Article
Widespread Mislocalization of FUS Is Associated With Mitochondrial Abnormalities in Skeletal Muscle in Amyotrophic Lateral Sclerosis With FUS Mutations.
Journal of neuropathology and experimental neurology - 24 Feb 2022
Yu Meng, Zhao Xutong, Wu Wei, Wang Qingqing, Liu Jing, Zhang Wei, Yuan Yun, Hong Daojun, Wang Zhaoxia, Deng Jianwen
Abstract excerpt
Mutations in the fused in sarcoma (FUS) gene have been reported to be the most common genetic cause of early-onset amyotrophic lateral sclerosis (ALS); cytoplasmic inclusions containing FUS protein are the predominant pathological feature. Recent studies indicated that mutant FUS impaired neuromuscular junctions and induced muscle intrinsic toxicity in cell and animal models. However, the role of FUS in muscle...
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