Genomes and Genes
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-like protein, fused in sarcoma, fusion gene in myxoid liposarcoma, heterogeneous nuclear ribonucleoprotein P2, oncogene FUS, oncogene TLS, translocated in liposarcoma protein
Publications257 found, 100 shown here
- ALS-associated mutations in TDP-43 increase its stability and promote TDP-43 complexes with FUS/TLSShuo Chien Ling
Ludwig Institute for Cancer Research and Department of Neuroscience, University of California at San Diego, La Jolla, CA 92093 0670, USA
Proc Natl Acad Sci U S A 107:13318-23. 2010..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 ..
- Loss and gain of FUS function impair neuromuscular synaptic transmission in a genetic model of ALSGary A B Armstrong
Department of Pathology and Cell Biology and Groupe de Recherche sur le Système Nerveux Central, Universite de Montreal, Montreal, QC, Canada H3C 3J7
Hum Mol Genet 22:4282-92. 2013..Mutations in the gene fused in sarcoma (FUS) are associated with ALS and cause impairment in motor function in animal models...
- FUS binds the CTD of RNA polymerase II and regulates its phosphorylation at Ser2Jacob C Schwartz
Howard Hughes Medical Institute
Genes Dev 26:2690-5. 2012Mutations 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...
- Mislocalised FUS mutants stall spliceosomal snRNPs in the cytoplasmValeria Gerbino
Fondazione Santa Lucia IRCCS, Rome, Italy Dipartimento di Biologia, Universita di Roma Tor Vergata, Rome, Italy
Neurobiol Dis 55:120-8. 2013..b>FUS, a nuclear protein, forms cytoplasmic aggregates in cells affected by amyotrophic lateral sclerosis (ALS), and ..
- FET proteins TAF15 and EWS are selective markers that distinguish FTLD with FUS pathology from amyotrophic lateral sclerosis with FUS mutationsManuela Neumann
Institute of Neuropathology, Schmelzbergstr 12, 8091 Zurich, Switzerland
Brain 134:2595-609. 2011Accumulation 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 ..
- p62/sequestosome 1 binds to TDP-43 in brains with frontotemporal lobar degeneration with TDP-43 inclusionsKunikazu Tanji
Department of Neuropathology, Institute of Brain Science, Hirosaki University Graduate School of Medicine, Hirosaki, Japan
J Neurosci Res 90:2034-42. 2012..Our results suggest that the interaction of TDP-43 and p62 is disrupted and may participate in the pathogenesis of TDP-43 proteinopathy...
- TLS-CHOP represses miR-486 expression, inducing upregulation of a metastasis regulator PAI-1 in human myxoid liposarcomaNariso Borjigin
Department of Molecular Pathology, Tokyo Medical University, Tokyo 160 8402, Japan
Biochem Biophys Res Commun 427:355-60. 2012Myxoid liposarcomas (MLSs) are characterized by t(12;16)(q13;p11) translocation and expression of TLS-CHOP chimeric oncoprotein. However, the molecular functions of TLS-CHOP have not been fully understood...
- Fused in sarcoma (FUS) protein lacking nuclear localization signal (NLS) and major RNA binding motifs triggers proteinopathy and severe motor phenotype in transgenic miceTatyana A Shelkovnikova
School of Biosciences, Cardiff University, Cardiff, Wales, United Kingdom
J Biol Chem 288:25266-74. 2013Dysfunction 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 ..
- Myxoid liposarcoma FUS-DDIT3 fusion oncogene induces C/EBP beta-mediated interleukin 6 expressionMelker Göransson
Lundberg Laboratory for Cancer Research LLCR, Department of Pathology, Goteborg University, Gothenburg, Sweden
Int J Cancer 115:556-60. 2005The myxoid/round cell liposarcoma oncogene FUS-DDIT3 is the result of a translocation derived gene fusion between the splicing factor FUS and DDIT3...
- Two families with familial amyotrophic lateral sclerosis are linked to a novel locus on chromosome 16qDeborah M Ruddy
Department of Medical and Molecular Genetics, Guy s, King s, and St Thomas School of Medicine, London, United Kingdom
Am J Hum Genet 73:390-6. 2003..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...
