Fus

Summary

Gene Symbol: Fus
Description: fused in sarcoma
Alias: D430004D17Rik, D930039C12Rik, Fus1, Tls, RNA-binding protein FUS, fusion, derived from t(12;16) malignant liposarcoma, hnRNP P2, pigpen protein, protein pigpen, translocated in liposarcoma
Species: mouse

Top Publications

  1. doi A protein-protein interaction of stress-responsive myosin VI endowed to inhibit neural progenitor self-replication with RNA binding protein, TLS, in murine hippocampus
    Takeshi Takarada
    Laboratory of Molecular Pharmacology, Division of Pharmaceutical Sciences, Kanazawa University Graduate School of Natural Science and Technology, Kakuma machi, Kanazawa, Ishikawa 920 1192, Japan
    J Neurochem 110:1457-68. 2009
  2. pmc Induced ncRNAs allosterically modify RNA-binding proteins in cis to inhibit transcription
    Xiangting Wang
    Howard Hughes Medical Institute
    Nature 454:126-30. 2008
  3. doi Arginine methylation by PRMT1 regulates nuclear-cytoplasmic localization and toxicity of FUS/TLS harbouring ALS-linked mutations
    Miranda L Tradewell
    Department of Neurology Neurosurgery and Montreal Neurological Institute, McGill University, Montreal, Quebec, Canada H3A 2B4
    Hum Mol Genet 21:136-49. 2012
  4. pmc The RNA-binding protein Fus directs translation of localized mRNAs in APC-RNP granules
    Kyota Yasuda
    Laboratory of Cellular and Molecular Biology, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892
    J Cell Biol 203:737-46. 2013
  5. pmc Intracellular localization and splicing regulation of FUS/TLS are variably affected by amyotrophic lateral sclerosis-linked mutations
    Yoshihiro Kino
    Laboratory for Structural Neuropathology, Brain Science Institute, RIKEN, 2 1, Hirosawa, Wako Shi, Saitama, 351 0198, Japan
    Nucleic Acids Res 39:2781-98. 2011
  6. pmc TDP-43 and FUS RNA-binding proteins bind distinct sets of cytoplasmic messenger RNAs and differently regulate their post-transcriptional fate in motoneuron-like cells
    Claudia Colombrita
    Department of Neurology and Laboratory of Neuroscience, IRCCS Istituto Auxologico Italiano, Milan 20149, Italy
    J Biol Chem 287:15635-47. 2012
  7. doi Tumour suppressor Fus1 provides a molecular link between inflammatory response and mitochondrial homeostasis
    Roman Uzhachenko
    Department of Medicine, Division of Hematology Oncology, Vanderbilt University Medical Center, Nashville, TN, USA
    J Pathol 227:456-69. 2012
  8. pmc Position-dependent FUS-RNA interactions regulate alternative splicing events and transcriptions
    Shinsuke Ishigaki
    Department of Neurology, Division of Neurogenetics, Center for Neurological Diseases and Cancer, Nagoya University Graduate School of Medicine, Nagoya, Japan
    Sci Rep 2:529. 2012
  9. pmc Widespread binding of FUS along nascent RNA regulates alternative splicing in the brain
    Boris Rogelj
    Centre for Neurodegeneration Research, King s College London, Institute of Psychiatry, De Crespigny Park, London, UK
    Sci Rep 2:603. 2012
  10. pmc Divergent roles of ALS-linked proteins FUS/TLS and TDP-43 intersect in processing long pre-mRNAs
    Clotilde Lagier-Tourenne
    Ludwig Institute for Cancer Research, University of California at San Diego, La Jolla, California, USA
    Nat Neurosci 15:1488-97. 2012

Research Grants

Scientific Experts

  • Tatyana A Shelkovnikova
  • Chantelle F Sephton
  • Yoshihiro Kino
  • Christer Thomsen
  • Pietro Fratta
  • Sylvia R Alappat
  • Liu Yang
  • M Mencinger
  • Geoffrey G Hicks
  • Rene Rodriguez
  • Shinsuke Ishigaki
  • Ritsuko Fujii
  • Aarti Sharma
  • Haiyan Qiu
  • Eric J Huang
  • Michael Tibshirani
  • Toru Takumi
  • Wen yuan Wang
  • Miranda L Tradewell
