Analysis Tools
mortality/aging
|
• at P28, homozygotes show a ~10% increase in lethality relative to wild-type controls
|
|
• homozygotes are born in normal Mendelian ratios but exhibit progressive postnatal lethality
|
growth/size/body
|
• mice are smaller in size relative to wild-type controls
|
|
• body weight is reduced at P28
|
nervous system
|
• mice show a complete loss of multiple ciliated cells (MCCs) in brain ependymal tissue
• MCCs differentiate a single cilium, unlike in wild-type controls; however, nuclear-localized FOXJ1 is detected in cells bearing single long cilium
• the length and width of the monocilium are similar to the multiple cilia of MCCs but different from the shorter, thinner primary cilia present on neighboring cells
|
hydrocephaly
(
J:274880
)
|
• mice exhibit hydrocephalus, suggesting dysfunctional ependymal multiciliated cells
|
|
• hydrocephalic mice show dilation of the lateral ventricles
|
|
• hydrocephalic mice show dilation of the third ventricle
|
reproductive system
 |
• mice show a complete loss of multiple ciliated cells (MCCs) in oviduct tissue
• MCCs differentiate a single cilium, unlike in wild-type controls
• the length and width of the monocilium are similar to the multiple cilia of MCCs but different from the shorter, thinner primary cilia present on neighboring cells
|
|
|
• all females fail to breed, when in-crossed or out-crossed
|
 |
• all males fail to breed, when in-crossed or out-crossed
|
respiratory system
| N |
• under pathogen-free (SPF) conditions, mice exhibit no obvious symptoms of airway disease at the behavioral or histopathology level
|
|
• mice show a complete loss of multiple ciliated cells (MCCs) in tracheal tissue
• MCCs differentiate a single cilium, unlike in wild-type controls; however, nuclear-localized FOXJ1 is detected in cells bearing single long cilium
• the length and width of the monocilium are similar to the multiple cilia of MCCs but different from the shorter, thinner primary cilia present on neighboring cells
• SEM analysis of the tracheal MCCs revealed that the single cilium has dimensions similar to an individual motile cilium of wild-type MCCs
• whereas multiple basal bodies are observed in wild-type MCCs, only a single basal body associated with the single cilium is detected by multiple marker and TEM analysis
|
cellular
|
• although multiple ciliated cell (MCC) precursors are specified normally, they fail to generate multiple basal bodies and thus differentiate a single cilium instead of multiple cilia; the single cilium localizes motile cilia-specific proteins (RSPH1, RSPH9, and CCDC40)
• deuterosomes (spherical, electron dense, cytoplasmic structures involved in de novo assembly of centrioles) are severely reduced in number and incapable of supporting centriole biogenesis
|
|
• mice show a complete loss of multiple ciliated cells (MCCs) in brain ependymal tissue
• MCCs differentiate a single cilium, unlike in wild-type controls; however, nuclear-localized FOXJ1 is detected in cells bearing single long cilium
• the length and width of the monocilium are similar to the multiple cilia of MCCs but different from the shorter, thinner primary cilia present on neighboring cells
|
|
|
• mice show a complete loss of multiple ciliated cells (MCCs) in oviduct tissue
• MCCs differentiate a single cilium, unlike in wild-type controls
• the length and width of the monocilium are similar to the multiple cilia of MCCs but different from the shorter, thinner primary cilia present on neighboring cells
|
|
• although multiple ciliated cell (MCC) precursors are specified normally, they fail to generate multiple basal bodies and thus differentiate a single cilium instead of multiple cilia; the single cilium localizes motile cilia-specific proteins (RSPH1, RSPH9, and CCDC40)
• deuterosomes (spherical, electron dense, cytoplasmic structures involved in de novo assembly of centrioles) are severely reduced in number and incapable of supporting centriole biogenesis
|