- The multifunctional FUS, EWS and TAF15 proto-oncoproteins show cell type-specific expression patterns and involvement in cell spreading and stress responseMattias K Andersson
Lundberg Laboratory for Cancer Research, Department of Pathology, Sahlgrenska Academy at Goteborg University, Goteborg, Sweden
BMC Cell Biol 9:37. 2008b>FUS, EWS and TAF15 are structurally similar multifunctional proteins that were first discovered upon characterization of fusion oncogenes in human sarcomas and leukemias...
- Structural basis and specificity of human otubain 1-mediated deubiquitinationMariola J Edelmann
Henry Wellcome Building for Molecular Physiology, Department of Clinical Medicine, University of Oxford, Roosevelt Drive, Oxford OX37BN, UK
Biochem J 418:379-90. 2009..spectrometry) experiments demonstrated that FUS [fusion involved in t(12;6) in malignant liposarcoma; also known as TLS (translocation in liposarcoma) or CHOP (CCAAT/enhancer-binding protein homologous protein)] and RACK1 [receptor for ..
- The occurrence of mutations in FUS in a Belgian cohort of patients with familial ALSP Van Damme
Department of Neurology, Leuven University Hospital, Herestraat 49, 3000 Leuven, Belgium
Eur J Neurol 17:754-6. 2010Mutations 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.
- A comparative clinical, pathological, biochemical and genetic study of fused in sarcoma proteinopathiesTammaryn Lashley
Queen Square Brain Bank, Institute of Neurology, Queen Square, London WC1N 3BG, UK
Brain 134:2548-64. 2011..Recently, mutations in the fused in sarcoma gene have been shown to cause familial amyotrophic lateral sclerosis and fused in sarcoma-positive neuronal ..
- 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 mutationsManuela Neumann
Institute of Neuropathology, University Hospital Zurich, Switzerland
Acta Neuropathol 124:705-16. 2012Accumulation 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 ..
- Expression patterns of the human sarcoma-associated genes FUS and EWS and the genomic structure of FUSP Aman
Department of Clinical Genetics, University Hospital of Lund, Sweden
Genomics 37:1-8. 1996FUS (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)...
- FUS-immunoreactive inclusions are a common feature in sporadic and non-SOD1 familial amyotrophic lateral sclerosisHan Xiang Deng
Division of Neuromuscular Medicine, Davee Department of Neurology and Clinical Neurosciences, Chicago, IL 60611, USA
Ann Neurol 67:739-48. 2010..Recent studies have shown that mutations in FUS are causal in approximately 4 to 5% of FALS and some apparent SALS cases...
- FUS, TARDBP, and SOD1 mutations in a Taiwanese cohort with familial ALSChing Paio Tsai
Department of Neurology, National Yang Ming University School of Medicine, Taipei, Taiwan
Neurobiol Aging 32:553.e13-21. 2011..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...
- mRNA and protein levels of FUS, EWSR1, and TAF15 are upregulated in liposarcomaJessica I Spitzer
Howard Hughes Medical Institute, Laboratory of RNA Molecular Biology, The Rockefeller University, New York, NY 10065, USA
Genes Chromosomes Cancer 50:338-47. 2011Translocations or mutations of FUS, EWSR1, and TAF15 (FET) result in distinct genetic diseases...
- Structural and energetic basis of ALS-causing mutations in the atypical proline-tyrosine nuclear localization signal of the Fused in Sarcoma protein (FUS)Zi Chao Zhang
Department of Pharmacology, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA
Proc Natl Acad Sci U S A 109:12017-21. 2012Mutations in the proline/tyrosine-nuclear localization signal (PY-NLS) of the Fused in Sarcoma protein (FUS) cause amyotrophic lateral sclerosis (ALS)...
- Oncoprotein TLS interacts with serine-arginine proteins involved in RNA splicingL Yang
Medical Research Service, Veterans Affairs Puget Sound Health Care System, Seattle, Washington 98108, USA
J Biol Chem 273:27761-4. 1998The 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...
- TLS-ERG leukemia fusion protein inhibits RNA splicing mediated by serine-arginine proteinsL Yang
Medical Research Service, VA Puget Sound Health Care System, Seattle, Washington 98108, USA
Mol Cell Biol 20:3345-54. 2000The 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...
