Abstract: ［Abstract］ 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.