Characterization of the NF2 protein merlin and the ERM protein ezrin in human, rat, and mouse central nervous system

Characterization of the NF2 protein merlin and the ERM protein ezrin in human, rat, and mouse central nervous system
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DOI:
10.1016/j.mcn.2004.11.014
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发表时间:
2005-04-01
影响因子:
3.5
通讯作者:
Carpén, O
Carpén, O
中科院分区:
医学3区
文献类型:
--
作者:
Grönholm, M;Teesalu, T;Carpén, O

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神经纤维瘤病 2 (NF2) 蛋白 merlin 在结构上与膜细胞骨架连接体的 ERM (ezrin-radixin-moesin) 蛋白家族相关,并且在神经系统肿瘤中发生突变。除了肿瘤抑制活性之外,merlin 的功能还知之甚少。我们比较了 merlin 和 ezrin 在发育中和成人大脑以及脑源性祖细胞中的定位和表达。这两种蛋白在人类、大鼠和小鼠大脑中广泛但存在差异表达。在脑组织和神经祖细胞培养物中,merlin 主要在神经元中发现,而ezrin 在星形胶质细胞中表达。 Merlin 在小鼠胚胎中的 Ell 中表达,而 ezrin 则更早出现。这两种蛋白均在胚胎小鼠神经球中表达,其中埃兹蛋白特异性地定位于贴壁神经元祖细胞的丝状伪足中。亚细胞分析表明,星形胶质细胞的细丝状足结构中存在埃兹蛋白,而神经元突触连接中检测到了梅林。 merlin 在大脑中的广泛表达及其与蛋白激酶 A 的关联表明 merlin 在大脑生物学中的作用。 (c) 2004 Elsevier Inc. 保留所有权利。
The neurofibromatosis 2 (NF2) protein, merlin, is structurally related to the ERM (ezrin-radixin-moesin) protein family of membrane-cytoskeleton linkers and is mutated in nervous system tumors. Apart from tumor suppressor activity, merlin's functions are poorly understood. We compared the localization and expression of merlin and ezrin in developing and adult brain and in brain-derived progenitor cells. Both proteins were widely but differentially expressed in human, rat, and mouse brain. In brain tissue and neuronal progenitor cell cultures merlin was predominantly found in neurons while ezrin was expressed in astrocytes. Merlin expression was seen from Ell in mouse embryos, whereas ezrin was present earlier. Both proteins were expressed in embryonic mouse neurospheres, where ezrin was specifically localized in filopodia of adherent neuronal progenitor cells. Subcellular analysis demonstrated ezrin in fine filopodial structures in astrocytes, while merlin was detected in neuronal synaptic junctions. The widespread expression of merlin in brain and its association with protein kinase A suggest a role for merlin in brain biology. (c) 2004 Elsevier Inc. All rights reserved.