FUS

Summary

Gene Symbol: FUS
Description: FUS RNA binding protein
Alias: ALS6, ETM4, FUS1, HNRNPP2, POMP75, TLS, RNA-binding protein FUS, 75 kDa DNA-pairing protein, FUS/ERG fusion protein, fus-like protein, fused in sarcoma, fusion gene in myxoid liposarcoma, heterogeneous nuclear ribonucleoprotein P2, oncogene FUS, oncogene TLS, translocated in liposarcoma protein
Species: human
Products:     FUS

Top Publications

  1. Yamashita S, Mori A, Sakaguchi H, Suga T, Ishihara D, Ueda A, et al. Sporadic juvenile amyotrophic lateral sclerosis caused by mutant FUS/TLS: possible association of mental retardation with this mutation. J Neurol. 2012;259:1039-44 pubmed publisher
    ..Of note, the first patient was mentally retarded before the onset of weakness. Fused in sarcoma/translocated in liposarcoma (FUS/TLS) gene analyses revealed mutations of p. G492EfsX527 (c...
  2. Yamamoto Watanabe Y, Watanabe M, Okamoto K, Fujita Y, Jackson M, Ikeda M, et al. A Japanese ALS6 family with mutation R521C in the FUS/TLS gene: a clinical, pathological and genetic report. J Neurol Sci. 2010;296:59-63 pubmed publisher
    ..The proband was found to have mutation R521C in the FUS/TLS gene, and was diagnosed as having ALS6. Autopsy material was available from the mother of the proband and FUS-immunoreactive neuronal and glial ..
  3. Spitzer J, Ugras S, Runge S, Decarolis P, Antonescu C, Tuschl T, et al. mRNA and protein levels of FUS, EWSR1, and TAF15 are upregulated in liposarcoma. Genes Chromosomes Cancer. 2011;50:338-47 pubmed publisher
    Translocations or mutations of FUS, EWSR1, and TAF15 (FET) result in distinct genetic diseases...
  4. Rogelj B, Easton L, Bogu G, Stanton L, Rot G, Curk T, et al. Widespread binding of FUS along nascent RNA regulates alternative splicing in the brain. Sci Rep. 2012;2:603 pubmed publisher
    b>Fused in sarcoma (FUS) and TAR DNA-binding protein 43 (TDP-43) are RNA-binding proteins pathogenetically linked to amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD), but it is not known if they regulate the ..
  5. Ruddy D, Parton M, Al Chalabi A, Lewis C, Vance C, Smith B, et al. Two families with familial amyotrophic lateral sclerosis are linked to a novel locus on chromosome 16q. Am J Hum Genet. 2003;73:390-6 pubmed
    ..5 Mb on the Marshfield map. Bioinformatic analysis of the region has identified 18 known genes and 70 predicted genes in this region, and sequencing of candidate genes has now begun. ..
  6. Andersson M, Ståhlberg A, Arvidsson Y, Olofsson A, Semb H, Stenman G, et al. The multifunctional FUS, EWS and TAF15 proto-oncoproteins show cell type-specific expression patterns and involvement in cell spreading and stress response. BMC Cell Biol. 2008;9:37 pubmed publisher
    b>FUS, EWS and TAF15 are structurally similar multifunctional proteins that were first discovered upon characterization of fusion oncogenes in human sarcomas and leukemias...
  7. Engström K, Willen H, Kåbjörn Gustafsson C, Andersson C, Olsson M, Göransson M, et al. The myxoid/round cell liposarcoma fusion oncogene FUS-DDIT3 and the normal DDIT3 induce a liposarcoma phenotype in transfected human fibrosarcoma cells. Am J Pathol. 2006;168:1642-53 pubmed
    ..In conclusion, because the fusion oncogene FUS-DDIT3 and the normal DDIT3 induce a liposarcoma phenotype when expressed in a primitive sarcoma cell line, MLS/..
  8. Verbeeck C, Deng Q, DeJesus Hernandez M, Taylor G, Ceballos Diaz C, Kocerha J, et al. Expression of Fused in sarcoma mutations in mice recapitulates the neuropathology of FUS proteinopathies and provides insight into disease pathogenesis. Mol Neurodegener. 2012;7:53 pubmed publisher
    Mutations in the gene encoding the RNA-binding protein fused in sarcoma (FUS) can cause familial and sporadic amyotrophic lateral sclerosis (ALS) and rarely frontotemproal dementia (FTD)...
  9. Hallier M, Lerga A, Barnache S, Tavitian A, Moreau Gachelin F. The transcription factor Spi-1/PU.1 interacts with the potential splicing factor TLS. J Biol Chem. 1998;273:4838-42 pubmed
    ..1 binds RNA and interferes in vitro with the splicing process. Here we report that Spi-1 interacts in vivo with TLS (translocated in liposarcoma), a RNA-binding protein involved in human tumor-specific chromosomal translocations...

More Information

Publications122 found, 100 shown here

  1. Wu Y, Foo J, Tan L, Chen C, Prakash K, Chen Y, et al. Identification of a novel risk variant in the FUS gene in essential tremor. Neurology. 2013;81:541-4 pubmed publisher
    A nonsense mutation in the amyotrophic lateral sclerosis gene FUS has been found to segregate in a large family with essential tremor (ET). Coding variants in this gene have not been comprehensively evaluated in ET...
  2. Iko Y, Kodama T, Kasai N, Oyama T, Morita E, Muto T, et al. Domain architectures and characterization of an RNA-binding protein, TLS. J Biol Chem. 2004;279:44834-40 pubmed
    Translocated in liposarcoma (TLS) is an important protein component of the heterogeneous nuclear ribonucleoprotein complex involved in the splicing of pre-mRNA and the export of fully processed mRNA to the cytoplasm...