  • Clotilde Lagier-Tourenne
  • Roman Uzhachenko
  • Neil A Shneider
  • Douglas M Anderson
  • Hanqian Mao
  • Alexander K Lyashchenko
  • Mélanie Lalancette-Hébert
  • Hannah K Robinson
  • Tsuyoshi Udagawa
  • Shuying Sun
  • Stuart L Rulten
  • Hyun Hee Ryu
  • Agnieszka Bronisz
  • Heather D Durham
  • Li Huei Tsai
  • Yusuke Fujioka
  • Shuo Chien Ling
  • Masahisa Katsuno
  • Don W Cleveland
  • Gen Sobue
  • Kinji Ohno
  • Gene W Yeo
  • E M Yazlovitskaya
  • Sudeepta Kumar Panda
  • Ewout J N Groen
  • M Indriati Hood
  • Tadashi Nakaya
  • Arnaud Tauffenberger
  • Kyota Yasuda
  • Reddy Ranjith K Sama
  • Christian Haass
  • Kelli Boyd
  • Fumihiko Urano
  • Alla V Ivanova
  • Denise Orozco
  • Boris Rogelj
  • Nicholas A Lanson
  • Atsushi Yamaguchi
  • Claudia Colombrita
  • Jozsef Gal
  • Cristina Sánchez-Ramos
  • Tomoko Yoshida
  • Takeaki Sugawara
  • Cao Huang
  • Takeshi Takarada
  • M Kuroda
  • Xiangting Wang
  • Mary Gardiner
  • Luisa Cironi
  • Jing Pan
  • Hiroshi Doi
  • Benoît Kornmann
  • Juan Carlos Tapia
  • Eric N Olson
  • Benjamin R Nelson
  • John McAnally
  • Margot Elmaleh
  • John J McMahon
  • Adriana Nemes
  • Yun Liu
  • Monica Mendelsohn
  • Debra L Silver
  • Lei Lu
  • Kelly M Anderson
  • Rhonda Bassel-Duby
  • George Z Mentis
  • Weichun Lin
  • Ning Liu
  • Yi Hsuan Tsai
  • Hui Li
  • Jessica Cannavino

Detail Information

Publications59

  1. doi A protein-protein interaction of stress-responsive myosin VI endowed to inhibit neural progenitor self-replication with RNA binding protein, TLS, in murine hippocampus
    Takeshi Takarada
    Laboratory of Molecular Pharmacology, Division of Pharmaceutical Sciences, Kanazawa University Graduate School of Natural Science and Technology, Kakuma machi, Kanazawa, Ishikawa 920 1192, Japan
    J Neurochem 110:1457-68. 2009
    ..Immunoprecipitation analysis revealed the interaction between Myo6 and the RNA-binding protein, translocated in liposarcoma (TLS), while TLS was predominantly expressed by neurons in the cortex, striatum, cerebellum, and ..
  2. pmc Induced ncRNAs allosterically modify RNA-binding proteins in cis to inhibit transcription
    Xiangting Wang
    Howard Hughes Medical Institute
    Nature 454:126-30. 2008
    ..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 the basis of its ..
  3. doi Arginine methylation by PRMT1 regulates nuclear-cytoplasmic localization and toxicity of FUS/TLS harbouring ALS-linked mutations
    Miranda L Tradewell
    Department of Neurology Neurosurgery and Montreal Neurological Institute, McGill University, Montreal, Quebec, Canada H3A 2B4
    Hum Mol Genet 21:136-49. 2012
    Mutations in FUS/TLS (fused in sarcoma/translated in liposarcoma) cause an inheritable form of amyotrophic lateral sclerosis (ALS6)...
  4. pmc The RNA-binding protein Fus directs translation of localized mRNAs in APC-RNP granules
    Kyota Yasuda
    Laboratory of Cellular and Molecular Biology, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892
    J Cell Biol 203:737-46. 2013
    ..Here, we show that APC-RNPs associate with the RNA-binding protein Fus/TLS (fused in sarcoma/translocated in liposarcoma)...
  5. pmc Intracellular localization and splicing regulation of FUS/TLS are variably affected by amyotrophic lateral sclerosis-linked mutations
    Yoshihiro Kino
    Laboratory for Structural Neuropathology, Brain Science Institute, RIKEN, 2 1, Hirosawa, Wako Shi, Saitama, 351 0198, Japan
    Nucleic Acids Res 39:2781-98. 2011
    TLS (translocated in liposarcoma), also known as FUS (fused in sarcoma), is an RNA/DNA-binding protein that plays regulatory roles in transcription, pre-mRNA splicing and mRNA transport...
