Conformational change of RNA-helicase DHX30 by ALS/FTD-linked FUS induces mitochondrial dysfunction and cytosolic aggregates

Genetic mutations in fused in sarcoma (FUS) cause amyotrophic lateral sclerosis (ALS). Although mitochondrial dysfunction and stress granule have been crucially implicated in FUS proteinopathy, the molecular basis remains unclear. Here, we show that DHX30, a component of mitochondrial RNA granules r...

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Detalles Bibliográficos
Autores principales: Hikiami, Ryota, Morimura, Toshifumi, Ayaki, Takashi, Tsukiyama, Tomoyuki, Morimura, Naoko, Kusui, Makiko, Wada, Hideki, Minamiyama, Sumio, Shodai, Akemi, Asada-Utsugi, Megumi, Muramatsu, Shin-ichi, Ueki, Takatoshi, Takahashi, Ryosuke, Urushitani, Makoto
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2022
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9512926/
https://www.ncbi.nlm.nih.gov/pubmed/36163369
http://dx.doi.org/10.1038/s41598-022-20405-2

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9512926/
https://www.ncbi.nlm.nih.gov/pubmed/36163369
http://dx.doi.org/10.1038/s41598-022-20405-2

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