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KAKEN_25860722seika.pdf
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Download
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:785.2 KB
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:Aug 18, 2023 |
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Total downloads since Aug 18, 2023 : 571
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球脊髄性筋萎縮症患者由来iPS細胞の樹立とポリグルタミン病の病態研究
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キュウセキズイセイ キン イシュクショウ カンジャ ユライ iPS サイボウ ノ ジュリツ ト ポリグルタミンビョウ ノ ビョウタイ ケンキュウ
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Kyusekizuisei kin ishukusho kanja yurai iPS saibo no juritsu to porigurutaminbyo no byotai kenkyu
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Establishment of iPS cells from spinal and bulbar muscular atrophy and the research of polyglutamine diseases
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二瓶, 義廣
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ニヘイ, ヨシヒロ
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Nihei, Yoshihiro
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慶應義塾大学・医学部・助教
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Research team head
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科研費研究者番号 : 60468501
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2016
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科学研究費補助金研究成果報告書
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2015
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ポリグルタミン病の一つである球脊髄性筋萎縮症(SBMA)患者由来iPS細胞の樹立と運動ニューロンへの分化誘導に成功した。分化誘導された神経細胞が, テストステロンによる凝集アンドロゲン受容体(AR)の増加を有意に強く引き起こすことを確認した。更に, 治療薬の候補である17-AAGがARの代謝を促すことも確認できた。これらの結果は, 疾患特異的iPS細胞が病態・創薬研究の新たなツールとなり得ることを示すものと考えられた。我々は更にその他のポリグルタミン病(マシャドジョゼフ病, DRPLA)や神経変性疾患患者由来iPS細胞を樹立している。これらを用いて今後ポリグルタミン病の病態研究を展開していく。
We established spinal and bulbar muscular atrophy (SBMA)-derived iPS cells (iPSCs) and confirmed motor neuron differentiation. Aggregation of androgen receptor in SBMA-iPSC-derived neurons is enhanced by dihydrotestosterone. And one of the candidate drugs, 17-AAG promoted to degrade AR aggregation. These findings show iPSCs technology makes us be able to recapitulate disease-specific biochemical features and demonstrate the potential for identification and validation of candidate drugs. We also established iPSCs from other polyglutamine diseases (Machado-Joseph disease, Dentatorubral-pallidoluysian atrophy) and neurodegenerative diseases (amyotrophic lateral sclerosis, Parkinson's disease). We are going to advance the polyglutamine disease researches by using the established iPSC lines.
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研究種目 : 若手研究(B)
研究期間 : 2013～2015
課題番号 : 25860722
研究分野 : 神経変性疾患
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| Aug 18, 2023 | | Creator,Abstract 内容,Note 注記 を変更 |
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