Tanz Center for Research in Neurodegenerative Diseases, University of Toronto, Toronto, Ontario, Canada.
Epilepsia. 2012 Mar;53(3):e50-4. doi: 10.1111/j.1528-1167.2011.03369.x. Epub 2012 Jan 5.
Cerebral hyaline astrocytic inclusions have been observed in a subset of patients with early onset epilepsy, brain structural anomalies, and developmental delay, which indicates that it may represent a unique clinicopathologic entity. To further characterize this condition we use proteomics to investigate differentially expressed proteins in epileptic brain tissue from three pediatric epileptic patients with cerebral hyaline astrocytic inclusions, ranging in age from 5-13 years, and compare to brain tissue from two normal controls. Catalase and carbonic anhydrase I both exhibited increased expression in epileptic brain tissue compared to controls. These findings were confirmed by Western blot analysis. Furthermore, both proteins were localized to astrocytes and in epileptic brain were located within the cerebral hyaline astrocytic inclusions, suggesting a potential role in the generation of this pathologic feature of early onset epilepsy with cerebral hyaline astrocytic inclusions.
脑透明样星形细胞包涵体已在一组具有早发性癫痫、脑结构异常和发育迟缓的患者中观察到，这表明它可能代表一种独特的临床病理实体。为了进一步描述这种情况，我们使用蛋白质组学技术研究了来自 3 名年龄在 5-13 岁之间的具有脑透明样星形细胞包涵体的小儿癫痫患者的癫痫脑组织中的差异表达蛋白，并与来自 2 名正常对照的脑组织进行了比较。与对照组相比，过氧化氢酶和碳酸酐酶 I 在癫痫脑组织中的表达均增加。Western blot 分析证实了这一发现。此外，这两种蛋白质都定位于星形胶质细胞中，在癫痫脑组织中位于脑透明样星形细胞包涵体中，提示它们在早发性癫痫伴脑透明样星形细胞包涵体这一病理特征的产生中可能具有潜在作用。
