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Protein aggregation in motor neuron disease Jun-ichi Niwa 1 , Gen Sobue 1 1Department of Neurology, Nagoya University Graduate School of Medicine, Showa-ku Keyword: 運動ニューロン疾患 , ユビキチン化封入体 , タンパク質凝集 pp.74-85
Published Date 2004/2/10
DOI https://doi.org/10.11477/mf.1431100181
  • Abstract
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 Neuronal toxicity associated with abnormal protein aggregation is one of major hypotheses for many neurodegenerative disorders. Evidence from cell culture models and transgenic mice show protein accumulation in motor system to be closely associated with motor dysfunction of X-linked spinobulbar muscular atrophy(SBMA)and familial amyotrophic lateral sclerosis(ALS)associated with superoxide dismutase 1(SOD1)mutation. In SBMA, androgen-dependent translocation and aggregation of mutant androgen receptor with expanded polyglutamine repeats into the nucleus is a critical step for pathogenesis and anti-androgen therapy offer hope for effective treatment. In ALS with SOD1mutation, there are several lines of evidence that cytoplasmic aggregation of mutant SOD1 leads to motor neuronal cell dysfunction, but pathogenetic mechanisms remains to be elucidated.


Copyright © 2004, 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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