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Tsc1 mutant neural stem/progenitor cells exhibit migration deficits and give rise to subependymal lesions in the lateral ventricle
Authors:Zhou Jing  Shrikhande Gayatri  Xu Jing  McKay Renée M  Burns Dennis K  Johnson Jane E  Parada Luis F
Institution:Department of Developmental Biology, University of Texas Southwestern Medical Center, Dallas, Texas 75390, USA.
Abstract:Subependymal nodules (SENs) and subependymal giant cell astrocytomas (SEGAs) are common brain lesions found in patients with tuberous sclerosis complex (TSC). These brain lesions present a mixed glioneuronal phenotype and have been hypothesized to originate from neural stem cells. However, this hypothesis has not been tested empirically. Here, we report that loss of Tsc1 in mouse subventricular zone (SVZ) neural stem/progenitor cells (NSPCs) results in formation of SEN- and SEGA-like structural abnormalities in the lateral ventricle, the consequence of abnormal migration of NSPCs following Tsc1 loss.
Keywords:neural stem/progenitor cells  subependymal giant cell astrocytomas (SEGAs)  subependymal nodules (SENs)  tuberous sclerosis complex (TSC)  lateral ventricle (LV)
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