Tsc1 mutant neural stem/progenitor cells exhibit migration deficits and give rise to subependymal lesions in the lateral ventricle
- Jing Zhou1,
- Gayatri Shrikhande1,
- Jing Xu1,
- Renée M. McKay1,
- Dennis K. Burns2,
- Jane E. Johnson3 and
- Luis F. Parada1,4
- 1Department of Developmental Biology, University of Texas Southwestern Medical Center, Dallas, Texas 75390, USA;
- 2Department of Pathology, University of Texas Southwestern Medical Center, Dallas, Texas 75390, USA;
- 3Department of Neuroscience, 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)
Footnotes
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↵4 Corresponding author.
E-mail luis.parada{at}utsouthwestern.edu.
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Supplemental material is available for this article.
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Article is online at http://www.genesdev.org/cgi/doi/10.1101/gad.16750211.
- Received April 6, 2011.
- Accepted July 1, 2011.
- Copyright © 2011 by Cold Spring Harbor Laboratory Press