  3. Bentmann E, Haass C, Dormann D. Stress granules in neurodegeneration--lessons learnt from TAR DNA binding protein of 43 kDa and fused in sarcoma. FEBS J. 2013;280:4348-70 pubmed publisher
    ..of these SG marker proteins were found to label pathological TAR DNA binding protein of 43 kDa (TDP-43)- or fused in sarcoma (FUS)-positive cytoplasmic inclusions in patients with amyotrophic lateral sclerosis and frontotemporal lobar ..
  4. Mastrocola A, Kim S, Trinh A, Rodenkirch L, Tibbetts R. The RNA-binding protein fused in sarcoma (FUS) functions downstream of poly(ADP-ribose) polymerase (PARP) in response to DNA damage. J Biol Chem. 2013;288:24731-41 pubmed publisher
    ..Here, we provide evidence that the RNA-binding protein fused in sarcoma/translocated in liposarcoma (FUS) is a novel component of the DNA damage response...
  5. Blair I, Williams K, Warraich S, Durnall J, Thoeng A, Manavis J, et al. FUS mutations in amyotrophic lateral sclerosis: clinical, pathological, neurophysiological and genetic analysis. J Neurol Neurosurg Psychiatry. 2010;81:639-45 pubmed publisher
    b>FUS gene mutations were recently identified in familial amyotrophic lateral sclerosis (ALS)...
  6. Huang C, Zhou H, Tong J, Chen H, Liu Y, Wang D, et al. FUS transgenic rats develop the phenotypes of amyotrophic lateral sclerosis and frontotemporal lobar degeneration. PLoS Genet. 2011;7:e1002011 pubmed publisher
    b>Fused in Sarcoma (FUS) proteinopathy is a feature of frontotemporal lobar dementia (FTLD), and mutation of the fus gene segregates with FTLD and amyotrophic lateral sclerosis (ALS)...
  7. Page T, Gitcho M, Mosaheb S, Carter D, Chakraverty S, Perry R, et al. FUS immunogold labeling TEM analysis of the neuronal cytoplasmic inclusions of neuronal intermediate filament inclusion disease: a frontotemporal lobar degeneration with FUS proteinopathy. J Mol Neurosci. 2011;45:409-21 pubmed publisher
    b>Fused in sarcoma (FUS)-immunoreactive neuronal and glial inclusions define a novel molecular pathology called FUS proteinopathy...
  8. Suzuki N, Kato S, Kato M, Warita H, Mizuno H, Kato M, et al. FUS/TLS-immunoreactive neuronal and glial cell inclusions increase with disease duration in familial amyotrophic lateral sclerosis with an R521C FUS/TLS mutation. J Neuropathol Exp Neurol. 2012;71:779-88 pubmed publisher
    ..Mutations in the fused in sarcoma/translocated in liposarcoma (FUS/TLS) gene have recently been identified as a cause of FALS...
  9. Lagier Tourenne C, Polymenidou M, Hutt K, Vu A, Baughn M, Huelga S, et al. Divergent roles of ALS-linked proteins FUS/TLS and TDP-43 intersect in processing long pre-mRNAs. Nat Neurosci. 2012;15:1488-97 pubmed publisher
    FUS/TLS (fused in sarcoma/translocated in liposarcoma) and TDP-43 are integrally involved in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia...
  10. Rapp T, Yang L, Conrad E, Mandahl N, Chansky H. RNA splicing mediated by YB-1 is inhibited by TLS/CHOP in human myxoid liposarcoma cells. J Orthop Res. 2002;20:723-9 pubmed
    ..chromosomal translocation that results in fusion of the N-terminal domain of the translocated in liposarcoma (TLS) protein to the C/EBP homologous protein (CHOP)...
  11. Kim S, Shanware N, Bowler M, Tibbetts R. Amyotrophic lateral sclerosis-associated proteins TDP-43 and FUS/TLS function in a common biochemical complex to co-regulate HDAC6 mRNA. J Biol Chem. 2010;285:34097-105 pubmed publisher
    ..mutations in two related RNA-binding proteins, TDP-43 (43-kDa TAR DNA-binding domain protein) and FUS/TLS (fused in sarcoma/translated in liposarcoma) cause a subset of ALS...
  12. Göransson M, Wedin M, Aman P. Temperature-dependent localization of TLS-CHOP to splicing factor compartments. Exp Cell Res. 2002;278:125-32 pubmed
    The myxoid/round cell liposarcoma oncogene TLS-CHOP belongs to a growing family of tumor type specific fusion genes generated by chromosome translocations...
  13. Deng H, Zhai H, Bigio E, Yan J, Fecto F, Ajroud K, et al. FUS-immunoreactive inclusions are a common feature in sporadic and non-SOD1 familial amyotrophic lateral sclerosis. Ann Neurol. 2010;67:739-48 pubmed publisher
    ..Recent studies have shown that mutations in FUS are causal in approximately 4 to 5% of FALS and some apparent SALS cases...
  14. Schwartz J, Ebmeier C, Podell E, Heimiller J, Taatjes D, Cech T. FUS binds the CTD of RNA polymerase II and regulates its phosphorylation at Ser2. Genes Dev. 2012;26:2690-5 pubmed publisher
    Mutations in the RNA-binding protein FUS (fused in sarcoma)/TLS have been shown to cause the neurodegenerative disease amyotrophic lateral sclerosis (ALS), but the normal role of FUS is incompletely understood...
  15. Baron D, Kaushansky L, Ward C, Sama R, Chian R, Boggio K, et al. Amyotrophic lateral sclerosis-linked FUS/TLS alters stress granule assembly and dynamics. Mol Neurodegener. 2013;8:30 pubmed publisher
    Amyotrophic lateral sclerosis (ALS)-linked fused in sarcoma/translocated in liposarcoma (FUS/TLS or FUS) is concentrated within cytoplasmic stress granules under conditions of induced stress...
