Mouse Genome Informatics
ot
    Mecp2tm1Hzo/Y
involves: 129S7/SvEvBrd * C57BL/6J
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
mortality/aging
• most survive to at least one year of age, however about 10% die after 10 months of age (J:78009)

behavior/neurological
• 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 (J:78009)
• develop subtle tremors at around 6 weeks of age that worsen with age and are visibly apparent by 4 months of age (J:78009)
• young mice perform well on motor function tests but become deficient as they age (J:78009)
• 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 (J:78009)
• in a vertical pole test, mutants fall off the pole more readily than wild-type (J:78009)
• in a wire suspension test, mutants drop earlier than wild-type (J:78009)
• on a thin horizontal wooden dowel, mutants rapidly lose balance and fall off the dowel (J:78009)
• mutants rapidly and repetitively move their forelimbs, often bringing them together and sometimes holding them together for several seconds (J:78009)
• although overall level of rearing is not significantly lower, males rear less during the last 10 min interval of testing (J:78009)
• travel shorter distances and spend less time walking in the open-field test (J:78009)
• show normal activity during the first 10 min interval but reduced activity during the second and third 10 min time intervals (J:78009)
• mutants rapidly and repetitively move their forelimbs, often bringing them together and sometimes holding them together for several seconds (J:78009)
• in a tube test for social interaction, wild-type mice typically retreated when confronted with mutants, while mutants did not (J:78009)
• total time that wild-type intruder mice spent interacting with mutants is significantly shorter than the time spent interacting with wild-type mice (J:78009)
• spontaneous behavioral myoclonic jerks and seizures are seen in some mice after 8 months of age (J:78009)

nervous system
• spontaneous behavioral myoclonic jerks and seizures are seen in some mice after 8 months of age (J:78009)

cellular
• exhibit histone H3 hyperacetylation in the cerebellum, cerebral cortex, and spleen, indicating that chromatin architecture is abnormal (J:78009)

skeleton
• 40% develop kyphosis after 5 months of age (J:78009)

integument
• after 8 months of age, fur is noticeably more disheveled (J:78009)
• after 8 months of age, fur is noticeably more oily (J:78009)

Mouse Models of Human Disease
OMIM IDRef(s)
Rett Syndrome; RTT 312750 J:78009