Table 2:
Summary of circuit dysfunctions in Rett syndrome mouse model
| Asymptomatic 2 weeks | Pre-symptomatic 4 –5 weeks | Symptomatic 8 weeks | ||
|---|---|---|---|---|
| Hippocampus | In vivo | Reduced theta frequency during locomotion (D'Cruz et al. 2010) CA1 hypersynchrony in vivo & in vitro (Lu et a 1.2016) Impaired place field stability in familiar env at P90 (Kee et al. 2018) |
||
| In vitor | Normal LTP and LTD (Asaka et al. 2006) | Increased hippocampal excitability (Zhang et al. 2008; Calfa et al. 2011) Reduced FR in PVBC+ in area CA3 due to decreased glutaminergic drive (Calfa etal.2015) Decreased LTP and absent LTD (Asaka et al. 2006; Chao et al. 2010) |
||
| Sensory-motor cortex | Anatomy | Normal cortical thickness (Fukuda et al. 2005) Absence of PV cells (Fukuda et al 2005) |
No difference in PV+ density (Morello et al. 2018) Reduced cortical thickness (mostly in layer ll/lll) (Fukuda etal. 2005) |
Increased PV+expression in upper layers (Morelloetal. 2018) Normal PV+ density (Fukuda etal. 2005) Increased excitatory input to PV+ INs (Morello etal 2018) Reduced cortical thickness (mostly in layer ll/lll) (Fukuda et al. 2005) Reduced dendritic complexity in PC in layer ll/lll (Fukuda et. 2005) |
| In vitro | 2-fold FR reduction In PC in layer V (Dani et al. 2005) | 4-fold FR reduction in PC in layer V (Dani etal. 2005) Increased inhibitory & decreased excitatory drive into PC in layer V (Dani etal 2005) |
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FR: firing rate; env: environment; PC: principal cells