About   Help   FAQ
Phenotypes Associated with This Genotype
Genotype
MGI:3624551
Allelic
Composition
Mecp2tm1Hzo/Y
Genetic
Background
involves: 129S7/SvEvBrd * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mecp2tm1Hzo mutation (1 available); any Mecp2 mutation (38 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• most survive to at least one year of age, however about 10% die after 10 months of age

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
• develop subtle tremors at around 6 weeks of age that worsen with age and are visibly apparent by 4 months of age
• 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
• mutants rapidly and repetitively move their forelimbs, often bringing them together and sometimes holding them together for several seconds
• although overall level of rearing is not significantly lower, males rear less during the last 10 min interval of testing
• 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
• mutants rapidly and repetitively move their forelimbs, often bringing them together and sometimes holding them together for several seconds
• 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
• spontaneous behavioral myoclonic jerks and seizures are seen in some mice after 8 months of age

nervous system
• spontaneous behavioral myoclonic jerks and seizures are seen in some mice after 8 months of age

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

skeleton
• 40% develop kyphosis after 5 months of age

integument
• after 8 months of age, fur is noticeably more disheveled
• after 8 months of age, fur is noticeably more oily

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Rett syndrome DOID:1206 OMIM:312750
OMIM:613454
J:78009


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
Citing These Resources
Funding Information
Warranty Disclaimer, Privacy Notice, Licensing, & Copyright
Send questions and comments to User Support.
last database update
04/16/2024
MGI 6.23
The Jackson Laboratory