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. 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...
  2. 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)...
  3. 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-..
  4. 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...
  5. 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 ..
  6. 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
    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...
  7. 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...
  8. 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. ..
  9. 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 ..
  10. 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...

Detail Information

Publications96

  1. 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...
  2. 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)...
  3. 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-..
  4. 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...
  5. 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 ..
  6. 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
    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...
  7. 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...
  8. 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. ..
  9. 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 ..
  10. 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...
  11. 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 ..
  12. 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...
  13. 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...
  14. 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...
  15. 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 ..
  16. 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)]...
  17. 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...
  18. 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...
  19. 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 ..
  20. 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...
  21. 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 ..
  22. 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)...
  23. Chio 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)...
  24. 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-..
  25. 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 ..
  26. 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)...
  27. 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...
  28. 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...
  29. 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), ..
  30. 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 ..
  31. 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...
  32. 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...
  33. 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)...
  34. 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)...
  35. 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)...
  36. 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)...
  37. 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...
  38. 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...
  39. 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
    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)...
  40. 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...
  41. 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 ..
  42. 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 ..
  43. 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)...
  44. 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...
  45. 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...
  46. 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...
  47. 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 ..
  48. 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 ..
  49. 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)...
  50. 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...
  51. 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...
  52. 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...
  53. 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 ..
  54. 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...
  55. 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 ..
  56. 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...
  57. 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)...
  58. 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)...
  59. 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...
  60. 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 ..
  61. 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 ..
  62. 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)...
  63. 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)...
  64. 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...
  65. Vance C, Rogelj B, Hortobagyi 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...
  66. 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...
  67. 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...
  68. 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...
  69. 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
    ..isolated RBP56 cDNA by PCR using mixed primers designed from the conserved sequences of the RNA binding domain of FUS/TLS and EWS proteins...
  70. 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...
  71. Kryndushkin D, Wickner R, Shewmaker F. FUS/TLS forms cytoplasmic aggregates, inhibits cell growth and interacts with TDP-43 in a yeast model of amyotrophic lateral sclerosis. Protein Cell. 2011;2:223-36 pubmed publisher
    ..Both wild-type and mutant forms of the RNA-binding proteins FUS and TDP-43 accumulate in cytoplasmic inclusions in the neurons of ALS patients...
  72. Tsai C, Soong B, Lin K, Tu P, Lin J, Lee Y. FUS, TARDBP, and SOD1 mutations in a Taiwanese cohort with familial ALS. Neurobiol Aging. 2011;32:553.e13-21 pubmed publisher
    ..The authors analyzed these genes, including SOD1, FUS, VAPB, ANG, TDP-43, FIG4, and CHMP2B, in a cohort of 15 index patients of Han Chinese descent with adult-onset FALS...
  73. 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...
  74. Nakaya T, Alexiou P, Maragkakis M, Chang A, Mourelatos Z. FUS regulates genes coding for RNA-binding proteins in neurons by binding to their highly conserved introns. RNA. 2013;19:498-509 pubmed publisher
    Dominant mutations and mislocalization or aggregation of Fused in Sarcoma (FUS), an RNA-binding protein (RBP), cause neuronal degeneration in Amyotrophic Lateral Sclerosis (ALS) and Frontotemporal Lobar Degeneration (FTLD), two incurable ..
