth/size/body
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• as early as 6 weeks
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