Subcellular organization of UBE3A in neurons.

Subcellular organization of UBE3A in neurons.
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DOI:
10.1002/cne.24063
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发表时间:
2017-02-01
影响因子:
2.5
通讯作者:
Weinberg, Richard J.
Weinberg, Richard J.
中科院分区:
医学3区
文献类型:
--
作者:
Burette, Alain C.;Judson, Matthew C.;Burette, Susan;Phend, Kristen D.;Philpot, Benjamin D.;Weinberg, Richard J.

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泛素化调节广泛的细胞过程,泛素化缺陷与多种神经系统疾病有关。 E3 泛素蛋白连接酶 UBE3A 的缺失会导致天使综合征。尽管具有临床重要性,但 UBE3A 在神经元中的正常作用仍不清楚。作为破译其可能功能的一步,我们进行了高分辨率光和电子显微镜免疫细胞化学。我们报告了 UBE3A 在神经元中的广泛分布,突出显示在轴突末端和富含常染色质的核域中的浓度。我们的研究结果表明,UBE3A 可能在局部发挥作用来调节个体突触,同时还通过基因转录的调节来介导全局、神经元范围的影响。
Ubiquitination regulates a broad array of cellular processes, and defective ubiquitination is implicated in several neurological disorders. Loss of the E3 ubiquitin-protein ligase UBE3A causes Angelman syndrome. Despite its clinical importance, the normal role of UBE3A in neurons is still unclear. As a step toward deciphering its possible functions, we performed high-resolution light and electron microscopic immunocytochemistry. We report a broad distribution of UBE3A in neurons, highlighted by concentrations in axon terminals and euchromatin-rich nuclear domains. Our findings suggest that UBE3A may act locally to regulate individual synapses, while also mediating global, neuron-wide influences through the regulation of gene transcription.
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