Article
Conformational change of RNA-helicase DHX30 by ALS/FTD-linked FUS induces mitochondrial dysfunction and cytosolic aggregates.
Scientific reports - 26 Sept 2022
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
Abstract excerpt
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 required for mitochondrial ribosome assembly, interacts with FUS, and plays a crucial role in ALS-FUS. WT FUS did not...
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