Analysis Tools
mortality/aging
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• mice that survive birth die between 3 and 4 weeks of age with normal or reversed body situs and domed heads indicative of hydrocephaly
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• mice with heterotaxia exhibit heart defects and die before or shortly after birth
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growth/size/body
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• mice that survive birth die between 3 and 4 weeks of age with domed heads indicative of hydrocephaly
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heterotaxia
(
J:130755
)
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• 40% of mice exhibit heterotaxia, 36% situs invertus and 24% situs solitus
• mice with heterotaxia exhibit heart defects and die before or shortly after birth
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• in one mouse
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• in some mice
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• mice exhibit varying degrees of lung and bronchial tree left isomerism
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• 36% of mice exhibit situs inversus
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cardiovascular system
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• in some mice
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• in one mouse
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• inferior vena cava (IVC) abnormalities are observed in some mice including azygos continuation, interruption and duplication of the IVC
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• mice exhibit a range of cardiac defects at varying degrees of penetrance including tetralogy of Fallot, transposition of the great arteries, a common atrioventricular canal, dextro- or meso-cardia, a common atrium, double outlet right ventricle, and septal defects
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• some mice exhibit L ventricular loop with or without superior-inferior positioning of the ventricles
• some mice exhibit tetralogy of Fallot
• some mice exhibit a concordant vein and artery alignment in the great arteries
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• in some mice
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• in some mice
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• some mice exhibit a single atrioventricular valve of mitral morphology
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• in one mouse
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• in some mice
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• some mice exhibit a common atrium
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• some mice exhibit secundum atrial septal defects
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dextrocardia
(
J:130755
)
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• in some mice
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mesocardia
(
J:130755
)
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• in some mice
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• in some mice
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• some mice exhibit a common or unbalanced atrioventricular canal committed to the right or left ventricle
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• in some mice
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respiratory system
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• mice exhibit a wide range of tracheal cilia disorganization
• mice exhibit reduced numbers of outer dynein arms in their tracheal cilia compared to in wild-type mice
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• tracheal cilia are immotile or slow and dyskinetic
• displacement of fluorescent beads deposited above the epithelia is reduced in velocity and directionality compared to in wild-type mice
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• mice exhibit varying degrees of lung and bronchial tree left isomerism
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digestive/alimentary system
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• in some mice the pancreas or the stomach are mal-positioned along the midline or the right side
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hematopoietic system
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• in some mice the spleen is mal-positioned
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• in some mice
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nervous system
hydrocephaly
(
J:130755
)
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• mice that survive birth die between 3 and 4 weeks of age with domed heads indicative of hydrocephaly
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liver/biliary system
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• some mice exhibit a symmetric bilobed liver
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immune system
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• in some mice the spleen is mal-positioned
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• in some mice
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cellular
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• mice exhibit a wide range of tracheal cilia disorganization
• mice exhibit reduced numbers of outer dynein arms in their tracheal cilia compared to in wild-type mice
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• tracheal cilia are immotile or slow and dyskinetic
• displacement of fluorescent beads deposited above the epithelia is reduced in velocity and directionality compared to in wild-type mice
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| primary ciliary dyskinesia 3 | DOID:0110599 |
OMIM:608644 |
J:130755 | |
| tetralogy of Fallot | DOID:6419 |
OMIM:187500 |
J:130755 | |
