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Clinical and Genetic Features of Patients with Juvenile Amyotrophic Lateral Sclerosis with Fused in Sarcoma (FUS) Mutation.

Yu X, Zhao Z, Shen H, Bing Q, Li N, Hu J.

Med Sci Monit. 2018 Dec 3;24:8750-8757. doi: 10.12659/MSM.913724.


Mutant FUS causes DNA ligation defects to inhibit oxidative damage repair in Amyotrophic Lateral Sclerosis.

Wang H, Guo W, Mitra J, Hegde PM, Vandoorne T, Eckelmann BJ, Mitra S, Tomkinson AE, Van Den Bosch L, Hegde ML.

Nat Commun. 2018 Sep 11;9(1):3683. doi: 10.1038/s41467-018-06111-6.


The prionlike domain of FUS is multiphosphorylated following DNA damage without altering nuclear localization.

Rhoads SN, Monahan ZT, Yee DS, Leung AY, Newcombe CG, O'Meally RN, Cole RN, Shewmaker FP.

Mol Biol Cell. 2018 Aug 1;29(15):1786-1797. doi: 10.1091/mbc.E17-12-0735. Epub 2018 Jun 13.


FUS Phase Separation Is Modulated by a Molecular Chaperone and Methylation of Arginine Cation-π Interactions.

Qamar S, Wang G, Randle SJ, Ruggeri FS, Varela JA, Lin JQ, Phillips EC, Miyashita A, Williams D, Ströhl F, Meadows W, Ferry R, Dardov VJ, Tartaglia GG, Farrer LA, Kaminski Schierle GS, Kaminski CF, Holt CE, Fraser PE, Schmitt-Ulms G, Klenerman D, Knowles T, Vendruscolo M, St George-Hyslop P.

Cell. 2018 Apr 19;173(3):720-734.e15. doi: 10.1016/j.cell.2018.03.056.


Phase Separation of FUS Is Suppressed by Its Nuclear Import Receptor and Arginine Methylation.

Hofweber M, Hutten S, Bourgeois B, Spreitzer E, Niedner-Boblenz A, Schifferer M, Ruepp MD, Simons M, Niessing D, Madl T, Dormann D.

Cell. 2018 Apr 19;173(3):706-719.e13. doi: 10.1016/j.cell.2018.03.004.


Astrocytes expressing ALS-linked mutant FUS induce motor neuron death through release of tumor necrosis factor-alpha.

Kia A, McAvoy K, Krishnamurthy K, Trotti D, Pasinelli P.

Glia. 2018 May;66(5):1016-1033. doi: 10.1002/glia.23298. Epub 2018 Jan 30.


ALS-Related Mutant FUS Protein Is Mislocalized to Cytoplasm and Is Recruited into Stress Granules of Fibroblasts from Asymptomatic FUS P525L Mutation Carriers.

Lo Bello M, Di Fini F, Notaro A, Spataro R, Conforti FL, La Bella V.

Neurodegener Dis. 2017;17(6):292-303. doi: 10.1159/000480085. Epub 2017 Oct 17.


FUS Mutant Human Motoneurons Display Altered Transcriptome and microRNA Pathways with Implications for ALS Pathogenesis.

De Santis R, Santini L, Colantoni A, Peruzzi G, de Turris V, Alfano V, Bozzoni I, Rosa A.

Stem Cell Reports. 2017 Nov 14;9(5):1450-1462. doi: 10.1016/j.stemcr.2017.09.004. Epub 2017 Oct 5.


Structure of FUS Protein Fibrils and Its Relevance to Self-Assembly and Phase Separation of Low-Complexity Domains.

Murray DT, Kato M, Lin Y, Thurber KR, Hung I, McKnight SL, Tycko R.

Cell. 2017 Oct 19;171(3):615-627.e16. doi: 10.1016/j.cell.2017.08.048. Epub 2017 Sep 21.


Dendritic Homeostasis Disruption in a Novel Frontotemporal Dementia Mouse Model Expressing Cytoplasmic Fused in Sarcoma.

