Analysis Tools
mortality/aging
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• mice are born at normal ratios with no apparent defects but die within the first postnatal week
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growth/size/body
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• mice exhibit severe growth retardation relative to wild-type or heterozygous controls
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• mice show poor postnatal weight gain relative to controls
• however, feeding behavior is normal
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respiratory system
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• at P0, cilia are absent in tracheal epithelial cells, unlike in wild-type controls
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• at P0, mice exhibit increased numbers of mucus-secreting (Muc5ac-positive) cells in the trachea relative to wild-type controls
• in contrast, keratin 5-expressing basal progenitor cells appear unaffected
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• at P0, tracheal epithelial cells lack detectable cilia and exhibit only a couple of centrioles per cell, unlike in wild-type controls where multiple cilia are detected protruding from multiple basal bodies docked in the cell membrane
• mRNA expression of Mcidas and Foxj1 is severely reduced in tracheal epithelial cells at E16.5 and undetectable at P0, indicating that multiciliated cell differentiation is blocked at an early stage of airway development
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cellular
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• at P0, cilia are absent in tracheal epithelial cells, unlike in wild-type controls
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