  6. pmc TDP-43 and FUS RNA-binding proteins bind distinct sets of cytoplasmic messenger RNAs and differently regulate their post-transcriptional fate in motoneuron-like cells
    Claudia Colombrita
    Department of Neurology and Laboratory of Neuroscience, IRCCS Istituto Auxologico Italiano, Milan 20149, Italy
    J Biol Chem 287:15635-47. 2012
    The RNA-binding proteins TDP-43 and FUS form abnormal cytoplasmic aggregates in affected tissues of patients with amyotrophic lateral sclerosis and frontotemporal lobar dementia...
  7. doi Tumour suppressor Fus1 provides a molecular link between inflammatory response and mitochondrial homeostasis
    Roman Uzhachenko
    Department of Medicine, Division of Hematology Oncology, Vanderbilt University Medical Center, Nashville, TN, USA
    J Pathol 227:456-69. 2012
    b>Fus1, encoded by a 3p21.3 tumour suppressor gene, is down-regulated, mutated or lost in the majority of inflammatory thoracic malignancies...
  8. pmc Position-dependent FUS-RNA interactions regulate alternative splicing events and transcriptions
    Shinsuke Ishigaki
    Department of Neurology, Division of Neurogenetics, Center for Neurological Diseases and Cancer, Nagoya University Graduate School of Medicine, Nagoya, Japan
    Sci Rep 2:529. 2012
    b>FUS is an RNA-binding protein that regulates transcription, alternative splicing, and mRNA transport. Aberrations of FUS are causally associated with familial and sporadic ALS/FTLD...
  9. pmc Widespread binding of FUS along nascent RNA regulates alternative splicing in the brain
    Boris Rogelj
    Centre for Neurodegeneration Research, King s College London, Institute of Psychiatry, De Crespigny Park, London, UK
    Sci Rep 2:603. 2012
    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 ..
  10. pmc Divergent roles of ALS-linked proteins FUS/TLS and TDP-43 intersect in processing long pre-mRNAs
    Clotilde Lagier-Tourenne
    Ludwig Institute for Cancer Research, University of California at San Diego, La Jolla, California, USA
    Nat Neurosci 15:1488-97. 2012
    FUS/TLS (fused in sarcoma/translocated in liposarcoma) and TDP-43 are integrally involved in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia...
  11. doi Identification and characterization of FUS/TLS as a new target of ATM
    Mary Gardiner
    MRC Protein Phosphorylation Unit, Sir James Black Centre, University of Dundee, Dundee DD1 5EH, UK
    Biochem J 415:297-307. 2008
    ..This resulted in the identification of FUS (fused in sarcoma)/TLS (translocated in liposarcoma) as an in vitro target of the PIKKs...
  12. doi RNA-binding protein TLS is a major nuclear aggregate-interacting protein in huntingtin exon 1 with expanded polyglutamine-expressing cells
    Hiroshi Doi
    Laboratory for Structural Neuropathology, RIKEN Brain Science Institute, 2 1 Hirosawa Wako shi, Saitama, Japan
    J Biol Chem 283:6489-500. 2008
    ..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 ..
  13. ncbi TLS facilitates transport of mRNA encoding an actin-stabilizing protein to dendritic spines
    Ritsuko Fujii
    Osaka Bioscience Institute, Suita, Osaka 565 0874, Japan
    J Cell Sci 118:5755-65. 2005
    b>TLS (translocation in liposarcoma), an RNA-binding protein, was originally identified as a heterogeneous ribonuclear protein (hnRNP)...
  14. ncbi The RNA binding protein TLS is translocated to dendritic spines by mGluR5 activation and regulates spine morphology
    Ritsuko Fujii
    Osaka Bioscience Institute, Suita, Osaka 565 0874, Japan
    Curr Biol 15:587-93. 2005
    ..TLS (translocated in liposarcoma), previously identified as a component of hnRNP complexes, unexpectedly showed somatodendritic ..
