Fused in sarcoma (FUS) protein lacking nuclear localization signal (NLS) and major RNA binding motifs triggers proteinopathy and severe motor phenotype in transgenic mice

• Main Authors: Shelkovnikova, T.A, Peters, O.M, Deykin, A.V, Connor-Robson, N, Robinson, H, Ustyugov, A.A, Bachurin, S.O, Ermolkevich, T.G, Goldman, I.L, Sadchikova, E.R, Kovrazhkina, E.A, Skvortsova, V.I, Ling, S.-C, Da Cruz, S, Parone, P.A, Buchman, V.L, Ninkina, N.N
• Other Authors: PHYSIOLOGY
• Format: Article
• Published: 2020
• Subjects: Amyotrophic lateral sclerosis; Fused in sarcomata; Nuclear localization signal; Protein aggregation; RNA-binding motif; Transgenic mice; Mammals; Neurodegenerative diseases; Proteins; fused in sarcoma protein; amyotrophic lateral sclerosis; animal cell; animal experiment; animal model; animal tissue; article; clinical feature; controlled study; disease course; disease severity; lifespan; mortality; motoneuron; motor dysfunction; mouse; nerve cell lesion; nerve fiber; nervous system inflammation; nonhuman; nuclear localization signal; pathophysiology; phenotype; priority journal; protein defect; protein expression; protein motif; RNA binding; transgenic mouse; Amyotrophic Lateral Sclerosis (Lou Gehrig's Disease); Animal Models; Motor Neuron Disease; Neurodegeneration; Protein Aggregation; Proteinopathy; RNA Metabolism; RNA-binding Proteins; TDP-43; Transgenic Mouse; Amino Acid Motifs; Amino Acid Sequence; Amyotrophic Lateral Sclerosis; Animals; Axons; Cytoplasm; Humans; Mice; Mice, Transgenic; Motor Neurons; Nuclear Localization Signals; Phenotype; RNA; RNA-Binding Protein FUS; Sequence Deletion
• Online Access: https://scholarbank.nus.edu.sg/handle/10635/180783
• Institution: National University of Singapore

10.1074/jbc.M113.492017