Ezrin and moesin expression within the developing human cerebrum and tuberous sclerosis-associated cortical tubers
Ezrin and moesin expression within the developing human cerebrum and tuberous sclerosis-associated cortical tubers
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DOI：
10.1007/s00401-002-0540-x
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发表时间：
2002-08-01
影响因子：
12.7
通讯作者：
Vinters, HV
中科院分区：
文献类型：
--
作者：
Johnson, MW;Miyata, H;Vinters, HV
ERM （ezrin， radixin和moesin）蛋白属于带-4.1超家族的膜-细胞骨架连接蛋白，通过其c端序列与肌动蛋白细胞骨架结合，并结合ERM结合膜蛋白（ermbps）。我们研究了两种ERM蛋白（ezrin和moesin）在发育中的人类大脑皮层和结节性硬化症（TSC）患者皮质块茎中的免疫组织化学表达，以评估TSC基因产物功能障碍或失活在发育中的大脑中与ERM蛋白表达相关的可能后果。Ezrin在产前人类大脑的放射状胶质细胞和中间区迁移细胞中大量表达，而moesin主要在发育和成人大脑的血管内皮细胞以及成人大脑的分散小胶质细胞中表达。此外，在tsc相关皮质块茎的异常细胞群中，ezrin和moesin都与hamartin和tuberin大量共表达。这两种蛋白（主要是ezrin）的表达表明，它们在大脑皮层发育过程中受到发育调节，并在生发基质和/或迁移细胞中大量表达。在tsc相关的皮质块茎中，这两种蛋白似乎都被上调，并在块茎中常见的异常神经胶质细胞群中共定位。在这些细胞中，这些蛋白的表达及其与tuberin和错构体的共定位可能表明对TSC基因突变的代偿性上调。
The ERM (ezrin, radixin, and moesin) proteins belong to the band-4.1 superfamily of membrane-cytoskeleton-linking proteins which bind to the actin cytoskeleton via their C-terminal sequences and bind ERM binding membrane proteins (ERMBMPs). We investigated the immunohistochemical expression of two of the ERM proteins (ezrin and moesin) in developing human cerebral cortex and in cortical tubers from patients with tuberous sclerosis (TSC), to assess possible consequences of TSC gene product malfunction or inactivation in the developing brain in relation to ERM protein expression. Ezrin is abundantly expressed within radial glia and migrating cells in the intermediate zone in the prenatal human cerebrum, while moesin is primarily expressed in vascular endothelial cells in developing and adult human brain and scattered microglia in adult brain. In addition, both ezrin and moesin are abundantly co-expressed with hamartin and tuberin within a population of abnormal cells in TSC-associated cortical tubers. The expression of these two proteins - primarily ezrin - suggests that they are developmentally regulated and abundantly expressed in germinal matrix and/or migrating cells during cerebral cortical development. In TSC-associated cortical tubers, both proteins appeared to be up-regulated and are co-localized within a population of abnormal neuroglial cells typical of those seen in tubers. Expression of these proteins and their co-localization with tuberin and hamartin in these cells may suggest a compensatory up-regulation in response to TSC gene mutation.