ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function.

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第一作者： Aarti,Sharma

第一单位： Department of Neurology, Center for Motor Neuron Biology and Disease, Columbia University, 630 W 168th Street, P&S Building, Room 5-423, New York, New York 10032, USA.

作者： Aarti,Sharma ^[1] ; Alexander K,Lyashchenko ^[1] ; Lei,Lu ^[1] ; Sara Ebrahimi,Nasrabady ^[2] ; Margot,Elmaleh ^[1] ; Monica,Mendelsohn ^[2] ; Adriana,Nemes ^[3] ; Juan Carlos,Tapia ^[4] ; George Z,Mentis ^[2] ; Neil A,Shneider ^[1]

作者单位： Department of Neurology, Center for Motor Neuron Biology and Disease, Columbia University, 630 W 168th Street, P&S Building, Room 5-423, New York, New York 10032, USA. ^[1] Department of Pathology and Cell Biology, Center for Motor Neuron Biology and Disease, Columbia University, New York, New York 10032, USA. ^[2] Department of Neuroscience, Howard Hughes Medical Institute, Columbia University, New York, New York 10032, USA. ^[3] Department of Neuroscience, Columbia University, New York, New York 10032, USA. ^[4]

主题词肌萎缩侧索硬化(Amyotrophic Lateral Sclerosis);动物(Animals);细胞存活(Cell Survival);疾病模型, 动物(Disease Models, Animal);人类(Humans);小鼠(Mice);小鼠, 基因敲除(Mice, Knockout);小鼠, 转基因(Mice, Transgenic);显微镜检查, 共焦(Microscopy, Confocal);显微镜检查, 电子(Microscopy, Electron);运动神经元(Motor Neurons);肌, 骨骼(Muscle, Skeletal);突变(Mutation);神经变性(Nerve Degeneration);神经肌肉接头(Neuromuscular Junction);表型(Phenotype);RNA结合蛋白质FUS(RNA-Binding Protein FUS);脊髓(Spinal Cord)

DOI 10.1038/ncomms10465

PMID 26842965

发布时间 2024-01-04

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Nature communications

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ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function.

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ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function.

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