Tsc2 null murine neuroepithelial cells are a model for human tuber giant cells, and show activation of an mTOR pathway

H Onda, PB Crino, H Zhang, RD Murphey… - Molecular and Cellular …, 2002 - Elsevier
Cortical tubers are developmental brain malformations in the tuberous sclerosis complex
(TSC) that cause epilepsy and autism in TSC patients whose pathogenesis is uncertain.
Tsc2 null murine neuroepithelial progenitor (NEP) cells display persistent growth when
growth factors are withdrawn, express GFAP at high levels, and have reduced expression of
a set of early neuronal lineage markers. Tsc2 null NEP cells exhibit aberrant differentiation
into giant cells that express both βIII-tubulin and GFAP and that are morphologically similar …