Analysis Tools|
Allele Symbol Allele Name Allele ID |
Scn8aClth cloth-ears MGI:3827095 |
| Summary |
4 genotypes |
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
| N |
• mice exhibit normal vestibular function, no evidence of otitis media and normal cochlear hair cell morphology and function
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• more so than in heterozygotes
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• response to the click-box
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
| N |
• mice exhibit a normal lifespan
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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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| 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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• slightly
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• slightly
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• as early as 6 weeks
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
| N |
• mice exhibit normal vestibular function, no evidence of otitis media and normal cochlear hair cell morphology and function
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• mild to absent response to the click-box
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• mild to absent response to the click-box
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• as early as P25, some mice exhibit episodic periods of extended freezing behavior, piloerection, a hunched posture, and intermittent side-to-side coarse tremors unlike wild-type mice
• however, mice exhibit normal vestibular-dependent behaviors
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• Peyer's reflex is mild or absent unlike in wild-type mice
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• in some mice
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• at 5 weeks of age, startle reflex is mild or absent compared to in wild-type mice
• impaired startle reflex worsens with age
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• mice exhibit episodic tremors that increase in frequency and severity with age unlike wild-type mice
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| N |
• outer, middle, and inner ears are morphologically normal
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• mild semi-dominant
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• as early as 18 weeks
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Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO) |
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last database update 11/12/2024 MGI 6.24 |
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