- Genomic structure of the human RBP56/hTAFII68 and FUS/TLS genesF Morohoshi
Radiobiology Division, National Cancer Center Research Institute, 5 1 1 Tsukiji, Chuo Ku, Tokyo 104 0045, Japan
Gene 221:191-8. 1998..isolated RBP56 cDNA by PCR using mixed primers designed from the conserved sequences of the RNA binding domain of FUS/TLS and EWS proteins...
- Human POMp75 is identified as the pro-oncoprotein TLS/FUS: both POMp75 and POMp100 DNA homologous pairing activities are associated to cell proliferationP Bertrand
CEA, DSV, DRR, CNRS UMR 217, Fontenay aux Roses, France
Oncogene 18:4515-21. 1999..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...
- The myxoid/round cell liposarcoma fusion oncogene FUS-DDIT3 and the normal DDIT3 induce a liposarcoma phenotype in transfected human fibrosarcoma cellsKatarina Engström
Department of Oncology, Lundberg Laboratory for Cancer Research, Sahlgrenska University Hospital, Gothenburg, Sweden
Am J Pathol 168:1642-53. 2006..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/..
- RNA-binding protein TLS is a major nuclear aggregate-interacting protein in huntingtin exon 1 with expanded polyglutamine-expressing cellsHiroshi 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 disease cell model ..
- Temperature-dependent localization of TLS-CHOP to splicing factor compartmentsMelker Göransson
Lundberg Laboratory for Cancer Research, Department of Pathology, Goteborg University, Gula straket 8, S 413 45 Gothenburg, Sweden
Exp Cell Res 278:125-32. 2002The myxoid/round cell liposarcoma oncogene TLS-CHOP belongs to a growing family of tumor type specific fusion genes generated by chromosome translocations...
- Nucleolar localization of DGCR8 and identification of eleven DGCR8-associated proteinsAiko Shiohama
Department of Molecular Biology, Keio University School of Medicine, Tokyo, Japan GSP Center, The Leading Institute of Keio University, Ibaraki, Japan
Exp Cell Res 313:4196-207. 2007..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...
- Rethinking ALS: the FUS about TDP-43Clotilde Lagier-Tourenne
Department of Cellular and Molecular Medicine, University of California San Diego, Ludwig Institute for Cancer Research, La Jolla, CA 92093 0670, USA
Cell 136:1001-4. 2009..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...
- Multiple system degeneration with basophilic inclusions in Japanese ALS patients with FUS mutationTakahisa Tateishi
Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, 3 1 1 Maidashi, Higashi ku, Fukuoka, 812 8582, Japan
Acta Neuropathol 119:355-64. 2010Mutations in the fused in sarcoma gene (FUS) were recently found in patients with familial amyotrophic lateral sclerosis (ALS)...
- FUS immunogold labeling TEM analysis of the neuronal cytoplasmic inclusions of neuronal intermediate filament inclusion disease: a frontotemporal lobar degeneration with FUS proteinopathyTristan Page
Electron Microscope Division, Sussex Centre for Advanced Microscopy, John Maynard Smith Building, School of Life Sciences, University of Sussex, Falmer, Brighton, BN1 9QG, East Sussex, UK
J Mol Neurosci 45:409-21. 2011b>Fused in sarcoma (FUS)-immunoreactive neuronal and glial inclusions define a novel molecular pathology called FUS proteinopathy...
- ALS mutations in FUS cause neuronal dysfunction and death in Caenorhabditis elegans by a dominant gain-of-function mechanismTetsuro Murakami
Tanz Centre for Research in Neurodegenerative Diseases, University of Toronto, Toronto, Ontario, Canada
Hum Mol Genet 21:1-9. 2012It 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...
- Requirements for stress granule recruitment of fused in sarcoma (FUS) and TAR DNA-binding protein of 43 kDa (TDP-43)Eva Bentmann
Adolf Butenandt Institute, Biochemistry, Ludwig Maximilians University, Munchen, Germany
J Biol Chem 287:23079-94. 2012Cytoplasmic 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)...
- Expression of Fused in sarcoma mutations in mice recapitulates the neuropathology of FUS proteinopathies and provides insight into disease pathogenesisChristophe Verbeeck
Department of Neuroscience, Mayo Clinic, Jacksonville, FL, USA
Mol Neurodegener 7:53. 2012Mutations 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)...
