4.7 Article

Tsc1 mutant neural stem/progenitor cells exhibit migration deficits and give rise to subependymal lesions in the lateral ventricle

Journal

GENES & DEVELOPMENT
Volume 25, Issue 15, Pages 1595-1600

Publisher

COLD SPRING HARBOR LAB PRESS, PUBLICATIONS DEPT
DOI: 10.1101/gad.16750211

Keywords

neural stem/progenitor cells; subependymal giant cell astrocytomas (SEGAs); subependymal nodules (SENs); tuberous sclerosis complex (TSC); lateral ventricle (LV)

Funding

  1. American Cancer Society

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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.

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