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

Research output: Contribution to journalArticle

46 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)1192-1202
Number of pages11
JournalJournal of Biological Chemistry
Volume289
Issue number2
DOIs
Publication statusPublished - 2014 Jan 10

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Amyotrophic Lateral Sclerosis
Agglomeration
Mutation
In Vitro Techniques

ASJC Scopus subject areas

  • Biochemistry
  • Cell Biology
  • Molecular Biology

Cite this

Intranuclear aggregation of mutant FUS/TLS as a molecular pathomechanism of amyotrophic lateral sclerosis. / Nomura, Takao; Watanabe, Shoji; Kaneko, Kumi; Yamanaka, Koji; Nukina, Nobuyuki; Furukawa, Yoshiaki.

In: Journal of Biological Chemistry, Vol. 289, No. 2, 10.01.2014, p. 1192-1202.

Research output: Contribution to journalArticle

Nomura, Takao ; Watanabe, Shoji ; Kaneko, Kumi ; Yamanaka, Koji ; Nukina, Nobuyuki ; Furukawa, Yoshiaki. / Intranuclear aggregation of mutant FUS/TLS as a molecular pathomechanism of amyotrophic lateral sclerosis. In: Journal of Biological Chemistry. 2014 ; Vol. 289, No. 2. pp. 1192-1202.
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