- RNA seeds higher-order assembly of FUS proteinJacob C Schwartz
Howard Hughes Medical Institute, Department of Chemistry and Biochemistry, BioFrontiers Institute, University of Colorado, Boulder, CO 80309, USA
Cell Rep 5:918-25. 2013The 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...
- Human 75-kDa DNA-pairing protein is identical to the pro-oncoprotein TLS/FUS and is able to promote D-loop formationH Baechtold
Basel Institute for Immunology, Grenzacherstrasse 487, CH 4005 Basel, Switzerland
J Biol Chem 274:34337-42. 1999..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...
- Involvement of the pro-oncoprotein TLS (translocated in liposarcoma) in nuclear factor-kappa B p65-mediated transcription as a coactivatorH Uranishi
Department of Molecular Genetics and First Department of Internal Medicine, Nagoya City University Medical School, 1 Kawasumi, Mizuho cho, Mizuho ku, Nagoya, Aichi 467 8601, Japan
J Biol Chem 276:13395-401. 2001In 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 ..
- 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 diseaseChitta S Kasyapa
Department of Cancer Genetics, Roswell Park Cancer Institute, Elm and Carlton Streets, Buffalo, NY 14263, USA
Exp Cell Res 309:78-85. 2005..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 ..
- Induced ncRNAs allosterically modify RNA-binding proteins in cis to inhibit transcriptionXiangting 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 myxoid liposarcoma FUS-DDIT3 fusion oncoprotein deregulates NF-kappaB target genes by interaction with NFKBIZM Goransson
Lundberg Laboratory for Cancer Research LLCR, Department of Pathology, Sahlgrenska Academy at University of Gothenburg, Gothenburg, Sweden
Oncogene 28:270-8. 2009b>FUS (also called TLS), EWSR1 and TAF15 (also called TAF2N) are related genes involved in tumor type-specific fusion oncogenes in human malignancies...
- Mutations in the FUS/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosisT J Kwiatkowski
Department of Neurology, Massachusetts General Hospital, 114 16th Street, Charlestown, MA 02129, USA
Science 323:1205-8. 2009..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...
- Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6Caroline Vance
Department of Clinical Neuroscience, King s College London, Medical Research Council MRC Centre for Neurodegeneration Research, Institute of Psychiatry, London SE5 8AF, UK
Science 323:1208-11. 2009..We have identified a missense mutation in the gene encoding fused in sarcoma (FUS) in a British kindred, linked to ALS6...
- Abundant FUS-immunoreactive pathology in neuronal intermediate filament inclusion diseaseManuela Neumann
Institute of Neuropathology, University Hospital of Zurich, Zurich, Switzerland
Acta Neuropathol 118:605-16. 2009..Mutations in the gene encoding the fused in sarcoma (FUS) protein have recently been identified as a cause of familial amyotrophic lateral sclerosis (ALS)...
- C-terminal FUS/TLS mutations in familial and sporadic ALS in GermanyCarsten Drepper
Institute for Clinical Neurobiology, Zinklesweg 10, University of Wuerzburg, D 97078 Wuerzburg, Germany
Neurobiol Aging 32:548.e1-4. 2011..ALS patients, 41 familial ALS cases and other motor neuron disease patients from Germany for mutations in the FUS/TLS gene...
- A Japanese ALS6 family with mutation R521C in the FUS/TLS gene: a clinical, pathological and genetic reportYukiko Yamamoto-Watanabe
Department of Neurology, Hirosaki University Graduate School of Medicine, Hirosaki, Aomori 036 8562, Japan
J Neurol Sci 296:59-63. 2010..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 ..
- FUS mutations in frontotemporal lobar degeneration with amyotrophic lateral sclerosisOriane Broustal
CRicm INSERM UMRS_975, Hopital de la Pitie Salpetriere, Paris, France
J Alzheimers Dis 22:765-9. 2010..were made in the knowledge of amyotrophic lateral sclerosis (ALS) with the recent identification of TARDBP and FUS mutations in familial ALS...
- TLS and PRMT1 synergistically coactivate transcription at the survivin promoter through TLS arginine methylationKun Du
Division of Gene Structure and Function, Research Center for Genomic Medicine, Saitama Medical University, 1397 1 Yamane, Hidaka shi, Saitama ken 350 1241, Japan
Biochem Biophys Res Commun 404:991-6. 2011TLS (Translocated in LipoSarcoma), also termed FUS, is a multifunctional protein implicated in diverse cellular events such as maintaining genome integrity and regulating gene expression...
