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The role of CPEB family proteins in the nervous system function in the norm and pathology

机译:CPEB系列蛋白在规范和病理学中神经系统功能中的作用

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Posttranscriptional gene regulation includes mRNA transport, localization, translation, and regulation of mRNA stability. CPEB (cytoplasmic polyadenylation element binding) family proteins bind to specific sites within the 3′-untranslated region and mediate poly- and deadenylation of transcripts, activating or repressing protein synthesis. As part of ribonucleoprotein complexes, the CPEB proteins participate in mRNA transport and localization to different sub-cellular compartments. The CPEB proteins are evolutionarily conserved and have similar functions in vertebrates and invertebrates. In the nervous system, the CPEB proteins are involved in cell division, neural development, learning, and memory. Here we consider the functional features of these proteins in the nervous system of phylogenetically distant organisms: Drosophila, a well-studied model, and mammals. Disruption of the CPEB proteins functioning is associated with various pathologies, such as autism spectrum disorder and brain cancer. At the same time, CPEB gene regulation can provide for a recovery of the brain function in patients with fragile X syndrome and Huntington's disease, making the CPEB genes promising targets for gene therapy.
机译:后术语基因调节包括mRNA运输,定位,翻译和mRNA稳定的调节。 CPEB(细胞质多腺苷酸化元素结合)家族蛋白质与3'-未转过来的区域内的特异性位点结合,并介导转录物的多苯基化,活化或抑制蛋白质合成。作为核糖核糖蛋白复合物的一部分,CPEB蛋白参与MRNA转运和定位对不同的亚细胞室。 CPEB蛋白在进化上保存并在脊椎动物和无脊椎动物中具有类似的功能。在神经系统中,CPEB蛋白涉及细胞分裂,神经发展,学习和记忆。在这里,我们认为这些蛋白质的功能特征在阴影性遥远的生物体神经系统中:果蝇,学习良好的模型和哺乳动物。 CPEB蛋白质功能的破坏与各种病例相关,例如自闭症谱系障碍和脑癌。同时,CPEB基因调节可以提供易患X综合征和亨廷顿疾病患者的脑功能的恢复,使CPEB基因有前途的基因治疗目标。

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