  15. ncbi Fus deficiency in mice results in defective B-lymphocyte development and activation, high levels of chromosomal instability and perinatal death
    G G Hicks
    Manitoba Institute of Cell Biology and the University of Manitoba, Winnipeg, Canada
    Nat Genet 24:175-9. 2000
    The gene FUS (also known as TLS (for translocated in liposarcoma) and hnRNP P2) is translocated with the gene encoding the transcription factor ERG-1 in human myeloid leukaemias...
  16. pmc Male sterility and enhanced radiation sensitivity in TLS(-/-) mice
    M Kuroda
    Skirball Institute of Biomolecular Medicine, the Departments of Medicine, Cell Biology and the Kaplan Cancer Center, NYU Medical Center, New York, NY 10016, USA
    EMBO J 19:453-62. 2000
    b>TLS (also known as FUS) is an RNA-binding protein that contributes the N-terminal half of fusion oncoproteins implicated in the development of human liposarcomas and leukemias...
  17. ncbi Oncoprotein TLS interacts with serine-arginine proteins involved in RNA splicing
    L Yang
    Medical Research Service, Veterans Affairs Puget Sound Health Care System, Seattle, Washington 98108, USA
    J Biol Chem 273:27761-4. 1998
    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...
  18. pmc Loss of mitochondrial protein Fus1 augments host resistance to Acinetobacter baumannii infection
    M Indriati Hood
    Department of Pathology, Microbiology, and Immunology, Vanderbilt University School of Medicine, Nashville, Tennessee
    Infect Immun 81:4461-9. 2013
    b>Fus1 is a tumor suppressor protein with recently described immunoregulatory functions. Although its role in sterile inflammation is being elucidated, its role in regulating immune responses to infectious agents has not been examined...
  19. doi ALS-associated mutations in FUS disrupt the axonal distribution and function of SMN
    Ewout J N Groen
    Department of Neuroscience and Pharmacology, Rudolf Magnus Institute of Neuroscience, University Medical Centre Utrecht, Utrecht, The Netherlands
    Hum Mol Genet 22:3690-704. 2013
    Mutations in the RNA binding protein fused in sarcoma/translated in liposarcoma (FUS/TLS) cause amyotrophic lateral sclerosis (ALS)...
  20. doi Interaction of FUS and HDAC1 regulates DNA damage response and repair in neurons
    Wen yuan Wang
    1 Picower Institute for Learning and Memory, Department of Brain and Cognitive Sciences, Massachusetts Institute of Technology, Cambridge, Massachusetts, USA 2 Howard Hughes Medical Institute, Chevy Chase, Maryland, USA 3
    Nat Neurosci 16:1383-91. 2013
    ..We found that FUS, an RNA/DNA-binding protein that has been linked to amyotrophic lateral sclerosis (ALS) and frontotemporal lobar ..
  21. pmc Highly efficient targeted mutagenesis in mice using TALENs
    Sudeepta Kumar Panda
    Institute of Developmental Genetics, Helmholtz Zentrum Munchen, German Research Center for Environmental Health, 85764 Munich, Germany
    Genetics 195:703-13. 2013
    ..we introduced amyotrophic lateral sclerosis patient-derived missense mutations in the fused in sarcoma (Fus) gene at a rate of 6.8%...
  22. pmc PARP-1 dependent recruitment of the amyotrophic lateral sclerosis-associated protein FUS/TLS to sites of oxidative DNA damage
    Stuart L Rulten
    Genome Damage and Stability Centre, School of Life Sciences, University of Sussex, Falmer, Brighton, BN1 9RQ, UK and School of Life Sciences, University of Sussex, Falmer, Brighton, BN1 9QG
    Nucleic Acids Res 42:307-14. 2014
    ..this disease encode proteins involved in RNA processing, including fused-in-sarcoma/translocated-in-sarcoma (FUS/TLS)...
  23. pmc A novel radioprotective function for the mitochondrial tumor suppressor protein Fus1
    E M Yazlovitskaya
    Department of Medicine, Division of Nephrology, Vanderbilt University, Nashville, TN 37232, USA
    Cell Death Dis 4:e687. 2013
    b>FUS1/TUSC2 is a mitochondrial tumor suppressor with activity to regulate cellular oxidative stress by maintaining balanced ROS production and mitochondrial homeostasis...
