mortality/aging
• gradual decrease in survival after birth with only 20% surviving beyond weaning
|
digestive/alimentary system
• impaired
|
respiratory system
• the number of cilia per single multiciliated tracheal cell is reduced
• however, cilia length is similar to wild-type controls
• in tracheal cilia some basal bodies fail to position apically and the basal foot is absent
• in tracheal cilia the rotational polarity of axonemes is scattered
|
• the number of cilia per single multiciliated tracheal cell is reduced
|
• coordinated tracheal ciliary beating is disturbed with the direction of beating dispersed rather than in the lung to oral direction seen in wild-type mice
|
• in ex vivo bead assay the flow of fluorescent beads reveals only limited uncoordinated movement
|
• display a coughing/sneezing like phenotype
|
cellular
N |
• unexpectedly mice are born with primary cilia
|
• tendency toward a decrease in the number of cilia per cell in multiciliated cells in the oviduct and brain ventricle
• in cilia in the trachea, oviduct and brain ventricle basal feet are absent
|
• in cilia basal feet are absent
|
• tendency toward a decrease in the number of cilia per cell in multiciliated cells
|
• about 30% shorter than in wild-type controls
|
• tendency toward a decrease in the number of cilia per cell in multiciliated cells
• in cilia basal feet are absent
|
• the number of cilia per single multiciliated tracheal cell is reduced
• however, cilia length is similar to wild-type controls
• in tracheal cilia some basal bodies fail to position apically and the basal foot is absent
• in tracheal cilia the rotational polarity of axonemes is scattered
|
• the number of cilia per single multiciliated tracheal cell is reduced
|
• coordinated tracheal ciliary beating is disturbed with the direction of beating dispersed rather than in the lung to oral direction seen in wild-type mice
|
embryo
• about 30% shorter than in wild-type controls
|
growth/size/body
• about 70 - 80% of wild-type littermates
|
reproductive system
• tendency toward a decrease in the number of cilia per cell in multiciliated cells
• in cilia basal feet are absent
|
nervous system
• in cilia basal feet are absent
|
• tendency toward a decrease in the number of cilia per cell in multiciliated cells
|
hydrocephaly
(
J:181294
)
• slight tendency for hydrocephaly
|
immune system
N |
• no increases in inflammation levels in the lung are detected in mice kept in specific pathogen free conditions
|
hearing/vestibular/ear