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

被引：92

作者：

Doi, Hiroshi ^{[1
]}

Koyano, Shigeru ^{[1
]}

Suzuki, Yume ^{[1
]}

Nukina, Nobuyuki ^{[2
]}

Kuroiwa, Yoshiyuki ^{[1
]}

机构：

[1] Yokohama City Univ, Grad Sch Med, Dept Clin Neurol & Stroke Med, Kanazawa Ku, Yokohama, Kanagawa 2360004, Japan

[2] RIKEN, Brain Sci Inst, Lab Struct Neuropathol, Wako, Saitama 3510198, Japan

来源：

NEUROSCIENCE RESEARCH | 2010年 / 66卷 / 01期

关键词：

Polyglutamine; FUS/TLS; Amyotrophic lateral sclerosis; Neuronal intranuclear inclusion; Spinocerebellar ataxia; Dentatorubral-pallidoluysian atrophy; AMYOTROPHIC-LATERAL-SCLEROSIS; NEURODEGENERATION; LIPOSARCOMA; MUTATIONS; FUSION; CHOP; GENE; TLS;

D O I：

10.1016/j.neures.2009.10.004

中图分类号：

Q189 [神经科学];

学科分类号：

071006 ;

摘要：

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. (C) 2009 Elsevier Ireland Ltd and the Japan Neuroscience Society. All rights reserved.

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页码：131 / 133

页数：3

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