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
|
|
• hypomorphic but posterior crista are normal
|
growth/size/body
|
• severe intrauterine growth deficiency
• embryos are only about 1/2 the size of heterozygotes
|
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
|


Analysis Tools