Shiihashi G, Ito D, Arai I, Kobayashi Y, Hayashi K, Otsuka S, Nakajima K, Yuzaki M, Itohara S, Suzuki N.

EBioMedicine. 2017 Oct;24:102-115. doi: 10.1016/j.ebiom.2017.09.005. Epub 2017 Sep 9.


Phosphorylation of the FUS low-complexity domain disrupts phase separation, aggregation, and toxicity.

Monahan Z, Ryan VH, Janke AM, Burke KA, Rhoads SN, Zerze GH, O'Meally R, Dignon GL, Conicella AE, Zheng W, Best RB, Cole RN, Mittal J, Shewmaker F, Fawzi NL.

EMBO J. 2017 Oct 16;36(20):2951-2967. doi: 10.15252/embj.201696394. Epub 2017 Aug 8.


[The FUS protein: Physiological functions and a role in amyotrophic lateral sclerosis].

Efimova AD, Ovchinnikov RK, Roman AY, Maltsev AV, Grigoriev VV, Kovrazhkina EA, Skvortsova VI.

Mol Biol (Mosk). 2017 May-Jun;51(3):387-399. doi: 10.7868/S0026898417020094. Review. Russian.


Neuron-to-Neuron Transfer of FUS in Drosophila Primary Neuronal Culture Is Enhanced by ALS-Associated Mutations.

Feuillette S, Delarue M, Riou G, Gaffuri AL, Wu J, Lenkei Z, Boyer O, Frébourg T, Campion D, Lecourtois M.

J Mol Neurosci. 2017 May;62(1):114-122. doi: 10.1007/s12031-017-0908-y. Epub 2017 Apr 20.


ALS-linked FUS exerts a gain of toxic function involving aberrant p38 MAPK activation.

Sama RR, Fallini C, Gatto R, McKeon JE, Song Y, Rotunno MS, Penaranda S, Abdurakhmanov I, Landers JE, Morfini G, Brady ST, Bosco DA.

Sci Rep. 2017 Mar 8;7(1):115. doi: 10.1038/s41598-017-00091-1.


Long noncoding RNA SchLAH suppresses metastasis of hepatocellular carcinoma through interacting with fused in sarcoma.

Ge Z, Cheng Z, Yang X, Huo X, Wang N, Wang H, Wang C, Gu D, Zhao F, Yao M, Fan J, Qin W.

Cancer Sci. 2017 Apr;108(4):653-662. doi: 10.1111/cas.13200. Epub 2017 Apr 17.


A novel mutation of the C-terminal amino acid of FUS (Y526C) strengthens FUS gene as the most frequent genetic factor in aggressive juvenile ALS.

Corcia P, Danel V, Lacour A, Beltran S, Andres C, Couratier P, Blasco H, Vourc'h P.

Amyotroph Lateral Scler Frontotemporal Degener. 2017 May;18(3-4):298-301. doi: 10.1080/21678421.2016.1265564. Epub 2017 Jan 5.


Mislocated FUS is sufficient for gain-of-toxic-function amyotrophic lateral sclerosis phenotypes in mice.

Shiihashi G, Ito D, Yagi T, Nihei Y, Ebine T, Suzuki N.

Brain. 2016 Sep;139(Pt 9):2380-94. doi: 10.1093/brain/aww161. Epub 2016 Jun 30.


Minor intron splicing is regulated by FUS and affected by ALS-associated FUS mutants.

Reber S, Stettler J, Filosa G, Colombo M, Jutzi D, Lenzken SC, Schweingruber C, Bruggmann R, Bachi A, Barabino SM, Mühlemann O, Ruepp MD.

EMBO J. 2016 Jul 15;35(14):1504-21. doi: 10.15252/embj.201593791. Epub 2016 Jun 1.


Mechanisms of FUS mutations in familial amyotrophic lateral sclerosis.

Shang Y, Huang EJ.

Brain Res. 2016 Sep 15;1647:65-78. doi: 10.1016/j.brainres.2016.03.036. Epub 2016 Mar 28. Review.

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