Analysis Tools
hearing/vestibular/ear
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• in 7 weeks old homozygous mice, stereocilia were absent from the second and third row of outer hair cells
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• in older homozygous mutants
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• in 7 weeks old homozygous mice, inner hair cell and their afferent terminals were present although outer hair cells appeared apoptotic
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• in organotypic cochlear cultures, the sensitivity of mechanoelectrical transduction channels to hair bundle displacement in outer cochlear hair cells is reduced
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• organotypic cultures of cochleae from P0-P3 mice (studied at P1-P4) indicate that, although the amplitude and kinetics of transduction currents in mutant OHCs from the first row of the mid-apical turn are qualitatively similar to those of wild-type controls, the curves relating bundle displacement (X) to channel opening probability, Popen (X) are shifted positively with respect to controls
• this shift is less pronounced in Atp2b2dfw mice (117 nm) than in Atp2b2tm1Ges mice (175 nm)
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• in organotypic utricular cultures from P0-P3 mice (studied at P1-P4), Ca2+ imaging of vestibular hair cells indicates that dissipation of stereociliary Ca2+ transients induced by Ca2+ uncaging is compromised
• as a result, the Ca2+ clearing rate of stereocilia is decreased and therefore similar to the slower rate of cell soma
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• responses were absent at al f2s at experimental intensity
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• ABR are absent indicating that they are profoundly deaf
(J:108888)
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behavior/neurological
head bobbing
(
J:14114
)
abnormal gait
(
J:14114
)
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• walk with a hesitant and wobbly gait
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nervous system
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• in 7 weeks old homozygous mice, stereocilia were absent from the second and third row of outer hair cells
|
|
• in older homozygous mutants
|
|
• in 7 weeks old homozygous mice, inner hair cell and their afferent terminals were present although outer hair cells appeared apoptotic
|
|
• in organotypic cochlear cultures, the sensitivity of mechanoelectrical transduction channels to hair bundle displacement in outer cochlear hair cells is reduced
|
|
• organotypic cultures of cochleae from P0-P3 mice (studied at P1-P4) indicate that, although the amplitude and kinetics of transduction currents in mutant OHCs from the first row of the mid-apical turn are qualitatively similar to those of wild-type controls, the curves relating bundle displacement (X) to channel opening probability, Popen (X) are shifted positively with respect to controls
• this shift is less pronounced in Atp2b2dfw mice (117 nm) than in Atp2b2tm1Ges mice (175 nm)
|
|
• in organotypic utricular cultures from P0-P3 mice (studied at P1-P4), Ca2+ imaging of vestibular hair cells indicates that dissipation of stereociliary Ca2+ transients induced by Ca2+ uncaging is compromised
• as a result, the Ca2+ clearing rate of stereocilia is decreased and therefore similar to the slower rate of cell soma
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• in anteroventral cochlear nucleus, spherical cells soma size was smaller
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• in 7 weeks old homozygous mice
• in older homozygous mutants, remaining ganglion cells contained very few cytoplasmic organelles
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• in older homozygous mutants
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homeostasis/metabolism
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• in organotypic utricular cultures from P0-P3 mice (studied at P1-P4), Ca2+ imaging of vestibular hair cells indicates that dissipation of stereociliary Ca2+ transients induced by Ca2+ uncaging is compromised
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