  24. pmc The multifunctional protein fused in sarcoma (FUS) is a coactivator of microphthalmia-associated transcription factor (MITF)
    Agnieszka Bronisz
    From the Department of Molecular and Cellular Biochemistry, Comprehensive Cancer Center, and
    J Biol Chem 289:326-34. 2014
    ..Here, we demonstrate that the proto-oncogene fused in sarcoma (FUS), the chromatin remodeling ATPase BRG1, and MITF form a trimeric complex that is regulated by phosphorylation of ..
  25. pmc Gamma motor neurons survive and exacerbate alpha motor neuron degeneration in ALS
    Mélanie Lalancette-Hébert
    Center for Motor Neuron Biology and Disease, Columbia University, New York, NY 10032
    Proc Natl Acad Sci U S A 113:E8316-E8325. 2016
    ..ALS expressing mutant superoxide dismutase-1 (SOD1), TAR DNA-binding protein 43 (TDP-43), and fused in sarcoma (FUS), we demonstrate selective degeneration of alpha MNs (α-MNs) and complete sparing of gamma MNs (γ-MNs), which ..
  26. pmc ALS-associated mutation FUS-R521C causes DNA damage and RNA splicing defects
    Haiyan Qiu
    J Clin Invest 124:981-99. 2014
    Autosomal dominant mutations of the RNA/DNA binding protein FUS are linked to familial amyotrophic lateral sclerosis (FALS); however, it is not clear how FUS mutations cause neurodegeneration...
  27. pmc Multistep process of FUS aggregation in the cell cytoplasm involves RNA-dependent and RNA-independent mechanisms
    Tatyana A Shelkovnikova
    School of Biosciences, Cardiff University, Museum Avenue, Cardiff CF10 3AX, UK, Institute of Physiologically Active Compounds Russian Academy of Sciences, 1 Severniy Proezd, Chernogolovka 142432, Moscow Region, Russian Federation and
    Hum Mol Genet 23:5211-26. 2014
    Fused in sarcoma (FUS) is an RNA-binding protein involved in pathogenesis of several neurodegenerative diseases...
  28. doi Autophagy regulates amyotrophic lateral sclerosis-linked fused in sarcoma-positive stress granules in neurons
    Hyun Hee Ryu
    Department of Biotechnology and Biological Sciences, Hannam University, Daejeon, Korea Department of Life Science, Chung Ang University, Seoul, Korea
    Neurobiol Aging 35:2822-31. 2014
    Mutations in fused in sarcoma (FUS), a DNA/RNA binding protein, have been associated with familial amyotrophic lateral sclerosis (fALS), which is a fatal neurodegenerative disease that causes progressive muscular weakness and has ..
  29. pmc Cytoplasmic sequestration of FUS/TLS associated with ALS alters histone marks through loss of nuclear protein arginine methyltransferase 1
    Michael Tibshirani
    Montreal Neurological Institute and Department of Neurology Neurosurgery, McGill University, Montreal, Quebec, Canada H3A 2B4
    Hum Mol Genet 24:773-86. 2015
    Mutations in the RNA-binding protein FUS/TLS (FUS) have been linked to the neurodegenerative disease amyotrophic lateral sclerosis (ALS)...
  30. pmc Activity-dependent FUS dysregulation disrupts synaptic homeostasis
    Chantelle F Sephton
    Departments of Neuroscience and Institut Universitaire en Santé Mentale de Québec, Department of Psychiatry and Neuroscience, Universite Laval, Quebec City, QC, Canada G1J 2G3
    Proc Natl Acad Sci U S A 111:E4769-78. 2014
    The RNA-binding protein fused-in-sarcoma (FUS) has been associated with amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD), two neurodegenerative disorders that share similar clinical and pathological ..
  31. pmc ALS-causative mutations in FUS/TLS confer gain and loss of function by altered association with SMN and U1-snRNP
    Shuying Sun
    1 Ludwig Institute for Cancer Research, University of California at San Diego, La Jolla, California 92093, USA 2 Department of Cellular and Molecular Medicine, University of California at San Diego, La Jolla, California 92093, USA
    Nat Commun 6:6171. 2015
    The RNA-binding protein FUS/TLS, mutation in which is causative of the fatal motor neuron disease amyotrophic lateral sclerosis (ALS), is demonstrated to directly bind to the U1-snRNP and SMN complexes...
