The RNA-binding protein FUS/TLS is a common aggregate-interacting protein in polyglutamine diseases

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• 作者：Hiroshi Doi ; Shigeru Koyano ; Yume Suzuki ; Nobuyuki Nukina ; Yoshiyuki Kuroiwa
• 关键词：Polyglutamine ; FUS/TLS ; Amyotrophic lateral sclerosis ; Neuronal intranuclear inclusion ; Spinocerebellar ataxia ; Dentatorubral-pallidoluysian atrophy
• 刊名：Neuroscience Research
• 出版年：2010
• 出版时间：January 2010
• 年：2010
• 卷：66
• 期：1
• 页码：131-133
• 全文大小：189 K

文摘

Neuronal intranuclear inclusions (NIIs) are the pathological hallmark of polyglutamine (polyQ) diseases. We previously found that the RNA-binding protein FUS/TLS is the major component of nuclear polyQ aggregates of a cellular model of Huntington disease. In this study, we revealed that FUS/TLS binds to NIIs in the human brains from patients with spinocerebellar ataxia type 1, 2, 3, and dentatorubral-pallidoluysian atrophy. Recent reports have revealed that mutations in FUS/TLS gene are responsible for familial amyotrophic lateral sclerosis 6 (ALS6). Our results indicated that changing FUS/TLS to an insoluble form may be a common process in polyQ diseases and ALS6.