mortality/aging
• die immediately after birth of respiratory failure
|
hearing/vestibular/ear
• noticeably smaller and flattened
• posterior canal is obscured
|
• shortened and slightly widened
|
• 33% shorter
• decreased proliferation of prosensory cells, by 69% at E12.5 and by 54% at E13.5
|
• delayed development of hair cells and support cells
|
• but hair cell density is normal
|
• mis-oriented
|
• sometimes not aligned properly
• luminal surfaces are smaller
|
• lateral migration is delayed
|
• luminal surfaces are smaller
|
• truncated but lateral crista intact
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• hypomorphic but posterior crista are normal
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growth/size/body
• severe intrauterine growth deficiency
• embryos are only about 1/2 the size of heterozygotes
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respiratory system
• die immediately after birth of respiratory failure
|
nervous system
• delayed development of hair cells and support cells
|
• but hair cell density is normal
|
• mis-oriented
|
• sometimes not aligned properly
• luminal surfaces are smaller
|
• lateral migration is delayed
|
• luminal surfaces are smaller
|
cellular
• lateral migration is delayed
|
skeleton
• noticeably smaller and flattened
• posterior canal is obscured
|
craniofacial
• noticeably smaller and flattened
• posterior canal is obscured
|