Analysis Tools
mortality/aging
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• 8 of 28 mice died before P60
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growth/size/body
|
• 57.7% of mice exhibit small body size
|
heterotaxia
(
J:297786
)
|
• mice exhibit left-right asymmetry (LRA) abnormalities including situs inversus totalis
|
|
• stomach on right side (12.5%)
|
|
• reversed lung lobation (16.7%)
|
|
• mice exhibit situs inversus totalis with the heart malpositioned on the right side
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nervous system
hydrocephaly
(
J:297786
)
|
• 82.4% of mice exhibit hydrocephalus with a characteristic dome-shaped crown
• three males (P40, P42, and P59) and three females (P41, P42, and P47) showed hydrocephalus
|
craniofacial
|
• 82.4% of mice exhibit hydrocephalus with a characteristic dome-shaped crown
|
embryo
|
• high-speed videomicroscopy analysis (HVMA) of nodal ciliary beating revealed that mean rotational speed per second is significantly reduced, leading to impaired fluid flow at the left-right organizer
|
cardiovascular system
|
• aortic arch on right side (8.3%)
|
|
• azygos vein located on the right side (20.8%)
|
|
• vena cava located to the left of aorta (29.2%)
|
dextrocardia
(
J:297786
)
|
• dextrocardia (8.3%)
|
respiratory system
|
• reversed lung lobation (16.7%)
|
digestive/alimentary system
|
• stomach on right side (12.5%)
|
liver/biliary system
|
• abnormal liver lobation (25%)
|
reproductive system
| N |
• female mice exhibit normal fertility
• despite male infertility, sperm flagellar axonemes show normal ultrastructure in the midpiece (outer dense fiber) and principal piece (fibrous sheath) regions, respectively
|
 |
• sperm motility is significantly reduced; mean curvilinear velocity (microns per second) is significantly lower than that in heterozygous controls
• mean percentage of motile sperm is ~32.8% relative to ~76.7% in heterozygous controls
• whereas administration of 1 mM ATP alone does not significantly rescue microtubule sliding, a combination of either 4 mM AMP or 4 mM ADP with 1 mM ATP supports partial rescue of microtubule sliding in dyskinetic sperm; however, partial rescue is not observed using 1:1 molar ratios of ATP with either AMP or ADP
|
|
|
• sperm flagellar waveforms show reduced curvature and amplitude under conditions inducing hyperactivation
|
|
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• male mice are infertile
|
cellular
|
|
• sperm motility is significantly reduced; mean curvilinear velocity (microns per second) is significantly lower than that in heterozygous controls
• mean percentage of motile sperm is ~32.8% relative to ~76.7% in heterozygous controls
• whereas administration of 1 mM ATP alone does not significantly rescue microtubule sliding, a combination of either 4 mM AMP or 4 mM ADP with 1 mM ATP supports partial rescue of microtubule sliding in dyskinetic sperm; however, partial rescue is not observed using 1:1 molar ratios of ATP with either AMP or ADP
|
|
|
• sperm flagellar waveforms show reduced curvature and amplitude under conditions inducing hyperactivation
|
|
• high-speed videomicroscopy analysis (HVMA) of nodal ciliary beating revealed that mean rotational speed per second is significantly reduced, leading to impaired fluid flow at the left-right organizer
|
skeleton
|
• 82.4% of mice exhibit hydrocephalus with a characteristic dome-shaped crown
|