  16. Shiohama A, Sasaki T, Noda S, Minoshima S, Shimizu N. Nucleolar localization of DGCR8 and identification of eleven DGCR8-associated proteins. Exp Cell Res. 2007;313:4196-207 pubmed
    ..Thus, our studies provided additional new evidence for the involvement of various protein complexes in the molecular mechanisms of apparently complex innate RNA interference machinery. ..
  17. Vaccaro A, Tauffenberger A, Aggad D, Rouleau G, Drapeau P, Parker J. Mutant TDP-43 and FUS cause age-dependent paralysis and neurodegeneration in C. elegans. PLoS ONE. 2012;7:e31321 pubmed publisher
    Mutations in the DNA/RNA binding proteins TDP-43 and FUS are associated with Amyotrophic Lateral Sclerosis and Frontotemporal Lobar Degeneration...
  18. Niu C, Zhang J, Gao F, Yang L, Jia M, Zhu H, et al. FUS-NLS/Transportin 1 complex structure provides insights into the nuclear targeting mechanism of FUS and the implications in ALS. PLoS ONE. 2012;7:e47056 pubmed publisher
    The C-terminal nuclear localization sequence of FUsed in Sarcoma (FUS-NLS) is critical for its nuclear import mediated by transportin (Trn1). Familial amyotrophic lateral sclerosis (ALS) related mutations are clustered in FUS-NLS...
  19. Zinszner H, Sok J, Immanuel D, Yin Y, Ron D. TLS (FUS) binds RNA in vivo and engages in nucleo-cytoplasmic shuttling. J Cell Sci. 1997;110 ( Pt 15):1741-50 pubmed
    b>TLS, the product of a gene commonly translocated in liposarcomas (TLS), is prototypical of a newly identified class of nuclear proteins that contain a C-terminal domain with a distinct RNA recognition motif (RRM) surrounded by Arg-Gly-..
  20. Yamaguchi A, Kitajo K. The effect of PRMT1-mediated arginine methylation on the subcellular localization, stress granules, and detergent-insoluble aggregates of FUS/TLS. PLoS ONE. 2012;7:e49267 pubmed publisher
    b>Fused in sarcoma/translocated in liposarcoma (FUS/TLS) is one of causative genes for familial amyotrophic lateral sclerosis (ALS)...
  21. Takahama K, Takada A, Tada S, Shimizu M, Sayama K, Kurokawa R, et al. Regulation of telomere length by G-quadruplex telomere DNA- and TERRA-binding protein TLS/FUS. Chem Biol. 2013;20:341-50 pubmed publisher
    ..b>TLS/FUS is a human telomere-binding protein that was first identified as an oncogenic fusion protein in human myxoid ..
  22. Damme P, Goris A, Race V, Hersmus N, Dubois B, Bosch L, et al. The occurrence of mutations in FUS in a Belgian cohort of patients with familial ALS. Eur J Neurol. 2010;17:754-6 pubmed publisher
    Mutations in fused in sarcoma (FUS) were recently identified as a cause of familial amyotrophic lateral sclerosis (ALS). The frequency of occurrence of mutations in FUS in sets of patients with familial ALS remains to be established...
  23. Sama R, Ward C, Kaushansky L, Lemay N, Ishigaki S, Urano F, et al. FUS/TLS assembles into stress granules and is a prosurvival factor during hyperosmolar stress. J Cell Physiol. 2013;228:2222-31 pubmed publisher
    b>FUsed in Sarcoma/Translocated in LipoSarcoma (FUS/TLS or FUS) has been linked to several biological processes involving DNA and RNA processing, and has been associated with multiple diseases, including myxoid liposarcoma and amyotrophic ..
  24. Meissner M, Lopato S, Gotzmann J, Sauermann G, Barta A. Proto-oncoprotein TLS/FUS is associated to the nuclear matrix and complexed with splicing factors PTB, SRm160, and SR proteins. Exp Cell Res. 2003;283:184-95 pubmed
    b>TLS/FUS is a nucleic acid-binding protein whose N-terminal half functions as a transcriptional activator domain in fusion oncoproteins found in human leukemias and liposarcomas...
  25. Bertrand P, Akhmedov A, Delacote F, Durrbach A, Lopez B. Human POMp75 is identified as the pro-oncoprotein TLS/FUS: both POMp75 and POMp100 DNA homologous pairing activities are associated to cell proliferation. Oncogene. 1999;18:4515-21 pubmed
    ..In mammalian nuclear extracts, we detected two major DNA homologous pairing activities: POMp100 and POMp75. Here, we present the purification and identification of POMp75 as the pro-oncoprotein TLS/FUS...
  26. Wada K, Inoue K, Hagiwara M. Identification of methylated proteins by protein arginine N-methyltransferase 1, PRMT1, with a new expression cloning strategy. Biochim Biophys Acta. 2002;1591:1-10 pubmed
    ..This systematic screening of substrate proteins with the solid phase methylation reaction will contribute to identify new roles of PRMT family. ..
  27. Miguel L, Avequin T, Delarue M, Feuillette S, Frebourg T, Campion D, et al. Accumulation of insoluble forms of FUS protein correlates with toxicity in Drosophila. Neurobiol Aging. 2012;33:1008.e1-15 pubmed publisher
    Recently, the fused in sarcoma/translated in liposarcoma (FUS) protein has been identified as a major constituent of nuclear and/or cytoplasmic ubiquitin-positive inclusions in patients with frontotemporal lobar degeneration or ..
  28. Zhang D, Paley A, Childs G. The transcriptional repressor ZFM1 interacts with and modulates the ability of EWS to activate transcription. J Biol Chem. 1998;273:18086-91 pubmed
    ..Furthermore, two proteins, TLS and hTAFII68, which have extensive homology to EWS, also interact with ZFM1...