- Motor neuron apoptosis and neuromuscular junction perturbation are prominent features in a Drosophila model of Fus-mediated ALSRuohan Xia
Markey Cancer Center, University of Kentucky, Lexington, KY 40536, USA
Mol Neurodegener 7:10. 2012..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...
- Overexpression of human wild-type FUS causes progressive motor neuron degeneration in an age- and dose-dependent fashionJacqueline C Mitchell
Department of Clinical Neuroscience, Kings College London, King s Centre for Neurodegeneration Research, London SE5 8AF, UK
Acta Neuropathol 125:273-88. 2013..Cytoplasmic inclusions of fused in sarcoma (FUS) are the hallmark of several forms of FTLD and ALS patients with mutations in the FUS gene...
- FUS-NLS/Transportin 1 complex structure provides insights into the nuclear targeting mechanism of FUS and the implications in ALSChunyan Niu
Laboratory of Non coding RNA, Institute of Biophysics, Chinese Academy of Sciences, Beijing, China
PLoS ONE 7:e47056. 2012The 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...
- TARDBP and FUS mutations associated with amyotrophic lateral sclerosis: summary and updateSerena Lattante
institut du cerveau et de la moelle épinière, Centre de Recherche, CHU Pitie Salpetriere, INSERM, UMR_S975, CRicm, F 75013, Paris, France
Hum Mutat 34:812-26. 2013..b>Fused in sarcoma (FUS) was found to be responsible for a previously identified ALS6 locus, being mutated in both FALS and SALS ..
- Fusion of the dominant negative transcription regulator CHOP with a novel gene FUS by translocation t(12;16) in malignant liposarcomaT H Rabbitts
MRC Laboratory of Molecular Biology, Cambridge, UK
Nat Genet 4:175-80. 1993..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...
- Fusion of CHOP to a novel RNA-binding protein in human myxoid liposarcomaA Crozat
Department of Medicine, NYU Medical Center, New York 10016
Nature 363:640-4. 1993..of the translocation-associated CHOP gene product revealed a fusion between CHOP and a gene provisionally named TLS (translocated in liposarcoma)...
- TLS (FUS) binds RNA in vivo and engages in nucleo-cytoplasmic shuttlingH Zinszner
Skirball Institute of Biomolecular Medicine, the Deparment of Medicine, NYU Medical Center, New York 10016, USA
J Cell Sci 110:1741-50. 1997b>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-..
- TLS (translocated-in-liposarcoma) is a high-affinity interactor for steroid, thyroid hormone, and retinoid receptorsC A Powers
Division of Molecular Endocrinology, New York University Medical Center 10016, USA
Mol Endocrinol 12:4-18. 1998..p65 was identified as TLS (translocated-in-liposarcoma), a recently identified member of the RNP family of nuclear RNA-binding proteins whose ..
- The transcription factor Spi-1/PU.1 interacts with the potential splicing factor TLSM Hallier
INSERM U 248, Institut Curie, 26, rue d Ulm, 75248 Paris Cedex 05, France
J Biol Chem 273:4838-42. 1998..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...
- The transcriptional repressor ZFM1 interacts with and modulates the ability of EWS to activate transcriptionD Zhang
Department of Molecular Genetics, Albert Einstein College of Medicine, Bronx, New York 10461, USA
J Biol Chem 273:18086-91. 1998..Furthermore, two proteins, TLS and hTAFII68, which have extensive homology to EWS, also interact with ZFM1...
- 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, PKRL R Saunders
Department of Microbiology and Immunology and Sylvester Comprehensive Cancer Center, University of Miami School of Medicine, Miami, Florida 33136, USA
J Biol Chem 276:32300-12. 2001..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...
- RNA splicing mediated by YB-1 is inhibited by TLS/CHOP in human myxoid liposarcoma cellsTimothy B Rapp
Department of Orthopaedics and Sports Medicine, School of Medicine, University of Washington, Seattle 98108, USA
J Orthop Res 20:723-9. 2002..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)...
