title: Tsc1 mutant neural stem/progenitor cells exhibit migration deficits and give rise to subependymal lesions in the lateral ventricle.
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.
authors.affiliation: Department of Developmental Biology, University of Texas Southwestern Medical Center, Dallas, Texas 75390, USA.
authors.forename: Jing
authors.initials: J
authors.lastname: Zhou
authors.forename: Gayatri
authors.initials: G
authors.lastname: Shrikhande
authors.forename: Jing
authors.initials: J
authors.lastname: Xu
authors.forename: Renée M
authors.initials: RM
authors.lastname: McKay
authors.forename: Dennis K
authors.initials: DK
authors.lastname: Burns
authors.forename: Jane E
authors.initials: JE
authors.lastname: Johnson
authors.forename: Luis F
authors.initials: LF
authors.lastname: Parada
mesh_terms: D000818:Animals; D001254:Astrocytoma / Q000473:pathology; D001932:Brain Neoplasms / Q000473:pathology; D002454:Cell Differentiation; D002465:Cell Movement / Q000235:genetics; D020547:Lateral Ventricles / Q000166:cytology / Q000473:pathology*; D051379:Mice; D018345:Mice, Knockout; D009154:Mutation / Q000235:genetics*; D058953:Neural Stem Cells / Q000166:cytology / Q000473:pathology*; D014402:Tuberous Sclerosis / Q000473:pathology; D000077004:Tuberous Sclerosis Complex 1 Protein; D025521:Tumor Suppressor Proteins / Q000235:genetics*
chemical_list: C000624651:Tsc1 protein, mouse; D000077004:Tuberous Sclerosis Complex 1 Protein; D025521:Tumor Suppressor Proteins