  29. Kasyapa C, Kunapuli P, Cowell J. Mass spectroscopy identifies the splicing-associated proteins, PSF, hnRNP H3, hnRNP A2/B1, and TLS/FUS as interacting partners of the ZNF198 protein associated with rearrangement in myeloproliferative disease. Exp Cell Res. 2005;309:78-85 pubmed
    ..Other proteins identified were the spliceosomal components hnRNP A2/B1, hnRNP H3, and TLS/FUS. PSF is also known to interact with PTB, another member of the hnRNP family of proteins, and we further ..
  30. Wang X, Arai S, Song X, Reichart D, Du K, Pascual G, et al. Induced ncRNAs allosterically modify RNA-binding proteins in cis to inhibit transcription. Nature. 2008;454:126-30 pubmed publisher
    ..Here we show that an RNA-binding protein, TLS (for translocated in liposarcoma), serves as a key transcriptional regulatory sensor of DNA damage signals that, on ..
  31. Hewitt C, Kirby J, Highley J, Hartley J, Hibberd R, Hollinger H, et al. Novel FUS/TLS mutations and pathology in familial and sporadic amyotrophic lateral sclerosis. Arch Neurol. 2010;67:455-61 pubmed publisher
    To determine the frequency of and clinicopathologic phenotypes associated with FUS/TLS mutations in a large cohort of amyotrophic lateral sclerosis (ALS) cases from the north of England...
  32. Powers C, Mathur M, Raaka B, Ron D, Samuels H. TLS (translocated-in-liposarcoma) is a high-affinity interactor for steroid, thyroid hormone, and retinoid receptors. Mol Endocrinol. 1998;12:4-18 pubmed
    ..p65 was identified as TLS (translocated-in-liposarcoma), a recently identified member of the RNP family of nuclear RNA-binding proteins whose ..
  33. Rademakers R, Stewart H, DeJesus Hernandez M, Krieger C, Graff Radford N, Fabros M, et al. Fus gene mutations in familial and sporadic amyotrophic lateral sclerosis. Muscle Nerve. 2010;42:170-6 pubmed publisher
    Mutations in the fused in sarcoma (FUS) gene have recently been found to cause familial amyotrophic lateral sclerosis (FALS). We screened FUS in a cohort of 200 ALS patients [32 FALS and 168 sporadic ALS (SALS)]...
  34. Bosco D, Lemay N, Ko H, Zhou H, Burke C, Kwiatkowski T, et al. Mutant FUS proteins that cause amyotrophic lateral sclerosis incorporate into stress granules. Hum Mol Genet. 2010;19:4160-75 pubmed publisher
    Mutations in the RNA-binding protein FUS (fused in sarcoma) are linked to amyotrophic lateral sclerosis (ALS), but the mechanism by which these mutants cause motor neuron degeneration is not known...
  35. Sato S, Idogawa M, Honda K, Fujii G, Kawashima H, Takekuma K, et al. Beta-catenin interacts with the FUS proto-oncogene product and regulates pre-mRNA splicing. Gastroenterology. 2005;129:1225-36 pubmed
    ..beta-Catenin physically interacted with fusion (FUS)/translocated in liposarcoma (TLS) and various RNA-binding proteins...
  36. Daigle J, Lanson N, Smith R, Casci I, Maltare A, Monaghan J, et al. RNA-binding ability of FUS regulates neurodegeneration, cytoplasmic mislocalization and incorporation into stress granules associated with FUS carrying ALS-linked mutations. Hum Mol Genet. 2013;22:1193-205 pubmed publisher
    ..Several genes, including SOD1, TDP-43, FUS, Ubiquilin 2, C9orf72 and Profilin 1, have been linked with the sporadic and familiar forms of ALS...
  37. Lai S, Abramzon Y, Schymick J, Stephan D, Dunckley T, Dillman A, et al. FUS mutations in sporadic amyotrophic lateral sclerosis. Neurobiol Aging. 2011;32:550.e1-4 pubmed publisher
    Mutations in the FUS gene have recently been described as a cause of familial amyotrophic lateral sclerosis (ALS), but their role in the pathogenesis of sporadic ALS is unclear...
  38. Yan J, Deng H, Siddique N, Fecto F, Chen W, Yang Y, et al. Frameshift and novel mutations in FUS in familial amyotrophic lateral sclerosis and ALS/dementia. Neurology. 2010;75:807-14 pubmed publisher
    ..Mutations in the FUS gene were identified in patients with familial ALS (FALS) and patients with sporadic ALS (SALS) from a variety of ..
  39. Sapp P, Hosler B, McKenna Yasek D, Chin W, Gann A, Genise H, et al. Identification of two novel loci for dominantly inherited familial amyotrophic lateral sclerosis. Am J Hum Genet. 2003;73:397-403 pubmed
    ..The analysis of these genes will delineate pathways implicated as determinants of motor-neuron viability and provide insights into possible therapies for ALS. ..
  40. Thomsen C, Grundevik P, Elias P, Ståhlberg A, Aman P. A conserved N-terminal motif is required for complex formation between FUS, EWSR1, TAF15 and their oncogenic fusion proteins. FASEB J. 2013;27:4965-74 pubmed publisher
    The three FET (FUS, EWSR1, and TAF15) family RNA binding proteins are expressed in all tissues and almost all cell types. The disordered N-terminal parts are always present in FET fusion oncoproteins of sarcomas and leukemia...
