Please use this identifier to cite or link to this item: https://doi.org/10.1074/jbc.M113.492017
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dc.titleFused in sarcoma (FUS) protein lacking nuclear localization signal (NLS) and major RNA binding motifs triggers proteinopathy and severe motor phenotype in transgenic mice
dc.contributor.authorShelkovnikova, T.A
dc.contributor.authorPeters, O.M
dc.contributor.authorDeykin, A.V
dc.contributor.authorConnor-Robson, N
dc.contributor.authorRobinson, H
dc.contributor.authorUstyugov, A.A
dc.contributor.authorBachurin, S.O
dc.contributor.authorErmolkevich, T.G
dc.contributor.authorGoldman, I.L
dc.contributor.authorSadchikova, E.R
dc.contributor.authorKovrazhkina, E.A
dc.contributor.authorSkvortsova, V.I
dc.contributor.authorLing, S.-C
dc.contributor.authorDa Cruz, S
dc.contributor.authorParone, P.A
dc.contributor.authorBuchman, V.L
dc.contributor.authorNinkina, N.N
dc.date.accessioned2020-10-27T04:44:21Z
dc.date.available2020-10-27T04:44:21Z
dc.date.issued2013
dc.identifier.citationShelkovnikova, 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 (2013). Fused in sarcoma (FUS) protein lacking nuclear localization signal (NLS) and major RNA binding motifs triggers proteinopathy and severe motor phenotype in transgenic mice. Journal of Biological Chemistry 288 (35) : 25266-25274. ScholarBank@NUS Repository. https://doi.org/10.1074/jbc.M113.492017
dc.identifier.issn0021-9258
dc.identifier.urihttps://scholarbank.nus.edu.sg/handle/10635/180783
dc.description.abstractBackground: FUS inclusions are hallmarks of certain neurodegenerative diseases. Results: Expression of a highly aggregate prone FUS variant in transgenic mice causes proteinopathy and severe motor phenotype. Conclusion: Aggregation of FUS is sufficient to recapitulate motor pathology typical for amyotrophic lateral sclerosis. Significance: Understanding the role of protein aggregation in the development of human neurodegenerative diseases is crucial for designing efficient therapeutic approaches. © 2013 by The American Society for Biochemistry and Molecular Biology, Inc.
dc.rightsAttribution 4.0 International
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/
dc.sourceUnpaywall 20201031
dc.subjectAmyotrophic lateral sclerosis
dc.subjectFused in sarcomata
dc.subjectNuclear localization signal
dc.subjectProtein aggregation
dc.subjectRNA-binding motif
dc.subjectTransgenic mice
dc.subjectMammals
dc.subjectNeurodegenerative diseases
dc.subjectProteins
dc.subjectfused in sarcoma protein
dc.subjectamyotrophic lateral sclerosis
dc.subjectanimal cell
dc.subjectanimal experiment
dc.subjectanimal model
dc.subjectanimal tissue
dc.subjectarticle
dc.subjectclinical feature
dc.subjectcontrolled study
dc.subjectdisease course
dc.subjectdisease severity
dc.subjectlifespan
dc.subjectmortality
dc.subjectmotoneuron
dc.subjectmotor dysfunction
dc.subjectmouse
dc.subjectnerve cell lesion
dc.subjectnerve fiber
dc.subjectnervous system inflammation
dc.subjectnonhuman
dc.subjectnuclear localization signal
dc.subjectpathophysiology
dc.subjectphenotype
dc.subjectpriority journal
dc.subjectprotein defect
dc.subjectprotein expression
dc.subjectprotein motif
dc.subjectRNA binding
dc.subjecttransgenic mouse
dc.subjectAmyotrophic Lateral Sclerosis (Lou Gehrig's Disease)
dc.subjectAnimal Models
dc.subjectMotor Neuron Disease
dc.subjectNeurodegeneration
dc.subjectProtein Aggregation
dc.subjectProteinopathy
dc.subjectRNA Metabolism
dc.subjectRNA-binding Proteins
dc.subjectTDP-43
dc.subjectTransgenic Mouse
dc.subjectAmino Acid Motifs
dc.subjectAmino Acid Sequence
dc.subjectAmyotrophic Lateral Sclerosis
dc.subjectAnimals
dc.subjectAxons
dc.subjectCytoplasm
dc.subjectHumans
dc.subjectMice
dc.subjectMice, Transgenic
dc.subjectMotor Neurons
dc.subjectNuclear Localization Signals
dc.subjectPhenotype
dc.subjectRNA
dc.subjectRNA-Binding Protein FUS
dc.subjectSequence Deletion
dc.typeArticle
dc.contributor.departmentDEPT OF PHYSIOLOGY
dc.description.doi10.1074/jbc.M113.492017
dc.description.sourcetitleJournal of Biological Chemistry
dc.description.volume288
dc.description.issue35
dc.description.page25266-25274
dc.published.statePublished
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