  32. pmc FUS/TLS deficiency causes behavioral and pathological abnormalities distinct from amyotrophic lateral sclerosis
    Yoshihiro Kino
    Department of Neuroscience for Neurodegenerative Disorders, Juntendo University Graduate School of Medicine, Tokyo, Japan
    Acta Neuropathol Commun 3:24. 2015
    b>FUS/TLS is an RNA-binding protein whose genetic mutations or pathological inclusions are associated with neurological diseases including amyotrophic lateral sclerosis (ALS), frontotemporal lobar degeneration, and essential tremor (ET)...
  33. pmc FUS regulates AMPA receptor function and FTLD/ALS-associated behaviour via GluA1 mRNA stabilization
    Tsuyoshi Udagawa
    1 Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya 466 8550, Japan 2 Graduate School of Pharmaceutical Sciences, Tohoku University, Sendai 980 8578, Japan
    Nat Commun 6:7098. 2015
    b>FUS is an RNA/DNA-binding protein involved in multiple steps of gene expression and is associated with amyotrophic lateral sclerosis (ALS) and fronto-temporal lobar degeneration (FTLD)...
  34. pmc Early lethality and neuronal proteinopathy in mice expressing cytoplasm-targeted FUS that lacks the RNA recognition motif
    Hannah K Robinson
    a School of Biosciences, Cardiff University, Sir Martin Evans Building, Museum Avenue, Cardiff, UK
    Amyotroph Lateral Scler Frontotemporal Degener 16:402-9. 2015
    Mutations to the RNA binding protein, fused in sarcoma (FUS) occur in ∼5% of familial ALS and FUS-positive cytoplasmic inclusions are commonly observed in these patients...
  35. pmc ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function
    Aarti Sharma
    Department of Neurology, Center for Motor Neuron Biology and Disease, Columbia University, 630 W 168th Street, P and S Building, Room 5 423, New York, New York 10032, USA
    Nat Commun 7:10465. 2016
    Mutations in FUS cause amyotrophic lateral sclerosis (ALS), including some of the most aggressive, juvenile-onset forms of the disease...
  36. pmc Severe muscle wasting and denervation in mice lacking the RNA-binding protein ZFP106
    Douglas M Anderson
    Department of Molecular Biology, University of Texas Southwestern Medical Center, Dallas, TX 75390 Hamon Center for Regenerative Science and Medicine, University of Texas Southwestern Medical Center, Dallas, TX 75390 Senator Paul D Wellstone Muscular Dystrophy Cooperative Research Center, University of Texas Southwestern Medical Center, Dallas, TX 75390
    Proc Natl Acad Sci U S A 113:E4494-503. 2016
    ..These findings reveal a central role for Zfp106 in the maintenance of nerve-muscle signaling, and highlight the involvement of aberrant RNA processing in neuromuscular disease pathogenesis. ..
  37. pmc Haploinsufficiency for Core Exon Junction Complex Components Disrupts Embryonic Neurogenesis and Causes p53-Mediated Microcephaly
    Hanqian Mao
    Department of Molecular Genetics and Microbiology, Duke University School of Medicine, Durham, North Carolina, United States of America
    PLoS Genet 12:e1006282. 2016
    ..Altogether our study reveals new mechanisms to help explain how EJC mutations influence neurogenesis and underlie neurodevelopmental disease. ..
  38. doi PGC-1α regulates translocated in liposarcoma activity: role in oxidative stress gene expression
    Cristina Sánchez-Ramos
    Fundacion Centro Nacional de Investigaciones Cardiovasculares Carlos III, Madrid, Spain
    Antioxid Redox Signal 15:325-37. 2011
    b>Translocated in liposarcoma (TLS) is a poorly characterized multifunctional protein involved in the genotoxic response...
  39. doi TLS-ERG leukemia fusion protein deregulates cyclin-dependent kinase 1 and blocks terminal differentiation of myeloid progenitor cells
    Jing Pan
    Departments of Orthopedics and Medicine Hematology, University of Washington, 1660 South Columbian Way, GMR 151, Seattle, WA 98108
    Mol Cancer Res 6:862-72. 2008
    b>TLS-ERG fusion protein is derived from the t(16;21) translocation found in human myeloid leukemia...
  40. pmc FUS/TLS assembles into stress granules and is a prosurvival factor during hyperosmolar stress
    Reddy Ranjith K Sama
    Department of Neurology, University of Massachusetts Medical School, Worcester, Massachusetts, USA
    J Cell Physiol 228:2222-31. 2013
    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 ..
