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Updated: Sep 9, 2025

Assay to Measure Nucleocytoplasmic Transport in Real Time within Motor Neuron-like NSC-34 Cells
08:53

Assay to Measure Nucleocytoplasmic Transport in Real Time within Motor Neuron-like NSC-34 Cells

Published on: May 16, 2017

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C9orf72のALSを引き起こす変異は,ヌクレオポリンNup107の誤局化と集積をストレス粒に導きます

Saygın Bilican1,2, Yara Nabawi1,2, William Hongyu Zhang1,2

  • 1Institute for Integrated Stress Response Signaling, Faculty of Medicine, University Hospital Cologne, Germany.

FEBS letters
|September 2, 2025
PubMed
まとめ

研究者らは,Nup107はC9orf72遺伝子変異に罹患した運動神経細胞のストレス粒子を相互作用させ,アミオトロフィック横筋硬化症 (ALS) の重要な要因であることを発見した. 虫にNup107を投与した結果,ALSに関連する症状が改善されました.

キーワード:
アミオトロフィック・ラテラル・スクレロスC. エレガンスニュクレオポリンプロテオスタシスストレス・グランブルiPSC疾患モデリング

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