Table 3.
Summary of the Animal Models of Tuberous Sclerosis Complex
| How model is created | Histology | Spontaneous seizure | |
|---|---|---|---|
| Eker rat | Spontaneous inactivating mutation of TSC2 | Subependymal and subcortical hamartomas | No spontaneous seizure |
| No cortical tuber | Decrease in fluorotyl seizure threshold after a second hit | ||
| TSC1 +/− | Mice with heterozygote deletion | Numbers of astrocytes is increased | No |
| TSC2 +/− | TSC2 +/− : altered ultrasonic vocalizations | ||
| TSC1GFAP CKO | Conditional KO mice, astroctytes | Increase of GFAP-positive cells increases | Yes 1–2 months |
| Ectopic neurons within the hippocampus | |||
| No tuber | |||
| TSC2GFAP CKO | Conditional KO mice, astrocytes | Diffuse glial proliferation, dispersion of hippocampal pyramidal cells | Yes 3-week-old and higher seizure frequency than TSC1GFAP CKO |
| No tuber | |||
| TSC1synapsin CKO | Conditional KO mice, neurons | Enlarged and/or dysplastic cortical and hippocampal neurons | Yes |
CKO = conditional Knock-out; KO = Knock-out