Several RNA-processing genes have been implicated in the pathogenesis of Amyotrophic lateral sclerosis (ALS). In particular, causative mutations in the genes encoding for two DNA/RNA binding proteins, TAR DNA binding protein-43 (TDP-43) and fused in sarcoma/translocated in liposarcoma (FUS/TLS), were recently identified in ALS patients. These genetic findings and the presence of abnormal aggregates of these two RNA-binding proteins in ALS affected tissues suggest that molecular mechanisms regulating RNA metabolism are implicated in ALS pathogenesis through common pathways. In this review similarities and differences between TDP-43 and FUS/TLS proteins and their activities in physiological and pathological conditions will be discussed.

中文翻译：

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RNA结合蛋白和RNA代谢：肌萎缩性侧索硬化的发病机制中的新场景。

肌萎缩性侧索硬化症（ALS）的发病机制中涉及到几个RNA处理基因。特别是，最近在ALS患者中发现了编码两种DNA / RNA结合蛋白TAR DNA结合蛋白43（TDP-43）并融合在肉瘤中/易位于脂肪肉瘤（FUS / TLS）的基因中的致病突变。这些遗传学发现以及在ALS受影响的组织中这两种RNA结合蛋白的异常聚集体的存在表明，调节RNA代谢的分子机制通过共同途径参与了ALS的发病机理。在这篇综述中，将讨论TDP-43和FUS / TLS蛋白及其在生理和病理条件下的活性之间的异同。