Analysis Tools
vision/eye
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• mice exhibit abnormalities around the periciliary membrane complex and disorganized photoreceptors unlike in wild-type mice
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• at 28 to 33 months
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• light-adapted mice exhibit reduced b-wave amplitude compared with wild-type mice
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• dark-adapted mice exhibit reduced a- and b-waves compared to in wild-type mice
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hearing/vestibular/ear
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• at 2 months, mice exhibit only sporadic loss of hair cells in the mid-basal turn unlike wild-type mice
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• hair bundles of outer hair cells are compressed in the spiral dimension unlike in wild-type mice
• the angle between the two limbs of the V shape formation is smaller than in wild-type mice resulting in a U shape
• however, interstereocilia links of the outer hair cells are normal
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• mice exhibit patchy loss of the innermost (shortest) row of outer hair bundles compared with wild-type mice
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• at 2 and 9 months
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• profound
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nervous system
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• at 2 months, mice exhibit only sporadic loss of hair cells in the mid-basal turn unlike wild-type mice
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• hair bundles of outer hair cells are compressed in the spiral dimension unlike in wild-type mice
• the angle between the two limbs of the V shape formation is smaller than in wild-type mice resulting in a U shape
• however, interstereocilia links of the outer hair cells are normal
|
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• mice exhibit patchy loss of the innermost (shortest) row of outer hair bundles compared with wild-type mice
|
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• mice exhibit abnormalities around the periciliary membrane complex and disorganized photoreceptors unlike in wild-type mice
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| Usher syndrome type 2D | DOID:0110840 |
OMIM:611383 |
J:160677 | |
