| Literature DB >> 19833157 |
Hiroshi Doi1, Shigeru Koyano, Yume Suzuki, Nobuyuki Nukina, Yoshiyuki Kuroiwa.
Abstract
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. Copyright 2009 Elsevier Ireland Ltd and the Japan Neuroscience Society. All rights reserved.Entities:
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Year: 2009 PMID： 19833157 DOI： 10.1016/j.neures.2009.10.004
Source DB: PubMed Journal: Neurosci Res ISSN： 0168-0102 Impact factor: 3.304