- Identification of methylated proteins by protein arginine N-methyltransferase 1, PRMT1, with a new expression cloning strategyKazuhiro Wada
Department of Functional Genomics, Medical Research Institute, Tokyo Medical and Dental University, 1 5 45 Yushima, Bunkyo, Tokyo 113 8510, Japan
Biochim Biophys Acta 1591:1-10. 2002..This systematic screening of substrate proteins with the solid phase methylation reaction will contribute to identify new roles of PRMT family...
- Proto-oncoprotein TLS/FUS is associated to the nuclear matrix and complexed with splicing factors PTB, SRm160, and SR proteinsMichael Meissner
Institute of Cancer Research, Borschkegasse 8a, University of Vienna, A 1090 Vienna, Austria
Exp Cell Res 283:184-95. 2003b>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...
- Identification of two novel loci for dominantly inherited familial amyotrophic lateral sclerosisPeter C Sapp
Cecil B Day Laboratory for Neuromuscular Research, Massachusetts General Hospital East, Charlestown, MA 02129, USA
Am J Hum Genet 73:397-403. 2003..The analysis of these genes will delineate pathways implicated as determinants of motor-neuron viability and provide insights into possible therapies for ALS...
- Comparable transforming capacities and differential gene expression patterns of variant FUS/CHOP fusion transcripts derived from soft tissue liposarcomasMatthias Heinrich Martin Schwarzbach
Department of Surgery, University of Heidelberg, INF 110, 69120 Heidelberg, Germany
Oncogene 23:6798-805. 2004..It results in transcription of various chimeric FUS/CHOP fusion transcripts that encode different oncogenic proteins...
- Domain architectures and characterization of an RNA-binding protein, TLSYuko Iko
Biomolecular Engineering Research Institute, 6 2 3 Furuedai, Suita, Osaka 565 0874, Japan
J Biol Chem 279:44834-40. 2004Translocated 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...
- Beta-catenin interacts with the FUS proto-oncogene product and regulates pre-mRNA splicingSatoshi Sato
Chemotherapy Division and Cancer Proteomics Project, National Cancer Center Research Institute, Tokyo, Japan
Gastroenterology 129:1225-36. 2005..In this study, we used a proteomics approach to obtain further insight into the functional properties of nuclear beta-catenin...
- Two Italian kindreds with familial amyotrophic lateral sclerosis due to FUS mutationAdriano Chio
ALS Center, Department of Neuroscience, University of Turin, Turin, Italy
Neurobiol Aging 30:1272-5. 2009Recently, 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)...
- A new subtype of frontotemporal lobar degeneration with FUS pathologyManuela Neumann
Institute of Neuropathology, University Hospital of Zurich, Zurich, Switzerland
Brain 132:2922-31. 2009..Mutations in the fused in sarcoma (FUS) gene have recently been identified as a cause of familial amyotrophic lateral sclerosis, with these ..
- Analysis of FUS gene mutation in familial amyotrophic lateral sclerosis within an Italian cohortN Ticozzi
Department of Neurology, University of Massachusetts Medical School, Worcester, Massachusetts, USA
Neurology 73:1180-5. 2009Mutations in the FUS gene on chromosome 16 have been recently discovered as a cause of familial amyotrophic lateral sclerosis (FALS)...
- Mutations in FUS cause FALS and SALS in French and French Canadian populationsV V Belzil
CHUM Research Centre, Notre Dame Hospital, Y 3633 Montreal, Quebec, Canada
Neurology 73:1176-9. 2009The 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 ..
- Mutations of FUS gene in sporadic amyotrophic lateral sclerosisLucia Corrado
Department of Medical Sciences, Via Solaroli, 17, 28100 Novara, Italy
J Med Genet 47:190-4. 2010Mutations in the FUS gene have recently been discovered to be a major cause of familial amyotrophic lateral sclerosis (FALS).
- FUS mutations in amyotrophic lateral sclerosis: clinical, pathological, neurophysiological and genetic analysisIan P Blair
Northcott Neuroscience Laboratory, ANZAC Research Institute, Concord Hospital, Sydney, Australia
J Neurol Neurosurg Psychiatry 81:639-45. 2010b>FUS gene mutations were recently identified in familial amyotrophic lateral sclerosis (ALS)...