  41. Belzil V, Valdmanis P, Dion P, Daoud H, Kabashi E, Noreau A, et al. Mutations in FUS cause FALS and SALS in French and French Canadian populations. Neurology. 2009;73:1176-9 pubmed publisher
    The identification of mutations in the TARDBP and more recently the identification of mutations in the FUS gene as the cause of amyotrophic lateral sclerosis (ALS) is providing the field with new insight about the mechanisms involved in ..
  42. Chiò A, Restagno G, Brunetti M, Ossola I, Calvo A, Mora G, et al. Two Italian kindreds with familial amyotrophic lateral sclerosis due to FUS mutation. Neurobiol Aging. 2009;30:1272-5 pubmed publisher
    Recently, fused in sarcoma/translated in liposarcoma (FUS/TLS) gene, located on chromosome 16p11.2, has been identified as a disease gene in familial amyotrophic lateral sclerosis (FALS)...
  43. Mackenzie I, Ansorge O, Strong M, Bilbao J, Zinman L, Ang L, et al. Pathological heterogeneity in amyotrophic lateral sclerosis with FUS mutations: two distinct patterns correlating with disease severity and mutation. Acta Neuropathol. 2011;122:87-98 pubmed publisher
    Mutations in the gene encoding the fused in sarcoma (FUS) protein are responsible for ~3% of familial amyotrophic lateral sclerosis (ALS) and <1% of sporadic ALS (ALS-FUS)...
  44. Wang J, Brent J, Tomlinson A, Shneider N, McCabe B. The ALS-associated proteins FUS and TDP-43 function together to affect Drosophila locomotion and life span. J Clin Invest. 2011;121:4118-26 pubmed publisher
    ..The RNA/DNA-binding proteins fused in sarcoma (FUS; also known as TLS) and TAR DNA binding protein-43 (TDP-43) have recently been shown to be genetically ..
  45. Ito D, Suzuki N. Conjoint pathologic cascades mediated by ALS/FTLD-U linked RNA-binding proteins TDP-43 and FUS. Neurology. 2011;77:1636-43 pubmed publisher
    The RNA-binding proteins TAR DNA-binding protein (TDP-43) and fused in sarcoma (FUS) play central roles in neurodegeneration associated with familial amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration with ubiquitin-..
  46. Zhang Z, Chook Y. Structural and energetic basis of ALS-causing mutations in the atypical proline-tyrosine nuclear localization signal of the Fused in Sarcoma protein (FUS). Proc Natl Acad Sci U S A. 2012;109:12017-21 pubmed publisher
    Mutations in the proline/tyrosine-nuclear localization signal (PY-NLS) of the Fused in Sarcoma protein (FUS) cause amyotrophic lateral sclerosis (ALS)...
  47. Yang L, Embree L, Tsai S, Hickstein D. Oncoprotein TLS interacts with serine-arginine proteins involved in RNA splicing. J Biol Chem. 1998;273:27761-4 pubmed
    The gene encoding the human TLS protein, also termed FUS, is located at the site of chromosomal translocations in human leukemias and sarcomas where it forms a chimeric fusion gene with one of several different genes...
  48. Waibel S, Neumann M, Rosenbohm A, Birve A, Volk A, Weishaupt J, et al. Truncating mutations in FUS/TLS give rise to a more aggressive ALS-phenotype than missense mutations: a clinico-genetic study in Germany. Eur J Neurol. 2013;20:540-6 pubmed publisher
    Mutations in the FUS/TLS have been associated with amyotrophic lateral sclerosis (ALS) in a few percent of patients...
  49. Morlando M, Dini Modigliani S, Torrelli G, Rosa A, Di Carlo V, Caffarelli E, et al. FUS stimulates microRNA biogenesis by facilitating co-transcriptional Drosha recruitment. EMBO J. 2012;31:4502-10 pubmed publisher
    ..In this paper, we show that the FUS/TLS (fused in sarcoma/translocated in liposarcoma) protein, associated with familial forms of Amyotrophic Lateral Sclerosis (ALS), ..
  50. Drepper C, Herrmann T, Wessig C, Beck M, Sendtner M. C-terminal FUS/TLS mutations in familial and sporadic ALS in Germany. Neurobiol Aging. 2011;32:548.e1-4 pubmed publisher
    ..ALS patients, 41 familial ALS cases and other motor neuron disease patients from Germany for mutations in the FUS/TLS gene...
  51. Ling S, Albuquerque C, Han J, Lagier Tourenne C, Tokunaga S, Zhou H, et al. ALS-associated mutations in TDP-43 increase its stability and promote TDP-43 complexes with FUS/TLS. Proc Natl Acad Sci U S A. 2010;107:13318-23 pubmed publisher
    ..trans-activating response region (TAR) DNA-binding protein with a molecular mass of 43 KDa (TDP-43) and fused in sarcoma/translocation in liposarcoma (FUS/TLS), cause an inherited form of ALS that is accompanied by nuclear and ..
  52. Goransson M, Andersson M, Forni C, Stahlberg A, Andersson C, Olofsson A, et al. The myxoid liposarcoma FUS-DDIT3 fusion oncoprotein deregulates NF-kappaB target genes by interaction with NFKBIZ. Oncogene. 2009;28:270-8 pubmed publisher
    b>FUS (also called TLS), EWSR1 and TAF15 (also called TAF2N) are related genes involved in tumor type-specific fusion oncogenes in human malignancies...
  53. Waibel S, Neumann M, Rabe M, Meyer T, Ludolph A. Novel missense and truncating mutations in FUS/TLS in familial ALS. Neurology. 2010;75:815-7 pubmed publisher
    Mutations in the FUS/TLS gene have been associated with familial amyotrophic lateral sclerosis (FALS)...
  54. Armstrong G, Drapeau P. Loss and gain of FUS function impair neuromuscular synaptic transmission in a genetic model of ALS. Hum Mol Genet. 2013;22:4282-92 pubmed publisher
    ..Mutations in the gene fused in sarcoma (FUS) are associated with ALS and cause impairment in motor function in animal models...
