Developmental origin of subependymal giant cell astrocytoma in tuberous sclerosis complex
Developmental origin of subependymal giant cell astrocytoma in tuberous sclerosis complex
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DOI：
10.1212/01.wnl.0000158653.81008.49
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发表时间：
2005-04-26
期刊：
影响因子：
9.9
通讯作者：
Gutmann, DH
中科院分区：
文献类型：
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作者：
Ess, KC;Kamp, CA;Gutmann, DH
患有结节性硬化症 (TSC) 的儿童存在大脑发育异常（皮质结节）和低度肿瘤（室管膜下巨细胞星形细胞瘤 [SEGAs]）。作者利用基因表达谱鉴定 Tsc1 条件敲除小鼠中的神经胶质细胞分化标记物，证明 SEGA 的巨细胞异常表达与皮质块茎相似的神经胶质细胞分化标记物。这些结果表明，块茎和 SEGA 都是由大脑发育过程中祖细胞分化的相关缺陷引起的。
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.