Intranuclear aggregation of mutant FUS/TLS as a molecular pathomechanism of amyotrophic lateral sclerosis

Takao Nomura, Shoji Watanabe, Kumi Kaneko, Koji Yamanaka, Nobuyuki Nukina, Yoshiaki Furukawa

研究成果: Article査読

87 被引用数 (Scopus)

抄録

Background: Abnormal accumulation of mutant FUS/TLS is a pathological change in patients with amyotrophic lateralsclerosis (ALS). Results: A pathogenic mutation, G156E, increases propensities of FUS/TLS for aggregation in vitro and in vivo. Conclusion: Intranuclear aggregation of mutant FUS/TLS is a molecular pathomechanism of ALS. Significance: A loss of functional TLS/FUS in the nucleus will lead to neurodegeneration.

本文言語English
ページ(範囲)1192-1202
ページ数11
ジャーナルJournal of Biological Chemistry
289
2
DOI
出版ステータスPublished - 2014 1月 10

ASJC Scopus subject areas

  • 生化学
  • 分子生物学
  • 細胞生物学

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