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Phenotypes Associated with This Genotype
Genotype
MGI:5502689
Allelic
Composition
Tg(Tek-tTA)1Rwng/0
Tg(tetO-Notch4*)1Rwng/0
Genetic
Background
involves: FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• die between 2 and 5 weeks of age (J:139943)
• die between 2 and 5 weeks of age (J:139943)

behavior/neurological
• ataxia ranging in severity from mice being unable to right themselves to those with splayed rear legs (J:139943)
• ataxia ranging in severity from mice being unable to right themselves to those with splayed rear legs (J:139943)
• mice occasionally exhibit seizure episodes in which they run wildly before collapsing (J:139943)
• mice occasionally exhibit seizure episodes in which they run wildly before collapsing (J:139943)

cardiovascular system
• arteries, veins, and intervening vessels of the middle-cerebral vessels are enlarged (J:139943)
• vessel enlargement occurs before hemorrhage, neuronal cell death, or signs of neurological dysfunction (J:139943)
• vessel enlargement correlates with hemorrhage, with them most large vessels in the cerebellum, followed by neocortex (J:139943)
• vessel density is decreased significantly in the cerebellum, and progressively less so in the neocortex and brainstem (J:139943)
• arteries, veins, and intervening vessels of the middle-cerebral vessels are enlarged (J:139943)
• vessel enlargement occurs before hemorrhage, neuronal cell death, or signs of neurological dysfunction (J:139943)
• vessel enlargement correlates with hemorrhage, with them most large vessels in the cerebellum, followed by neocortex (J:139943)
• vessel density is decreased significantly in the cerebellum, and progressively less so in the neocortex and brainstem (J:139943)
• enlarged and tangled vessels resembling brain arteriovenous malformations (J:139943)
• arteriovenous shunts in the brain (J:139943)
• enlarged and tangled vessels resembling brain arteriovenous malformations (J:139943)
• arteriovenous shunts in the brain (J:139943)
• hemorrhage in the brain of all mutants, most often in the cerebellum, followed by the neocortex, but never in the brainstem (J:139943)
• hemorrhage is more widespread than neurological dysfunction, seen in all ill mutants without ataxia and is most severe in mice ill before P22 (J:139943)
• hemorrhage in the brain of all mutants, most often in the cerebellum, followed by the neocortex, but never in the brainstem (J:139943)
• hemorrhage is more widespread than neurological dysfunction, seen in all ill mutants without ataxia and is most severe in mice ill before P22 (J:139943)
• blood is seen in the ventricle of 4 of 11 ill mice before P21 (J:139943)
• blood is seen in the ventricle of 4 of 11 ill mice before P21 (J:139943)
• increase in carotid blood velocity in all mutants by 3 weeks of age (J:139943)
• carotid blood velocity increases between P17 and P21 and at P21, velocity in mutants with neurological dysfunction is higher than mutants without the dysfunction (J:139943)
• increase in carotid blood velocity in all mutants by 3 weeks of age (J:139943)
• carotid blood velocity increases between P17 and P21 and at P21, velocity in mutants with neurological dysfunction is higher than mutants without the dysfunction (J:139943)

homeostasis/metabolism
• thrombosis is seen in hemorrhagic areas (J:139943)
• thrombosis is seen in hemorrhagic areas (J:139943)

nervous system
• signs of calcification in the brain (J:139943)
• signs of calcification in the brain (J:139943)
• arteries, veins, and intervening vessels of the middle-cerebral vessels are enlarged (J:139943)
• vessel enlargement occurs before hemorrhage, neuronal cell death, or signs of neurological dysfunction (J:139943)
• vessel enlargement correlates with hemorrhage, with them most large vessels in the cerebellum, followed by neocortex (J:139943)
• vessel density is decreased significantly in the cerebellum, and progressively less so in the neocortex and brainstem (J:139943)
• arteries, veins, and intervening vessels of the middle-cerebral vessels are enlarged (J:139943)
• vessel enlargement occurs before hemorrhage, neuronal cell death, or signs of neurological dysfunction (J:139943)
• vessel enlargement correlates with hemorrhage, with them most large vessels in the cerebellum, followed by neocortex (J:139943)
• vessel density is decreased significantly in the cerebellum, and progressively less so in the neocortex and brainstem (J:139943)
• foci of pyknotic and karyorectic nuclei in the midbrain (J:139943)
• macrophage infiltrations in the midbrain (J:139943)
• foci of pyknotic and karyorectic nuclei in the midbrain (J:139943)
• macrophage infiltrations in the midbrain (J:139943)
• foci of pyknotic and karyorectic nuclei in the neocortex (J:139943)
• macrophage infiltrations in the neocortex (J:139943)
• foci of pyknotic and karyorectic nuclei in the neocortex (J:139943)
• macrophage infiltrations in the neocortex (J:139943)
• foci of pyknotic and karyorectic nuclei in the Purkinje and granular layers of the cerebellum (J:139943)
• macrophage infiltrations in the Purkinje and granular layers of the cerebellum (J:139943)
• foci of pyknotic and karyorectic nuclei in the Purkinje and granular layers of the cerebellum (J:139943)
• macrophage infiltrations in the Purkinje and granular layers of the cerebellum (J:139943)
• neurological dysfunction is seen in 27 of 107 mutants, including ataxia and seizures (J:139943)
• all but one of the cases of ataxia and seizure occur before P22 (J:139943)
• sick mutants at P20-21 treated with doxycycline to repress Notch4 expression appear healthy and active at P35 and P64 (J:139943)
• neurological dysfunction is seen in 27 of 107 mutants, including ataxia and seizures (J:139943)
• all but one of the cases of ataxia and seizure occur before P22 (J:139943)
• sick mutants at P20-21 treated with doxycycline to repress Notch4 expression appear healthy and active at P35 and P64 (J:139943)
• mice occasionally exhibit seizure episodes in which they run wildly before collapsing (J:139943)
• mice occasionally exhibit seizure episodes in which they run wildly before collapsing (J:139943)
• blood is seen in the ventricle of 4 of 11 ill mice before P21 (J:139943)
• blood is seen in the ventricle of 4 of 11 ill mice before P21 (J:139943)
• neuronal cell death adjacent to hemorrhage (J:139943)
• neuronal cell death adjacent to hemorrhage (J:139943)

cellular
• neuronal cell death adjacent to hemorrhage (J:139943)
• neuronal cell death adjacent to hemorrhage (J:139943)

Mouse Models of Human Disease
OMIM ID Ref(s)
Arteriovenous Malformations of the Brain 108010 J:139943


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last database update
02/02/2016
MGI 6.02
The Jackson Laboratory