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神经退行性疾病是一种神经元变性和继发性脱髓鞘所引起的慢性病,近年研究发现一种DNA/RNA结合蛋白——肉瘤融合/脂肪肉瘤转运蛋白(FUS/TLS)与肌萎缩侧索硬化症(ALS)、额颞叶痴呆(FTD)及多聚谷氨酰胺疾病等神经退行性病变相关.FUS/TLS的变异或失功能可引起相关基因转录和RNA加工、转运、翻译等过程出现异常,并且这些异常与神经退行性疾病发生发展高度相关.FUS/TLS的发现和相关研究不但有助于在RNA水平上对神经退行性疾病的认识,也为神经退行性疾病的防治提供一种新的思路.“,”Neurodegenerative diseases are chronic lesions caused by neuronal degeneration and secondary demyelination.Recent studies have shown that a DNA/RNA binding protein named fused in sarcoma/translocated in liposarcoma protein (FUS/TLS) is associated with neurodegenerative diseases,such as amyotrophic lateral sclerosis (ALS),frontotemporal dementia (FTD) and polyglutamine diseases.Mutations or dysfunction in FUS/TLS are responsible for the abnormal processes in the related gene transcription,RNA processing,transportation and translation and are highly correlated with the development of neurodegenerative disease.Discovery and the research of FUS/TLS not only contribute to the understanding of neurodegenerative disease at RNA level,but also provide a new way for the prevention and treatment of neurodegenerative diseases.