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

J Zhou, G Shrikhande, J Xu, RM McKay… - Genes & …, 2011 - genesdev.cshlp.org
J Zhou, G Shrikhande, J Xu, RM McKay, DK Burns, JE Johnson, LF Parada
Genes & development, 2011genesdev.cshlp.org
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 …
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.
genesdev.cshlp.org