Developmental origin of subependymal giant cell astrocytoma in tuberous sclerosis complex.
Children with tuberous sclerosis complex (TSC) harbor developmental brain abnormalities (cortical tubers) and low-grade tumors (subependymal giant cell astrocytomas [SEGAs]). Using gene expression profiling to identify neuroglial differentiation markers in Tsc1 conditional knockout mice, the authors demonstrate that giant cells of SEGAs aberrantly express similar neuroglial differentiation markers as do cortical tubers. These results suggest that both tubers and SEGAs result from related defects in progenitor cell differentiation during brain development.
Ess
Kevin C
KC
Department of Neurology, Washington University School of Medicine, St. Louis, MO 63110, USA.
Kamp
Christine A
CA
Tu
Benjamin P
BP
Gutmann
David H
DH
D000293:Adolescent; D000818:Animals; D001254:Astrocytoma / Q000235:genetics* / Q000378:metabolism* / Q000503:physiopathology; D001932:Brain Neoplasms / Q000235:genetics* / Q000378:metabolism* / Q000503:physiopathology; D002454:Cell Differentiation / Q000235:genetics*; D019070:Cell Lineage / Q000235:genetics; D002648:Child; D004195:Disease Models, Animal; D005260:Female; D020869:Gene Expression Profiling; D018507:Gene Expression Regulation, Developmental / Q000235:genetics; D005819:Genetic Markers / Q000235:genetics; D006801:Humans; D007223:Infant; D008297:Male; D051379:Mice; D018345:Mice, Knockout; D009419:Nerve Tissue Proteins / Q000235:genetics; D009474:Neurons / Q000378:metabolism; D013234:Stem Cells / Q000378:metabolism; D014402:Tuberous Sclerosis / Q000235:genetics* / Q000378:metabolism* / Q000503:physiopathology; D000077004:Tuberous Sclerosis Complex 1 Protein; D025521:Tumor Suppressor Proteins / Q000235:genetics
D005819:Genetic Markers; D009419:Nerve Tissue Proteins; C000624650:TSC1 protein, human; C000624651:Tsc1 protein, mouse; D000077004:Tuberous Sclerosis Complex 1 Protein; D025521:Tumor Suppressor Proteins