Phenotypes associated with this allele
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mecp2tm1Hzo mutation
(1 available);
any
Mecp2 mutation
(38 available)
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behavior/neurological
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• females display milder and more variable features of Rett Syndrome than males, presumably due to differences in the pattern of X chromosome inactivation
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• 62% of females exhibit tremors
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• in the wire suspension test, females show normal performance at 5-6 weeks of age, but are impaired at older ages (35-39 weeks)
• females perform as well as wild-type on a thin horizontal wooden dowel, even at 35-39 weeks of age
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• 69% of females display stereotypic forepaw movements (rapid and repetitive movement of the forelimbs, often bring them together)
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mecp2tm1Hzo mutation
(1 available);
any
Mecp2 mutation
(38 available)
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behavior/neurological
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• 100% of males display tremors
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• performance on a thin horizontal wooden dowel is impaired in older (35-36 weeks of age) but not younger (8-9 weeks of age) males
• in the wire suspension test, young males (8-9 weeks of age drop earlier than wild-type
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• 100% of males display stereotypic forepaw movements (rapid and repetitive movement of the forelimbs, often bring them together)
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Allelic
Composition |
Mecp2tm1Hzo/Y
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Genetic
Background |
involves: 129S7/SvEvBrd * C57BL/6J |
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mecp2tm1Hzo mutation
(1 available);
any
Mecp2 mutation
(38 available)
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mortality/aging
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• most survive to at least one year of age, however about 10% die after 10 months of age
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behavior/neurological
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• amount of exploratory behavior in the center of the open field does not change during testing like in wild-type mice which increase activity in the center space over time, likely reflecting heightened anxiety
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• develop subtle tremors at around 6 weeks of age that worsen with age and are visibly apparent by 4 months of age
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• young mice perform well on motor function tests but become deficient as they age
• exhibit a small impairment in the ability to stay on a modified rotarod (covered with duct tape to eliminate the grips), however do not exhibit defects in forepaw grip strength
• in a vertical pole test, mutants fall off the pole more readily than wild-type
• in a wire suspension test, mutants drop earlier than wild-type
• on a thin horizontal wooden dowel, mutants rapidly lose balance and fall off the dowel
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• mutants rapidly and repetitively move their forelimbs, often bringing them together and sometimes holding them together for several seconds
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• although overall level of rearing is not significantly lower, males rear less during the last 10 min interval of testing
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• travel shorter distances and spend less time walking in the open-field test
• show normal activity during the first 10 min interval but reduced activity during the second and third 10 min time intervals
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• mutants rapidly and repetitively move their forelimbs, often bringing them together and sometimes holding them together for several seconds
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• in a tube test for social interaction, wild-type mice typically retreated when confronted with mutants, while mutants did not
• total time that wild-type intruder mice spent interacting with mutants is significantly shorter than the time spent interacting with wild-type mice
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• spontaneous behavioral myoclonic jerks and seizures are seen in some mice after 8 months of age
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nervous system
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• spontaneous behavioral myoclonic jerks and seizures are seen in some mice after 8 months of age
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cellular
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• exhibit histone H3 hyperacetylation in the cerebellum, cerebral cortex, and spleen, indicating that chromatin architecture is abnormal
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skeleton
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• 40% develop kyphosis after 5 months of age
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integument
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• after 8 months of age, fur is noticeably more disheveled
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• after 8 months of age, fur is noticeably more oily
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Analysis Tools