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

doi:10.1074/jbc.M113.516492

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

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研究成果: Article › 査読

87 被引用数 (Scopus)

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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
巻	289
号	2
DOI	https://doi.org/10.1074/jbc.M113.516492
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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10.1074/jbc.M113.516492

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T1 - Intranuclear aggregation of mutant FUS/TLS as a molecular pathomechanism of amyotrophic lateral sclerosis

AU - Nomura, Takao

AU - Watanabe, Shoji

AU - Kaneko, Kumi

AU - Yamanaka, Koji

AU - Nukina, Nobuyuki

AU - Furukawa, Yoshiaki

PY - 2014/1/10

Y1 - 2014/1/10

N2 - 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.

AB - 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.

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

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