Table 1:
Brain region/cell type targeted | Target gene | Mouse model | Life span | Body weight | Brain weight | Behavioral phenotype | Ref. | ||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Breath regularity | Motility | Hindlimb clasping | Motor coord. | Anxiety | Social Interact. | Learning/memory | E/I balance | Hippo LTP | |||||||
Whole body | Mecp2 | Mecp2 −/− or Mecp2 -/y (Mecp2-KO) | ↓ | ↓/← | ↓ | ↓ | ↓ | ↑ | ↓ | ↓ | ↑/↓ | ↓ | CA1↑ CA3↑ Bs↑Ctx↓ | – | [44–51] |
Mecp2R306C/y | ↓ | – | ↓ | – | ↓ | ↑ | ↓ | – | – | – | – | – | [29] | ||
Mecp2T308A/y | – | ← | ↓ | – | – | ↑ | ↓ | – | – | – | – | – | [28] | ||
Ncor1/2 | Ncor1Y478A/Y478A; Ncor2Y470A/Y470A (NS-DADm) | ← * | ← | ← | ← | ↑ | ← | ↑ | ↓ | ↓ | ↓ | – | – | [19] | |
Shank3 | Shank3−/− | – | ← | – | – | ←/↓ | – | ↓/← | ←/↑ | ↓ | ↓/← | CA1↑/← | ↓ | [132–136] | |
Mef2c | Mef2c+/− | ↓ | ↓ | – | – | ← | ↑ | – | ↑ | ↓ | ↓ | Hippo↑ | – | [165] | |
Foxp2 | Foxp2−/− | ↓ | ↓ | – | – | – | – | ↓ | – | ↓ | – | – | – | [152, 153] | |
Foxp2+/− | ← | ←/↓ | – | – | – | - | ← | – | ↓ | ← | – | – | [152, 153] | ||
GABA neuron | Mecp2 | Vgat-Cre; Mecp2loxP/y | ↓ | ↓ | – | ↓ | ↓ | ↑ | ↓ | ← | ↑ | ↓ | Ctx PN↑ Str GN↑ | ↓ | [52] |
Ncor1/2 | Vgat-Cre; Ncor1loxP/loxP; Ncor2loxP/loxP (NS-V) | – | – | – | – | ← | – | – | ← | ↓ | ↓ | Hypo↑ CA3↓ | ↓ | [19] | |
Shank3 | Vgat-Cre;Shank3loxP/loxP | – | – | – | – | ↓ | – | – | ↑ | ←/↓ | – | DL Str Neuron↓ | – | [140] | |
Forebrain neuron | Mecp2 | Camk2-Cre;Mecp2loxP/loxP | ↓ | ↑ | ↓ | ↓ | ↓/← | ← | ↓ | ↑ | ↑ | ↓ | – | – | [53, 54] |
Hdac3 | Camk2-Cre;Hdac3loxP/loxP | – | – | – | – | ↑ | ↑ | ↓ | – | ↓ | ↓ | – | – | [43] | |
Mef2c | Camk2-Cre;Mef2cloxP/loxP | – | – | – | – | ↑ | ↑ | ↓ | ← | ← | ← | ← | ← | [169] | |
Neural progenitor | Mef2c | Nestin-Cre;Mef2cloxP/- | ↓ | ↓ | ↓ | – | ← | ↑ | - | ↑ | – | ↓ | Ctx↓ | – | [166] |
hGFAP-Cre;Mef2cloxP/- | – | ↓ | ← | – | ← | ↑ | ↓ | ← | – | ↓ | – | – | [167] | ||
Ctx and Hippo excitatory neuron | Mef2c | Emx1-Cre;Mef2cloxP/loxP | – | – | – | – | ↑ | – | ← | – | ↓ | ↓ | Ctx↓ | – | [168] |
Foxp2 | Nex-Cre;Foxp2lop/loxP | – | ← | – | – | – | – | – | ← | ↓ | – | – | – | [154] | |
Hippo | Hdac3 | AAV-Cre into CA1 of Hdac3loxP/loxP | – | – | – | – | – | – | – | – | – | ↑ | – | – | [23] |
AAV-Cre into CA3 of Hdac3loxP/loxP | – | – | – | – | – | – | – | – | – | ← | – | – | [19] | ||
Mecp2 | AAV-shRNA at adult dorsal hippocampus | – | – | – | – | ← | – | – | ← | – | ↓ | – | – | [55] | |
Hypo | Hdac3 | AAV-Cre into Hdac3loxP/loxP | – | – | – | – | – | – | – | – | – | ↓ | – | – | [19] |
Mecp2 | Sim1-Cre; Mecp2loxP/y | ← | ↑ | – | – | ← | ↑ | ← | ↑ | ← | ← | – | – | [56] |
no change
decrease
increase
not determined; Bs, brainstem; Ctx, Cortex; Hippo, hippocampus; Hypo, hypothalamus; Str, striatum; PN, pyramidal neuron; DL, dorsolateral.
survive for at least 1 year.