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Strategic approach for the prevention of Huntington disease Nobuyuki Nukina 1 1Laboratory for Structural Neuropathology, RIKEN Brain Science Institute Keyword: ハンチントン病 , ポリグルタミン病 , 治療 pp.899-903
Published Date 2005/12/10
DOI https://doi.org/10.11477/mf.1431100111
  • Abstract
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Recently, the experimental preventive therapies have been developed based on the pathomechanism of Huntington disease or polyglutamine diseases. Those are mainly targeted to neuronal cell death, mitochondrial dysfunction, transcriptional dysregulation, aggregate formation, and disease gene itself. The effects of those treatments were assayed using transgenic mouse R6/2 and some extension of lifespan was observed up to 25%. We have focused to find the inhibitory compound to aggregate formation and found trehalose as an effective compound at cellular and mouse level. We have also tried to inhibit the abnormal gene expression at mRNA level using siRNA of EGFP transduced by adenoassociated virus on our huntintin exon1+EGFP mouse. This treatment attenuated aggregation formation and the disease progression even after the onset of the disease, suggesting the siRNA treatment will be another therapy for polyglutamine diseases.


Copyright © 2005, Igaku-Shoin Ltd. All rights reserved.

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電子版ISSN 1882-1243 印刷版ISSN 0001-8724 医学書院

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