  55. Crozat A, Aman P, Mandahl N, Ron D. Fusion of CHOP to a novel RNA-binding protein in human myxoid liposarcoma. Nature. 1993;363:640-4 pubmed
    ..of the translocation-associated CHOP gene product revealed a fusion between CHOP and a gene provisionally named TLS (translocated in liposarcoma)...
  56. Neumann M, Roeber S, Kretzschmar H, Rademakers R, Baker M, Mackenzie I. Abundant FUS-immunoreactive pathology in neuronal intermediate filament inclusion disease. Acta Neuropathol. 2009;118:605-16 pubmed publisher
    ..Mutations in the gene encoding the fused in sarcoma (FUS) protein have recently been identified as a cause of familial amyotrophic lateral sclerosis (ALS)...
  57. Sabatelli M, Moncada A, Conte A, Lattante S, Marangi G, Luigetti M, et al. Mutations in the 3' untranslated region of FUS causing FUS overexpression are associated with amyotrophic lateral sclerosis. Hum Mol Genet. 2013;22:4748-55 pubmed publisher
    Mutations in the gene encoding fused-in-sarcoma (FUS) have been identified in a subset of patients with sporadic and familial amyotrophic lateral sclerosis (ALS)...
  58. Aman P, Panagopoulos I, Lassen C, Fioretos T, Mencinger M, Toresson H, et al. Expression patterns of the human sarcoma-associated genes FUS and EWS and the genomic structure of FUS. Genomics. 1996;37:1-8 pubmed
    b>FUS (TLS) was first identified as the 5'-part of a fusion gene with CHOP (GADD153, DDIT3) in myxoid liposarcomas with t(12; 16)(q13; p11)...
  59. Schwarzbach M, Koesters R, Germann A, Mechtersheimer G, Geisbill J, Winkler S, et al. Comparable transforming capacities and differential gene expression patterns of variant FUS/CHOP fusion transcripts derived from soft tissue liposarcomas. Oncogene. 2004;23:6798-805 pubmed
    ..It results in transcription of various chimeric FUS/CHOP fusion transcripts that encode different oncogenic proteins...
  60. Van Langenhove T, Van der Zee J, Sleegers K, Engelborghs S, Vandenberghe R, Gijselinck I, et al. Genetic contribution of FUS to frontotemporal lobar degeneration. Neurology. 2010;74:366-71 pubmed publisher
    Recently, the FUS gene was identified as a new causal gene for amyotrophic lateral sclerosis (ALS) in approximately 4% of patients with familial ALS...
  61. Dini Modigliani S, Morlando M, Errichelli L, Sabatelli M, Bozzoni I. An ALS-associated mutation in the FUS 3'-UTR disrupts a microRNA-FUS regulatory circuitry. Nat Commun. 2014;5:4335 pubmed publisher
    While the physiologic functions of the RNA-binding protein FUS still await thorough characterization, the pathonegetic role of FUS mutations in amyotrophic lateral sclerosis (ALS) is clearly established...
  62. Uranishi H, Tetsuka T, Yamashita M, Asamitsu K, Shimizu M, Itoh M, et al. Involvement of the pro-oncoprotein TLS (translocated in liposarcoma) in nuclear factor-kappa B p65-mediated transcription as a coactivator. J Biol Chem. 2001;276:13395-401 pubmed
    In this study, we have demonstrated that translocated in liposarcoma (TLS), also termed FUS, is an interacting molecule of the p65 (RelA) subunit of the transcription factor nuclear factor kappaB (NF-kappaB) using a yeast two-hybrid ..
  63. Neumann M, Valori C, Ansorge O, Kretzschmar H, Munoz D, Kusaka H, et al. Transportin 1 accumulates specifically with FET proteins but no other transportin cargos in FTLD-FUS and is absent in FUS inclusions in ALS with FUS mutations. Acta Neuropathol. 2012;124:705-16 pubmed publisher
    Accumulation of the DNA/RNA binding protein fused in sarcoma (FUS) as inclusions in neurons and glia is the pathological hallmark of amyotrophic lateral sclerosis patients with mutations in FUS (ALS-FUS) as well as in several subtypes of ..
  64. Rabbitts T, Forster A, Larson R, Nathan P. Fusion of the dominant negative transcription regulator CHOP with a novel gene FUS by translocation t(12;16) in malignant liposarcoma. Nat Genet. 1993;4:175-80 pubmed
    ..myxoid liposarcoma can be a fusion of the CHOP dominant negative transcription factor gene with a novel gene, FUS, which can result in fusion of the FUS glycine-rich protein with the whole CHOP coding region...
  65. Doi H, Okamura K, Bauer P, Furukawa Y, Shimizu H, Kurosawa M, et al. RNA-binding protein TLS is a major nuclear aggregate-interacting protein in huntingtin exon 1 with expanded polyglutamine-expressing cells. J Biol Chem. 2008;283:6489-500 pubmed publisher
    ..As a result, we found that the RNA-binding protein translocated in liposarcoma (TLS) was one of the major components of nuclear polyQ aggregate-interacting proteins in a Huntington disease cell model ..
  66. Broustal O, Camuzat A, Guillot Noël L, Guy N, Millecamps S, Deffond D, et al. FUS mutations in frontotemporal lobar degeneration with amyotrophic lateral sclerosis. J Alzheimers Dis. 2010;22:765-9 pubmed
    ..were made in the knowledge of amyotrophic lateral sclerosis (ALS) with the recent identification of TARDBP and FUS mutations in familial ALS...
