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The tish (telencephalic internal structural heterotopia) rat is a naturally occurring and unique model of a malformation of cortical development (MCD) arising from a sponeantous mutation in the Eml1 gene. Tish rats are characterized by a macroscopic bilateral heterotopic dysplastic cortex (HDCx) and an overlaying, intact normotopic neocortex (NNCx). These two cortices are functional and have been reported to innervate and establish connections with subcortical regions including the thalamus, resulting in a dual-cortical representation. Additionally, impaired GABAergic neurotransmission and early-onset spike wave discharge bursts have been reported in developing tish rats. Perineuronal nets (PNNs) are specialized extraceullar matrix structures that predominately surround and stabilize parvalbumin-positive (PV |
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