- Genetic contribution of FUS to frontotemporal lobar degenerationT Van Langenhove
Neurodegenerative Brain Diseases Group, Department of Molecular Genetics, VIB, University of Antwerp CDE, Universiteitsplein 1, B 2610 Antwerpen, Belgium
Neurology 74:366-71. 2010Recently, the FUS gene was identified as a new causal gene for amyotrophic lateral sclerosis (ALS) in approximately 4% of patients with familial ALS...
- FUS mutations in sporadic amyotrophic lateral sclerosisShiao Lin Lai
Neuromuscular Diseases Research Group, Laboratory of Neurogenetics, National Institute on Aging, NIH, Bethesda, MD 20892, USA
Neurobiol Aging 32:550.e1-4. 2011Mutations 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...
- FALS with FUS mutation in Japan, with early onset, rapid progress and basophilic inclusionNaoki Suzuki
Department of Neurology, Tohoku University School of Medicine, Sendai, Japan
J Hum Genet 55:252-4. 2010Mutations 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...
- De novo truncating FUS gene mutation as a cause of sporadic amyotrophic lateral sclerosisMariely DeJesus-Hernandez
Department of Neuroscience, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 32224, USA
Hum Mutat 31:E1377-89. 2010Mutations 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...
- Novel FUS/TLS mutations and pathology in familial and sporadic amyotrophic lateral sclerosisChristopher Hewitt
Academic Unit of Neurology, University of Sheffield, Medical School, Beech Hill Road, Sheffield S10 2RX, England
Arch Neurol 67:455-61. 2010To 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.
- Fus gene mutations in familial and sporadic amyotrophic lateral sclerosisRosa Rademakers
Department of Neuroscience, Mayo Clinic, Jacksonville, Florida, USA
Muscle Nerve 42:170-6. 2010Mutations 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)]...
- ALS-associated fused in sarcoma (FUS) mutations disrupt Transportin-mediated nuclear importDorothee Dormann
DZNE German Center for Neurodegenerative Diseases, Munich, Germany
EMBO J 29:2841-57. 2010Mutations in fused in sarcoma (FUS) are a cause of familial amyotrophic lateral sclerosis (fALS)...
- Novel missense and truncating mutations in FUS/TLS in familial ALSS Waibel
Department of Neurology, University of Ulm, Oberer Eselsberg 45, 89081 Ulm, Germany
Neurology 75:815-7. 2010Mutations in the FUS/TLS gene have been associated with familial amyotrophic lateral sclerosis (FALS).
- Frameshift and novel mutations in FUS in familial amyotrophic lateral sclerosis and ALS/dementiaJ Yan
Division of Neuromuscular Medicine, Davee Department of Neurology and Clinical Neurosciences, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA
Neurology 75:807-14. 2010..Mutations in the FUS gene were identified in patients with familial ALS (FALS) and patients with sporadic ALS (SALS) from a variety of ..
- Juvenile ALS with basophilic inclusions is a FUS proteinopathy with FUS mutationsD Baumer
Department of Neuropathology, John Radcliffe Hospital, Oxford, OX3 9DU, UK
Neurology 75:611-8. 2010..The nosologic position of this type of ALS in the molecular pathologic and genetic classification of ALS is unknown...
- Mutant FUS proteins that cause amyotrophic lateral sclerosis incorporate into stress granulesDaryl A Bosco
Department of Neurology, University of Massachusetts Medical School, 55 Lake Avenue North, Worcester, MA 01655, USA
Hum Mol Genet 19:4160-75. 2010Mutations 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...
- Amyotrophic lateral sclerosis-associated proteins TDP-43 and FUS/TLS function in a common biochemical complex to co-regulate HDAC6 mRNASang Hwa Kim
Department of Pharmacology, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin 53706, USA
J Biol Chem 285:34097-105. 2010..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...
- TDP-43 and FUS in amyotrophic lateral sclerosis and frontotemporal dementiaIan Ra Mackenzie
Department of Pathology and Laboratory Medicine, Vancouver General Hospital, Vancouver, BC, Canada
Lancet Neurol 9:995-1007. 2010..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 ..
- Identification of a FUS splicing mutation in a large family with amyotrophic lateral sclerosisVeronique V Belzil
Center of Excellence in Neuromics of Université de Montréal, CHUM Research Center, Montreal, Quebec, Canada
J Hum Genet 56:247-9. 2011..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...
