RNA-Binding Protein Hoip Accelerates PolyQ-Induced Neurodegeneration in Drosophila
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概要
- 論文の詳細を見る
Abnormal polyglutamine (polyQ) expansion in the N-terminal domain of the human androgen receptor (hAR) is known to cause spinobulbar muscular atrophy (SBMA), a hereditary human neurodegenerative disorder. To explore the molecular mechanisms of neurodegeneration in SBMA, we genetically screened modulators of neurodegeneration in a Drosophila SBMA experimental model system. We identified hoip as an accelerator of polyQ-induced neurodegeneration. We found that hoip forms a complex with 18s rRNA together nop56 and nop5 proteins, whose human homologs are known to form a snoRNP complex involved in ribosomal RNA processing. Significantly, the levels of mutant polyQ-hAR were up-regulated in a mutant line overexpressing hoip. Consistently, severe neurodegeneration phenotype (rough eye) was also observed in both nop56 and nop5 overexpression mutant lines. These findings suggest that the process of neurodegeneration induced by abnormal polyQ expansion in the hAR may be regulated by the activity of snoRNP complex.
- 社団法人 日本農芸化学会の論文
- 2008-09-23
著者
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MIURA Masayuki
Department of Neuroanatomy, Biomedical Center, Osaka University Graduate School of Medicine
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Kato Shigeaki
Institute Of Molecular And Cellular Biosciences The University Of Tokyo
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TAKEYAMA Ken-ichi
Institute of Molecular and Cellular Biosciences, The University of Tokyo
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UEDA Takashi
Institute of Science and Engineering, Kanazawa University
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Takeyama Ken-ichi
Inst. Of Molecular And Cellular Biosciences Univ. Of Tokyo
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Takeyama Ken-ichi
Institute Of Molecular And Cellular Biosciences The University Of Tokyo
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Kato Shigeaki
Institute Of Molecular And Cellular Bioscience University Of Tokyo
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Kato Shigeaki
Exploratory Res. For Advanced Technol. (erato) Japan Sci. And Technol. Agency (jst)
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Kato Shigeaki
Institute Of Molecular And Cellular Biosciences University Of Tokyo
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Zhao Yue
Inst. Of Molecular And Cellular Biosciences Univ. Of Tokyo
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KURANAGA Erina
Department of Veterinary Physiology, Veterinary Medical Science, The University of Tokyo
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FURUTANI Takashi
Institute for Drug Discovery Research, Yamanouchi Pharmaceutical Co., Ltd.
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ITO Saya
Institute of Molecular and Cellular Biosciences, University of Tokyo
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SUZUKI Eriko
Institute of Molecular and Cellular Biosciences, University of Tokyo
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SAWATSUBASHI Shun
Institute of Molecular and Cellular Biosciences, University of Tokyo
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YAMAGATA Kaoru
Institute of Molecular and Cellular Biosciences, University of Tokyo
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ZHAO Yue
Institute of Molecular and Cellular Biosciences, University of Tokyo
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Kuranaga Erina
Department Of Genetics Graduate School Of Pharmaceutical Sciences University Of Tokyo
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Suzuki Eriko
Institute Of Molecular And Cellular Biosciences University Of Tokyo
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Miura Masayuki
Department Of Genetics Graduate School Of Pharmaceutical Sciences University Of Tokyo
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Yamagata Kaoru
Institute Of Molecular And Cellular Biosciences University Of Tokyo
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Furutani Takashi
Institute For Drug Discovery Research Astellas Pharma Inc.
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Murata Takuya
Institute Of Molecular And Cellular Biosciences University Of Tokyo
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Ito Saya
Institute Of Molecular And Cellular Biosciences University Of Tokyo
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Tanabe Masahiko
Institute Of Molecular And Cellular Biosciences University Of Tokyo
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FUJIYAMA Sally
Institute of Molecular and Cellular Biosciences, University of Tokyo
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KIMURA Shuhei
Institute of Molecular and Cellular Biosciences, University of Tokyo
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MATSUKAWA Hiroyuki
Institute of Molecular and Cellular Biosciences, University of Tokyo
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KOUZMENKO Alexander
Institute of Molecular and Cellular Biosciences, University of Tokyo
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Zhao Yue
Institute Of Mobile Communications In Southwest Jiaotong University
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Fujiyama Sally
Institute Of Molecular And Cellular Biosciences University Of Tokyo
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Sawatsubashi Shun
Institute Of Molecular And Cellular Biosciences University Of Tokyo
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Matsukawa Hiroyuki
Institute Of Molecular And Cellular Biosciences University Of Tokyo
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Kouzmenko Alexandre
Institute Of Molecular And Cellular Biosciences University Of Tokyo
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Miura Masayuki
Department Of Biology Tokyo Metropolitan University:(present)division Of Regulation Of Macromolecula
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Kimura Shuhei
Institute Of Molecular And Cellular Biosciences University Of Tokyo
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Ueda Takashi
Institute Of Science And Engineering Kanazawa University
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Ueda Takashi
Institute Of Molecular And Cellular Biosciences University Of Tokyo
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Kato Shigeaki
Institute for Chemical Research, Kyoto University
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