额颞叶痴呆的小鼠模型。
Mouse models of frontotemporal dementia.
机构信息
Departments of Neurology and Neurobiology, Center for Neurodegeneration and Experimental Therapeutics, University of Alabama, Birmingham, AL 35294, USA.
出版信息
Ann Neurol. 2012 Dec;72(6):837-49. doi: 10.1002/ana.23722.
The pace of discovery in frontotemporal dementia (FTD) has accelerated dramatically with the discovery of new genetic causes and pathological substrates of the disease. MAPT/tau, GRN/progranulin, and C9ORF72 have emerged as common FTD genes, and TARDBP/TDP-43, VCP, FUS, and CHMP2B have been identified as less common genetic causes. TDP-43 and FUS have joined tau as common neuropathological substrates of the disease. Mouse models provide an important tool for understanding the role of these molecules in FTD pathogenesis. Here, we review recent progress with mouse models based on tau, TDP-43, progranulin, VCP, and CHMP2B. We also consider future prospects for FTD models, including developing new models to address unanswered questions. There are also opportunities for capitalizing on conservation of the salience network, which is selectively vulnerable in FTD, and the availability of FTD-related behavioral paradigms to analyze mouse models of the disease.
随着新的遗传病因和疾病病理基础的发现，额颞叶痴呆（FTD）的研究进展迅速加快。MAPT/tau、GRN/progranulin 和 C9ORF72 已成为常见的 FTD 基因，而 TARDBP/TDP-43、VCP、FUS 和 CHMP2B 则被确定为较少见的遗传病因。TDP-43 和 FUS 已与 tau 一起成为疾病的常见神经病理学基础。小鼠模型为理解这些分子在 FTD 发病机制中的作用提供了重要工具。在这里，我们综述了基于 tau、TDP-43、progranulin、VCP 和 CHMP2B 的最新小鼠模型研究进展。我们还考虑了 FTD 模型的未来前景，包括开发新模型以解决未解答的问题。利用 FTD 中选择性脆弱的突显网络的保守性以及与 FTD 相关的行为范式来分析疾病的小鼠模型，也存在着机会。
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