  41. pmc FUS regulates genes coding for RNA-binding proteins in neurons by binding to their highly conserved introns
    Tadashi Nakaya
    Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA
    RNA 19:498-509. 2013
    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 ..
  42. doi Reduction of polyglutamine toxicity by TDP-43, FUS and progranulin in Huntington's disease models
    Arnaud Tauffenberger
    CRCHUM, Universite de Montre al, Montreal, Quebec, Canada
    Hum Mol Genet 22:782-94. 2013
    The DNA/RNA binding proteins TAR DNA-binding protein 43 (TDP-43) and fused-in-sarcoma (FUS) are genetically linked to amyotrophic lateral sclerosis and frontotemporal lobar dementia, while the inappropriate cytoplasmic accumulations of ..
  43. pmc The effect of PRMT1-mediated arginine methylation on the subcellular localization, stress granules, and detergent-insoluble aggregates of FUS/TLS
    Atsushi Yamaguchi
    Department of Neurobiology, Graduate School of Medicine, Chiba University, Chiba, Japan
    PLoS ONE 7:e49267. 2012
    Fused in sarcoma/translocated in liposarcoma (FUS/TLS) is one of causative genes for familial amyotrophic lateral sclerosis (ALS)...
  44. doi FUS is not dysregulated by the spinal bulbar muscular atrophy androgen receptor polyglutamine repeat expansion
    Pietro Fratta
    Department of Neurodegenerative Disease, UCL Institute of Neurology, Queen Square, London, UK
    Neurobiol Aging 34:1516.e17-9. 2013
    ..Mutations in the FUS gene and fused in sarcoma (FUS) protein pathology have now been identified in some amyotrophic lateral sclerosis ..
  45. pmc Loss of fused in sarcoma (FUS) promotes pathological Tau splicing
    Denise Orozco
    German Center for Neurodegenerative Diseases DZNE, Munich, Germany
    EMBO Rep 13:759-64. 2012
    ..FTLD) patients present pathological redistribution and aggregation of the nuclear protein fused in sarcoma (FUS) in the cytoplasm...
  46. doi FUS-related proteinopathies: lessons from animal models
    Nicholas A Lanson
    Department of Genetics, Louisiana State University Health Sciences Center, New Orleans, LA 70112 2223, USA
    Brain Res 1462:44-60. 2012
    The recent identification of ALS-linked mutations in FUS and TDP-43 has led to a major shift in our thinking in regard to the potential molecular mechanisms of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (..
  47. doi Fused in sarcoma (FUS) interacts with the cytolinker protein plectin: implications for FUS subcellular localization and function
    Christer Thomsen
    Sahlgrenska Cancer Center, Department of Pathology, Sahlgrenska Academy at the University of Gothenburg, Box 425, 40530, Gothenburg, Sweden
    Exp Cell Res 318:653-61. 2012
    Fused in sarcoma (FUS) is a multifunctional protein involved in transcriptional control, pre-mRNA processing, RNA transport and translation...
  48. pmc Nuclear localization sequence of FUS and induction of stress granules by ALS mutants
    Jozsef Gal
    Department of Molecular and Cellular Biochemistry, College of Medicine, University of Kentucky, Lexington, KY 40536, USA
    Neurobiol Aging 32:2323.e27-40. 2011
    Mutations in fused in sarcoma (FUS) have been reported to cause a subset of familial amyotrophic lateral sclerosis (ALS) cases...
  49. ncbi Characterization of TFG in mus musculus and Caenorhabditis elegans
    M Mencinger
    Department of Clinical Genetics, University Hospital, Lund, S 221 85, Sweden
    Biochem Biophys Res Commun 257:67-73. 1999
    ..In situ hybridization with a TFG probe in 10, 5-day whole mouse embryos showed preferential staining of the limb buds, branchial arches, nasal processes, and brain, and weak staining of the primitive spinal cord and dorsal root ganglia...
  50. pmc Induction of a secreted protein by the myxoid liposarcoma oncogene
    M Kuroda
    Skirball Institute of Biomolecular Medicine, Departments of Medicine, Cell Biology, and the Kaplan Cancer Center, New York University Medical Center, New York, NY 10016, USA
    Proc Natl Acad Sci U S A 96:5025-30. 1999
    The TLS-CHOP oncoprotein, found in the majority of human myxoid liposarcomas, consists of a fusion between the transcription factor CHOP/GADD153 and the N terminus of an RNA-binding protein TLS/FUS...
