核载体和 RNA 结合蛋白在伴有肉瘤融合（FUS）病理性改变的额颞叶变性中。
Nuclear carrier and RNA-binding proteins in frontotemporal lobar degeneration associated with fused in sarcoma (FUS) pathological changes.
机构信息
Mental Health and Neurodegeneration Research Group, Faculty of Human and Medical Sciences, University of Manchester, ManchesterCerebral Function Unit, Greater Manchester Neuroscience Centre, Salford Royal Foundation Trust, SalfordDepartment of Neuropathology, Walton Centre for Neurology and Neurosurgery, LiverpoolNeuropathology/Cellular Pathology, Royal Victoria InfirmaryInstitute for Ageing and Health, Campus for Ageing and Vitality, Newcastle University, Newcastle upon Tyne, UKNorthwestern CNADC Neuropathology Core, Northwestern University Feinberg School of Medicine, Chicago, Illinois, Departments ofNeurologyPathology & Immunology, Washington University School of Medicine, St Louis, Missouri, USA.
出版信息
Neuropathol Appl Neurobiol. 2013 Feb;39(2):157-65. doi: 10.1111/j.1365-2990.2012.01274.x.
AIMS
We aimed to investigate the role of the nuclear carrier and binding proteins, transportin 1 (TRN1) and transportin 2 (TRN2), TATA-binding protein-associated factor 15 (TAF15) and Ewing's sarcoma protein (EWS) in inclusion body formation in cases of frontotemporal lobar degeneration (FTLD) associated with fused in sarcoma protein (FTLD-FUS).
METHODS
Eight cases of FTLD-FUS (five cases of atypical FTLD-U, two of neuronal intermediate filament inclusion body disease and one of basophilic inclusion body disease) were immunostained for FUS, TRN1, TRN2, TAF15 and EWS. Ten cases of FTLD associated with TDP-43 inclusions served as reference cases.
RESULTS
The inclusion bodies in FTLD-FUS contained TRN1 and TAF15 and, to a lesser extent, EWS, but not TRN2. The patterns of immunostaining for TRN1 and TAF15 were very similar to that of FUS. None of these proteins was associated with tau or TDP-43 aggregations in FTLD.
CONCLUSIONS
Data suggest that FUS, TRN1 and TAF15 may participate in a functional pathway in an interdependent way, and imply that the function of TDP-43 may not necessarily be in parallel with, or complementary to, that of FUS, despite each protein sharing many similar structural elements.
目的
我们旨在研究核载体和结合蛋白在融合肉瘤蛋白（FTLD-FUS）相关额颞叶变性（FTLD）包涵体形成中的作用，这些蛋白包括转运蛋白 1（TRN1）和转运蛋白 2（TRN2）、TATA 结合蛋白相关因子 15（TAF15）和尤因肉瘤蛋白（EWS）。
方法
对 8 例 FTLD-FUS（5 例非典型 FTLD-U、2 例神经元中间丝包涵体病和 1 例嗜碱性包涵体病）进行 FUS、TRN1、TRN2、TAF15 和 EWS 的免疫染色。10 例 FTLD 伴 TDP-43 包涵体作为参考病例。
结果
FTLD-FUS 中的包涵体包含 TRN1 和 TAF15，并且在较小程度上包含 EWS，但不包含 TRN2。TRN1 和 TAF15 的免疫染色模式与 FUS 非常相似。这些蛋白均不与 FTLD 中的 tau 或 TDP-43 聚集物相关。
结论
数据表明，FUS、TRN1 和 TAF15 可能以相互依赖的方式参与功能途径，并且暗示尽管每种蛋白都具有许多相似的结构元件，但 TDP-43 的功能不一定与 FUS 平行或互补。
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