  75. Lanson N, Maltare A, King H, Smith R, Kim J, Taylor J, et al. A Drosophila model of FUS-related neurodegeneration reveals genetic interaction between FUS and TDP-43. Hum Mol Genet. 2011;20:2510-23 pubmed publisher
    ..b>Fused in sarcoma/translated in liposarcoma (FUS/TLS) and TAR DNA-binding protein (TDP)-43 are DNA/RNA-binding proteins found ..
  76. Pokrishevsky E, Grad L, Yousefi M, Wang J, Mackenzie I, Cashman N. Aberrant localization of FUS and TDP43 is associated with misfolding of SOD1 in amyotrophic lateral sclerosis. PLoS ONE. 2012;7:e35050 pubmed publisher
    ..be caused by genetic mutations of Cu/Zn superoxide dismutase (SOD1), TAR-DNA binding protein 43 (TDP43), or fused in sarcoma/translocated in liposarcoma (FUS/TLS), or can occur in the absence of known mutation as sporadic disease...
  77. Kwiatkowski T, Bosco D, Leclerc A, Tamrazian E, Vanderburg C, Russ C, et al. Mutations in the FUS/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosis. Science. 2009;323:1205-8 pubmed publisher
    ..Ten percent of cases are inherited; most involve unidentified genes. We report here 13 mutations in the fused in sarcoma/translated in liposarcoma (FUS/TLS) gene on chromosome 16 that were specific for familial ALS...
  78. Ju S, Tardiff D, Han H, Divya K, Zhong Q, Maquat L, et al. A yeast model of FUS/TLS-dependent cytotoxicity. PLoS Biol. 2011;9:e1001052 pubmed publisher
    FUS/TLS is a nucleic acid binding protein that, when mutated, can cause a subset of familial amyotrophic lateral sclerosis (fALS)...
  79. Merner N, Girard S, Catoire H, Bourassa C, Belzil V, Rivière J, et al. Exome sequencing identifies FUS mutations as a cause of essential tremor. Am J Hum Genet. 2012;91:313-9 pubmed publisher
    ..We studied a large ET-affected family and identified a FUS p.Gln290(?) mutation as the cause of ET in this family...
  80. Lagier Tourenne C, Cleveland D. Rethinking ALS: the FUS about TDP-43. Cell. 2009;136:1001-4 pubmed publisher
    ..Two recent studies (Kwiatkowski et al., 2009; Vance et al., 2009) now report that mutations in FUS/TLS, another DNA/RNA-binding protein, also trigger premature degeneration of motor neurons...
  81. Baechtold H, Kuroda M, Sok J, Ron D, Lopez B, Akhmedov A. Human 75-kDa DNA-pairing protein is identical to the pro-oncoprotein TLS/FUS and is able to promote D-loop formation. J Biol Chem. 1999;274:34337-42 pubmed
    ..Previously, we detected two mammalian nuclear proteins of 100 and 75 kDa (POMp100 and POMp75, respectively) that are able to promote homologous DNA pairing, a key step in homologous recombination...
  82. Dormann D, Madl T, Valori C, Bentmann E, Tahirovic S, Abou Ajram C, et al. Arginine methylation next to the PY-NLS modulates Transportin binding and nuclear import of FUS. EMBO J. 2012;31:4258-75 pubmed publisher
    b>Fused in sarcoma (FUS) is a nuclear protein that carries a proline-tyrosine nuclear localization signal (PY-NLS) and is imported into the nucleus via Transportin (TRN)...
  83. Suzuki N, Aoki M, Warita H, Kato M, Mizuno H, Shimakura N, et al. FALS with FUS mutation in Japan, with early onset, rapid progress and basophilic inclusion. J Hum Genet. 2010;55:252-4 pubmed publisher
    Mutations in the fused in sarcoma (FUS, also known as translated in liposarcoma) gene have been recently discovered to be associated with familial amyotrophic lateral sclerosis (FALS) in African, European and American populations...
  84. Tan A, Manley J. TLS inhibits RNA polymerase III transcription. Mol Cell Biol. 2010;30:186-96 pubmed publisher
    ..Here we show that TLS (translocated in liposarcoma), a protein originally identified as the product of a chromosomal translocation and ..
  85. Baumer D, Hilton D, Paine S, Turner M, Lowe J, Talbot K, et al. Juvenile ALS with basophilic inclusions is a FUS proteinopathy with FUS mutations. Neurology. 2010;75:611-8 pubmed publisher
    ..Immunohistochemical findings prompted us to sequence the fused in sarcoma (FUS) gene. Motor symptoms began between ages 17 and 22. Disease progression was rapid without dementia...
  86. Belzil V, St Onge J, Daoud H, Desjarlais A, Bouchard J, Dupre N, et al. Identification of a FUS splicing mutation in a large family with amyotrophic lateral sclerosis. J Hum Genet. 2011;56:247-9 pubmed publisher
    ..dismutase 1 mutations has been extensively examined for over a decade, the recently identified FALS-associated FUS gene has been less studied. Therefore, we set out to screen our collection of FALS cases for FUS mutations...
  87. Gerbino V, Carri M, Cozzolino M, Achsel T. Mislocalised FUS mutants stall spliceosomal snRNPs in the cytoplasm. Neurobiol Dis. 2013;55:120-8 pubmed publisher
    ..b>FUS, a nuclear protein, forms cytoplasmic aggregates in cells affected by amyotrophic lateral sclerosis (ALS), and ..
  88. Lattante S, Rouleau G, Kabashi E. TARDBP and FUS mutations associated with amyotrophic lateral sclerosis: summary and update. Hum Mutat. 2013;34:812-26 pubmed publisher
    ..b>Fused in sarcoma (FUS) was found to be responsible for a previously identified ALS6 locus, being mutated in both FALS and SALS ..
  89. Vance C, Scotter E, Nishimura A, Troakes C, Mitchell J, Kathe C, et al. ALS mutant FUS disrupts nuclear localization and sequesters wild-type FUS within cytoplasmic stress granules. Hum Mol Genet. 2013;22:2676-88 pubmed publisher
    Mutations in the gene encoding Fused in Sarcoma (FUS) cause amyotrophic lateral sclerosis (ALS), a fatal neurodegenerative disorder. FUS is a predominantly nuclear DNA- and RNA-binding protein that is involved in RNA processing...
  90. Lashley T, Rohrer J, Bandopadhyay R, Fry C, Ahmed Z, Isaacs A, et al. A comparative clinical, pathological, biochemical and genetic study of fused in sarcoma proteinopathies. Brain. 2011;134:2548-64 pubmed publisher
    ..Recently, mutations in the fused in sarcoma gene have been shown to cause familial amyotrophic lateral sclerosis and fused in sarcoma-positive neuronal ..
  91. Saunders L, Perkins D, Balachandran S, Michaels R, Ford R, Mayeda A, et al. Characterization of two evolutionarily conserved, alternatively spliced nuclear phosphoproteins, NFAR-1 and -2, that function in mRNA processing and interact with the double-stranded RNA-dependent protein kinase, PKR. J Biol Chem. 2001;276:32300-12 pubmed
    ..activity were localized to the C terminus of the protein, a region that was found to specifically interact with FUS and SMN, proteins also known as regulators of RNA processing...
  92. Corrado L, Del Bo R, Castellotti B, Ratti A, Cereda C, Penco S, et al. Mutations of FUS gene in sporadic amyotrophic lateral sclerosis. J Med Genet. 2010;47:190-4 pubmed publisher
    Mutations in the FUS gene have recently been discovered to be a major cause of familial amyotrophic lateral sclerosis (FALS)...