  67. Ito D, Seki M, Tsunoda Y, Uchiyama H, Suzuki N. Nuclear transport impairment of amyotrophic lateral sclerosis-linked mutations in FUS/TLS. Ann Neurol. 2011;69:152-62 pubmed publisher
    The fused in sarcoma/translated in liposarcoma (FUS/TLS) protein was recently identified as a cause of familial amyotrophic lateral sclerosis (ALS), as well as a major component of the inclusion bodies found in subtypes of frontotemporal ..
  68. Tradewell M, Yu Z, Tibshirani M, Boulanger M, Durham H, Richard S. Arginine methylation by PRMT1 regulates nuclear-cytoplasmic localization and toxicity of FUS/TLS harbouring ALS-linked mutations. Hum Mol Genet. 2012;21:136-49 pubmed publisher
    Mutations in FUS/TLS (fused in sarcoma/translated in liposarcoma) cause an inheritable form of amyotrophic lateral sclerosis (ALS6)...
  69. Schwartz J, Wang X, Podell E, Cech T. RNA seeds higher-order assembly of FUS protein. Cell Rep. 2013;5:918-25 pubmed publisher
    The abundant nuclear RNA binding protein FUS binds the C-terminal domain (CTD) of RNA polymerase II in an RNA-dependent manner, affecting Ser2 phosphorylation and transcription...
  70. Brooke G, Culley R, Dart D, Mann D, Gaughan L, McCracken S, et al. FUS/TLS is a novel mediator of androgen-dependent cell-cycle progression and prostate cancer growth. Cancer Res. 2011;71:914-24 pubmed publisher
    ..Using a proteomic screen, we found the RNA-binding protein FUS/TLS (Fused in Ewing's Sarcoma/Translocated in Liposarcoma) to be downregulated in response to androgen...
  71. Bentmann E, Neumann M, Tahirovic S, Rodde R, Dormann D, Haass C. Requirements for stress granule recruitment of fused in sarcoma (FUS) and TAR DNA-binding protein of 43 kDa (TDP-43). J Biol Chem. 2012;287:23079-94 pubmed publisher
    Cytoplasmic inclusions containing TAR DNA-binding protein of 43 kDa (TDP-43) or Fused in sarcoma (FUS) are a hallmark of amyotrophic lateral sclerosis (ALS) and several subtypes of frontotemporal lobar degeneration (FTLD)...
  72. Mackenzie I, Rademakers R, Neumann M. TDP-43 and FUS in amyotrophic lateral sclerosis and frontotemporal dementia. Lancet Neurol. 2010;9:995-1007 pubmed publisher
    ..form of FTD, frontotemporal lobar degeneration with ubiquitinated inclusions, followed by identification of FUS as the novel pathological protein in a small subset of patients with ALS and various FTD subtypes provide clear ..
  73. Murakami T, Yang S, Xie L, Kawano T, Fu D, Mukai A, et al. ALS mutations in FUS cause neuronal dysfunction and death in Caenorhabditis elegans by a dominant gain-of-function mechanism. Hum Mol Genet. 2012;21:1-9 pubmed publisher
    It is unclear whether mutations in fused in sarcoma (FUS) cause familial amyotrophic lateral sclerosis via a loss-of-function effect due to titrating FUS from the nucleus or a gain-of-function effect from cytoplasmic overabundance...
  74. Tan A, Riley T, Coady T, Bussemaker H, Manley J. TLS/FUS (translocated in liposarcoma/fused in sarcoma) regulates target gene transcription via single-stranded DNA response elements. Proc Natl Acad Sci U S A. 2012;109:6030-5 pubmed publisher
    TLS/FUS (TLS) is a multifunctional protein implicated in a wide range of cellular processes, including transcription and mRNA processing, as well as in both cancer and neurological disease...
  75. Sun Z, Diaz Z, Fang X, Hart M, Chesi A, Shorter J, et al. Molecular determinants and genetic modifiers of aggregation and toxicity for the ALS disease protein FUS/TLS. PLoS Biol. 2011;9:e1000614 pubmed publisher
    TDP-43 and FUS are RNA-binding proteins that form cytoplasmic inclusions in some forms of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD). Moreover, mutations in TDP-43 and FUS are linked to ALS and FTLD...
  76. Shelkovnikova T, Peters O, Deykin A, Connor Robson N, Robinson H, Ustyugov A, et al. Fused in sarcoma (FUS) protein lacking nuclear localization signal (NLS) and major RNA binding motifs triggers proteinopathy and severe motor phenotype in transgenic mice. J Biol Chem. 2013;288:25266-74 pubmed publisher
    Dysfunction of two structurally and functionally related proteins, FUS and TAR DNA-binding protein of 43 kDa (TDP-43), implicated in crucial steps of cellular RNA metabolism can cause amyotrophic lateral sclerosis (ALS) and certain other ..
  77. Dormann D, Rodde R, Edbauer D, Bentmann E, Fischer I, Hruscha A, et al. ALS-associated fused in sarcoma (FUS) mutations disrupt Transportin-mediated nuclear import. EMBO J. 2010;29:2841-57 pubmed publisher
    Mutations in fused in sarcoma (FUS) are a cause of familial amyotrophic lateral sclerosis (fALS)...
  78. Mitchell J, McGoldrick P, Vance C, Hortobagyi T, Sreedharan J, Rogelj B, et al. Overexpression of human wild-type FUS causes progressive motor neuron degeneration in an age- and dose-dependent fashion. Acta Neuropathol. 2013;125:273-88 pubmed publisher
    ..Cytoplasmic inclusions of fused in sarcoma (FUS) are the hallmark of several forms of FTLD and ALS patients with mutations in the FUS gene...
  79. Neumann M, Rademakers R, Roeber S, Baker M, Kretzschmar H, Mackenzie I. A new subtype of frontotemporal lobar degeneration with FUS pathology. Brain. 2009;132:2922-31 pubmed publisher
    ..Mutations in the fused in sarcoma (FUS) gene have recently been identified as a cause of familial amyotrophic lateral sclerosis, with these ..
