Analysis Tools
hearing/vestibular/ear
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• at P1, kinocilia are disconnected from the IHC stereociliary bundles and misplaced
• however, tip links are present in IHCs at P6
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• at P6, OHC kinocilium position is more variable, suggesting defects in kinociliary links
• at P1, kinocilia are disconnected from the OHC stereociliary bundles and misplaced
• kinocilium position fails to predict OHC bundle orientation, unlike in wild-type controls
• 48.2% of OHCs exhibit kinocilia that are mislocalized by 15 degrees or more; in some cases, kinocilium position deviates up to 90 degrees or more
• at P1, the two centrioles and kinocilium are coordinately mislocalized, and the two centrioles are not aligned properly relative to each other along the axis of bundle polarity
• however, tip links are present in OHCs at P6
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• at E15.5, P1 and P6, kinocilia are disconnected from the stereociliary bundles of vestibular hair cells in the utricle, saccule and semicircular canals
• however, tip links are present at P6
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• at P6, OHC stereociliary bundles maintain cohesion and staircase arrangement but show highly variable polarity defects; 4% percent of OHC bundles form circles
• 55% of OHCs deviate by 15 degrees or more from the normal position; in some cases, OHC bundle orientation deviates up to 90 degrees or more
• OHC bundles are only occasionally fragmented and abundant links are found between stereocilia, suggesting that transient lateral links are formed
• asymmetric localization of core planar cell polarity (PCP) proteins in OHCs is normal
• tip links are present and P7 OHCs show normal mechanotransduction currents in response to mechanical stimulation in vitro
• IHC bundle polarity is normal
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• at P96, mice show widespread degeneration of OHC bundles
• however, remaining OHC stereocilia form connections to the tectorial membrane (TM), as evident from the imprint of the longest stereocilia in the TM
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• at P96, mice show degeneration of hair bundles throughout the cochlea
• however, hair bundles are still present in some parts of the cochlea
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• at P96, mice show widespread degeneration of OHC bundles
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• at 1 and 6 months of age, auditory thresholds are increased to above 90 dB
• at 1 month of age, pure tone ABR recordings showed that hearing function is affected at all frequencies analyzed (4-32 kHz)
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• at 1 month of age, DPOAEs are not detectable at all frequencies analyzed (6-28 kHz)
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behavior/neurological
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• despite the absence of kinociliary links, homozygotes do not circle and show normal vestibular function, as assessed by the vestibulo-ocular reflex response
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nervous system
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• at P1, kinocilia are disconnected from the IHC stereociliary bundles and misplaced
• however, tip links are present in IHCs at P6
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• at P6, OHC kinocilium position is more variable, suggesting defects in kinociliary links
• at P1, kinocilia are disconnected from the OHC stereociliary bundles and misplaced
• kinocilium position fails to predict OHC bundle orientation, unlike in wild-type controls
• 48.2% of OHCs exhibit kinocilia that are mislocalized by 15 degrees or more; in some cases, kinocilium position deviates up to 90 degrees or more
• at P1, the two centrioles and kinocilium are coordinately mislocalized, and the two centrioles are not aligned properly relative to each other along the axis of bundle polarity
• however, tip links are present in OHCs at P6
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• at E15.5, P1 and P6, kinocilia are disconnected from the stereociliary bundles of vestibular hair cells in the utricle, saccule and semicircular canals
• however, tip links are present at P6
|
|
• at P6, OHC stereociliary bundles maintain cohesion and staircase arrangement but show highly variable polarity defects; 4% percent of OHC bundles form circles
• 55% of OHCs deviate by 15 degrees or more from the normal position; in some cases, OHC bundle orientation deviates up to 90 degrees or more
• OHC bundles are only occasionally fragmented and abundant links are found between stereocilia, suggesting that transient lateral links are formed
• asymmetric localization of core planar cell polarity (PCP) proteins in OHCs is normal
• tip links are present and P7 OHCs show normal mechanotransduction currents in response to mechanical stimulation in vitro
• IHC bundle polarity is normal
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• at P96, mice show widespread degeneration of OHC bundles
• however, remaining OHC stereocilia form connections to the tectorial membrane (TM), as evident from the imprint of the longest stereocilia in the TM
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• at P96, mice show degeneration of hair bundles throughout the cochlea
• however, hair bundles are still present in some parts of the cochlea
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• at P96, mice show widespread degeneration of OHC bundles
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cellular
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• at P1, kinocilia are disconnected from the IHC stereociliary bundles and misplaced
• however, tip links are present in IHCs at P6
|
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• at P6, OHC kinocilium position is more variable, suggesting defects in kinociliary links
• at P1, kinocilia are disconnected from the OHC stereociliary bundles and misplaced
• kinocilium position fails to predict OHC bundle orientation, unlike in wild-type controls
• 48.2% of OHCs exhibit kinocilia that are mislocalized by 15 degrees or more; in some cases, kinocilium position deviates up to 90 degrees or more
• at P1, the two centrioles and kinocilium are coordinately mislocalized, and the two centrioles are not aligned properly relative to each other along the axis of bundle polarity
• however, tip links are present in OHCs at P6
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• at E15.5, P1 and P6, kinocilia are disconnected from the stereociliary bundles of vestibular hair cells in the utricle, saccule and semicircular canals
• however, tip links are present at P6
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