TDP-43 在 MND-FTLD 病理谱中的作用。
TDP-43 in the spectrum of MND-FTLD pathologies.
机构信息
Department of Neurology, Translational Neurotherapeutics Program, Laboratory for Dementia and Parkinsonism, Georgetown University Medical Center, Washington D.C. 20007, USA; Department of Pathology, Georgetown University Medical Center, Washington D.C., USA 20007.
Department of Neurology, Translational Neurotherapeutics Program, Laboratory for Dementia and Parkinsonism, Georgetown University Medical Center, Washington D.C. 20007, USA.
出版信息
Mol Cell Neurosci. 2017 Sep;83:46-54. doi: 10.1016/j.mcn.2017.07.001. Epub 2017 Jul 4.
The relationship between RNA-binding proteins, particularly TAR DNA binding protein 43 (TDP-43), and neurodegeneration is an important area of research. TDP-43 is involved in so many cellular processes that perturbation of protein homeostasis can lead to countless downstream effects. Understanding what leads to this disease-related protein imbalance and the resulting cellular and molecular effects will help to develop targets for disease intervention, whether it be prevention of protein accumulation, or addressing a secondary effect of protein accumulation. Here we review the current literature of TDP-43 and TDP-43 pathologies, the effects of TDP-43 overexpression and disruption of synaptic proteins through its binding of messenger RNA, leading to synaptic dysfunction. This review highlights some of the still-limited knowledge of the protein TDP-43 and how it can contribute to disease.
RNA 结合蛋白（尤其是 TAR DNA 结合蛋白 43，TDP-43）与神经退行性变之间的关系是一个重要的研究领域。TDP-43 参与了如此多的细胞过程，以至于蛋白质平衡的扰动可能导致无数的下游效应。了解导致这种与疾病相关的蛋白质失衡以及由此产生的细胞和分子效应将有助于确定疾病干预的靶点，无论是预防蛋白质积累，还是解决蛋白质积累的继发效应。在这里，我们回顾了 TDP-43 及其病理的最新文献，TDP-43 通过与信使 RNA 结合而过度表达和破坏突触蛋白，导致突触功能障碍。这篇综述强调了对蛋白质 TDP-43 的一些仍然有限的了解，以及它如何导致疾病。
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