  80. Ticozzi N, Silani V, Leclerc A, Keagle P, Gellera C, Ratti A, et al. Analysis of FUS gene mutation in familial amyotrophic lateral sclerosis within an Italian cohort. Neurology. 2009;73:1180-5 pubmed publisher
    Mutations in the FUS gene on chromosome 16 have been recently discovered as a cause of familial amyotrophic lateral sclerosis (FALS)...
  81. Tateishi T, Hokonohara T, Yamasaki R, Miura S, Kikuchi H, Iwaki A, et al. Multiple system degeneration with basophilic inclusions in Japanese ALS patients with FUS mutation. Acta Neuropathol. 2010;119:355-64 pubmed publisher
    Mutations in the fused in sarcoma gene (FUS) were recently found in patients with familial amyotrophic lateral sclerosis (ALS)...
  82. Du K, Arai S, Kawamura T, Matsushita A, Kurokawa R. TLS and PRMT1 synergistically coactivate transcription at the survivin promoter through TLS arginine methylation. Biochem Biophys Res Commun. 2011;404:991-6 pubmed publisher
    TLS (Translocated in LipoSarcoma), also termed FUS, is a multifunctional protein implicated in diverse cellular events such as maintaining genome integrity and regulating gene expression...
  83. Belzil V, Daoud H, St Onge J, Desjarlais A, Bouchard J, Dupre N, et al. Identification of novel FUS mutations in sporadic cases of amyotrophic lateral sclerosis. Amyotroph Lateral Scler. 2011;12:113-7 pubmed publisher
    Mutations in the FUS gene have been recently associated with amyotrophic lateral sclerosis (ALS)...
  84. Neumann M, Bentmann E, Dormann D, Jawaid A, DeJesus Hernandez M, Ansorge O, et al. FET proteins TAF15 and EWS are selective markers that distinguish FTLD with FUS pathology from amyotrophic lateral sclerosis with FUS mutations. Brain. 2011;134:2595-609 pubmed publisher
    Accumulation of the DNA/RNA binding protein fused in sarcoma as cytoplasmic inclusions in neurons and glial cells is the pathological hallmark of all patients with amyotrophic lateral sclerosis with mutations in FUS as well as in several ..
  85. Vance C, Rogelj B, Hortobágyi T, De Vos K, Nishimura A, Sreedharan J, et al. Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6. Science. 2009;323:1208-1211 pubmed publisher
    ..We have identified a missense mutation in the gene encoding fused in sarcoma (FUS) in a British kindred, linked to ALS6...
  86. DeJesus Hernandez M, Kocerha J, Finch N, Crook R, Baker M, Desaro P, et al. De novo truncating FUS gene mutation as a cause of sporadic amyotrophic lateral sclerosis. Hum Mutat. 2010;31:E1377-89 pubmed publisher
    Mutations in the gene encoding fused in sarcoma (FUS) were recently identified as a novel cause of amyotrophic lateral sclerosis (ALS), emphasizing the genetic heterogeneity of ALS...
  87. Zou Z, Peng Y, Feng X, Wang X, Sun Q, Liu M, et al. Screening of the FUS gene in familial and sporadic amyotrophic lateral sclerosis patients of Chinese origin. Eur J Neurol. 2012;19:977-83 pubmed publisher
    According to studies in European, North American, Australian, and Asian populations, FUS gene mutations occur in 0.6-20.2% of the patients with familial amyotrophic lateral sclerosis (ALS) and 0.4-2.0% of sporadic ALS cases...
  88. Xia R, Liu Y, Yang L, Gal J, Zhu H, Jia J. Motor neuron apoptosis and neuromuscular junction perturbation are prominent features in a Drosophila model of Fus-mediated ALS. Mol Neurodegener. 2012;7:10 pubmed publisher
    ..identified as their mutations can lead to familial ALS, including the recently reported RNA-binding protein fused in sarcoma (Fus). However, it is not clear how mutations of Fus lead to motor neuron degeneration in ALS...
  89. Scaramuzzino C, Monaghan J, Milioto C, Lanson N, Maltare A, Aggarwal T, et al. Protein arginine methyltransferase 1 and 8 interact with FUS to modify its sub-cellular distribution and toxicity in vitro and in vivo. PLoS ONE. 2013;8:e61576 pubmed publisher
    ..Mutations in the gene coding for fused in sarcoma/translocated in liposarcoma (FUS) are responsible for some cases of both familial and sporadic forms of ALS...
  90. Morohoshi F, Ootsuka Y, Arai K, Ichikawa H, Mitani S, Munakata N, et al. Genomic structure of the human RBP56/hTAFII68 and FUS/TLS genes. Gene. 1998;221:191-8 pubmed
    ..RBP56 cDNA by PCR using mixed primers designed from the conserved sequences of the RNA binding domain of FUS/TLS and EWS proteins...
  91. Göransson M, Elias E, Ståhlberg A, Olofsson A, Andersson C, Aman P. Myxoid liposarcoma FUS-DDIT3 fusion oncogene induces C/EBP beta-mediated interleukin 6 expression. Int J Cancer. 2005;115:556-60 pubmed
    The myxoid/round cell liposarcoma oncogene FUS-DDIT3 is the result of a translocation derived gene fusion between the splicing factor FUS and DDIT3...
  92. Yang L, Embree L, Hickstein D. TLS-ERG leukemia fusion protein inhibits RNA splicing mediated by serine-arginine proteins. Mol Cell Biol. 2000;20:3345-54 pubmed
    The translocation liposarcoma (TLS) gene is fused to the ETS-related gene (ERG) in human myeloid leukemia, resulting in the generation of a TLS-ERG protein...