mortality/aging
• 50% of mice die by 16 weeks of age
|
behavior/neurological
• mice exhibit RTT-like symptoms after 5 weeks of age
|
• impaired motor learning on a rotarod
|
• in an elevated zero maze
|
• at 13 weeks of age
|
• on a rotarod
• not as severe as in Mecp2tm1.1Jae hemizygotes
|
• with splaying hind limbs upon movement
|
• at 11, but not 3, weeks of age
• not as severe as in Mecp2tm1.1Jae hemizygotes
|
nervous system
• at P30 and P90
|
• at P30, mice exhibit reduction in event-related power in delta, sigma and alpha low-frequencies compared with wild-type mice
• at P30, mice exhibit less of an increase in phase-locking factor at delta and high gamma frequencies compared with wild-type mice
• at P90, awake mice exhibit increased high-gamma frequency oscillation power compared with wild-type mice
• at P90, mice exhibit an increase in latency of P1, N1 and P2 peaks and reduction in the amplitudes of the N1 and P2 peaks of event-related potential compared with wild-type mice
• at P90, mice exhibit attenuated event-related power in both low- and high-frequency oscillation compared with wild-type mice
• at P90, mice exhibit less of an increase in phase-locking factor at all frequencies compared with wild-type mice
• however, mice exhibit normal power and event-related potential at P30
|
growth/size/body
• between 4 and 8 weeks of age
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
Rett syndrome | DOID:1206 |
OMIM:312750 OMIM:613454 |
J:181311 |