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. 2012 Mar 31;9(2):245–261. doi: 10.1007/s13311-012-0119-8

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