  51. ncbi Characterization of a new brain-specific isoform of the EWS oncoprotein
    T Melot
    Laboratoire de Pathologie Moléculaire des Cancers, INSERM U509, Paris, France
    Eur J Biochem 268:3483-9. 2001
    EWS and related TAFII68 and TLS/FUS genes are fused with different genes encoding transcription factors in various human cancers...
  52. ncbi Mouse pigpen encodes a nuclear protein whose expression is developmentally regulated during craniofacial morphogenesis
    Sylvia R Alappat
    Department of Cell and Molecular Biology, Tulane University, New Orleans, Louisiana, USA
    Dev Dyn 228:59-71. 2003
    ..These data suggest that mouse Pigpen may activate transcription in vivo in response to specific growth factor signals and regulate proliferation and/or differentiation events during mouse organogenesis...
  53. pmc System-driven and oscillator-dependent circadian transcription in mice with a conditionally active liver clock
    Benoît Kornmann
    Department of Molecular Biology, University of Geneva, Geneva, Switzerland
    PLoS Biol 5:e34. 2007
    ..The finding that rhythmic mPer2 expression can be driven by both systemic cues and local oscillators suggests a plausible mechanism for the phase entrainment of subsidiary clocks in peripheral organs...
  54. pmc IGF1 is a common target gene of Ewing's sarcoma fusion proteins in mesenchymal progenitor cells
    Luisa Cironi
    Division of Experimental Pathology, Institute of Pathology CHUV, University of Lausanne, Lausanne, Switzerland
    PLoS ONE 3:e2634. 2008
    ..of Ewing's sarcoma family tumors (ESFT), the remaining 10-15% of cases expressing chimeric genes encoding EWS or FUS fused to one of several ets transcription factor family members, including ERG-1, FEV, ETV1 and ETV6...
  55. doi Pigpen, a nuclear coiled body component protein, is involved in angiogenesis
    Tomoko Yoshida
    Department of Nanomedicine, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan
    Cancer Sci 101:1170-6. 2010
    ..These data indicate that Pigpen is involved in angiogenesis and that pigpen may be a target for blocking tumor angiogenesis...
  56. doi FET family proto-oncogene Fus contributes to self-renewal of hematopoietic stem cells
    Takeaki Sugawara
    Department of Cellular and Molecular Medicine, Chiba University, Japan
    Exp Hematol 38:696-706. 2010
    b>Fus is the gene for a member of the FET family of RNA-binding proteins often involved in chromosomal translocations to generate oncogenic fusion genes in human cancers...
  57. pmc Sustained expression of TDP-43 and FUS in motor neurons in rodent's lifetime
    Cao Huang
    Department of Pathology, Anatomy and Cell Biology, Thomas Jefferson University, Philadelphia, PA 19107, USA
    Int J Biol Sci 6:396-406. 2010
    TAR DNA-binding protein (TDP-43) and fused in sarcoma (FUS) are two highly conserved ribonucleoproteins...
  58. doi FUS-CHOP fusion protein expression coupled to p53 deficiency induces liposarcoma in mouse but not in human adipose-derived mesenchymal stem/stromal cells
    Rene Rodriguez
    Andalusian Stem Cell Bank, Centro de Investigacion Biomedica, Consejería de Salud Universidad de Granada, Granada, Spain
    Stem Cells 29:179-92. 2011
    ..We attempted to develop a model of liposarcoma by expressing FUS (FUsed in Sarcoma; also termed TLS, Translocated in LipoSarcoma)-CHOP (C/EBP HOmologous Protein; also termed DDIT3, DNA Damage-Inducible Transcript 3), a hallmark ..

Research Grants2

  1. TLS and TLS Fusion Proteins in Leukemia
    Liu Yang; Fiscal Year: 2005
    ..myelogenous leukemia (AMIL), chronic myelogenous leukemia (CML) in blast crisis, and myelodysplastic syndromes, the TLS (translocation liposarcoma) gene is fused to the ERG (ets-related gene) gene through a recurrent t(16;21) ..
  2. CHONDROGENESIS AND HISTONE MODIFICATION ENZYMES
    Liu Yang; Fiscal Year: 2007
    ....