- FUS/TLS is a novel mediator of androgen-dependent cell-cycle progression and prostate cancer growthGreg N Brooke
Androgen Signalling Laboratory, Department of Surgery and Cancer, Imperial College London, Hammersmith Hospital Campus, United Kingdom
Cancer Res 71:914-24. 2011..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...
- Identification of novel FUS mutations in sporadic cases of amyotrophic lateral sclerosisVeronique V Belzil
Centre of Excellence in Neuromics of Université de Montréal, Quebec, Canada
Amyotroph Lateral Scler 12:113-7. 2011Mutations in the FUS gene have been recently associated with amyotrophic lateral sclerosis (ALS)...
- Nuclear transport impairment of amyotrophic lateral sclerosis-linked mutations in FUS/TLSDaisuke Ito
Department of Neurology, School of Medicine, Keio University, Tokyo, Japan
Ann Neurol 69:152-62. 2011The 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 ..
- FUS transgenic rats develop the phenotypes of amyotrophic lateral sclerosis and frontotemporal lobar degenerationCao Huang
Department of Pathology, Anatomy, and Cell Biology, Thomas Jefferson University, Philadelphia, Pennsylvania, United States of America
PLoS Genet 7:e1002011. 2011b>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)...
- FUS/TLS forms cytoplasmic aggregates, inhibits cell growth and interacts with TDP-43 in a yeast model of amyotrophic lateral sclerosisDmitry Kryndushkin
Laboratory of Biochemistry and Genetics, National Institute of Diabetes Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD 20892, USA
Protein Cell 2:223-36. 2011..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...
- A Drosophila model of FUS-related neurodegeneration reveals genetic interaction between FUS and TDP-43Nicholas A Lanson
Department of Genetics, Louisiana State University Health Sciences Center, New Orleans, LA 70112, USA
Hum Mol Genet 20:2510-23. 2011..b>Fused in sarcoma/translated in liposarcoma (FUS/TLS) and TAR DNA-binding protein (TDP)-43 are DNA/RNA-binding proteins found ..
- Molecular determinants and genetic modifiers of aggregation and toxicity for the ALS disease protein FUS/TLSZhihui Sun
Department of Cell and Developmental Biology, The University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, United States of America
PLoS Biol 9:e1000614. 2011TDP-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...
- A yeast model of FUS/TLS-dependent cytotoxicityShulin Ju
Department of Biochemistry and Chemistry, Rosenstiel Basic Medical Sciences Research Center, Brandeis University, Waltham, Massachusetts, United States of America
PLoS Biol 9:e1001052. 2011FUS/TLS is a nucleic acid binding protein that, when mutated, can cause a subset of familial amyotrophic lateral sclerosis (fALS)...
- Pathological heterogeneity in amyotrophic lateral sclerosis with FUS mutations: two distinct patterns correlating with disease severity and mutationIan R A Mackenzie
Department of Pathology, University of British Columbia and Vancouver General Hospital, Vancouver, BC V5Z 1M9, Canada
Acta Neuropathol 122:87-98. 2011Mutations 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)...
- The ALS-associated proteins FUS and TDP-43 function together to affect Drosophila locomotion and life spanJi Wu Wang
Center for Motor Neuron Biology and Disease, Columbia University Medical Center, New York, New York 10032, USA
J Clin Invest 121:4118-26. 2011..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 ..
- Conjoint pathologic cascades mediated by ALS/FTLD-U linked RNA-binding proteins TDP-43 and FUSDaisuke Ito
Department of Neurology, School of Medicine, Keio University, Tokyo, Japan
Neurology 77:1636-43. 2011The 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-..
- Arginine methylation by PRMT1 regulates nuclear-cytoplasmic localization and toxicity of FUS/TLS harbouring ALS-linked mutationsMiranda L Tradewell
Department of Neurology Neurosurgery and Montreal Neurological Institute, McGill University, Montreal, Quebec, Canada H3A 2B4
Hum Mol Genet 21:136-49. 2012Mutations in FUS/TLS (fused in sarcoma/translated in liposarcoma) cause an inheritable form of amyotrophic lateral sclerosis (ALS6)...
- Sporadic juvenile amyotrophic lateral sclerosis caused by mutant FUS/TLS: possible association of mental retardation with this mutationSatoshi Yamashita
Department of Neurology, Faculty of Life Sciences, Kumamoto University, 1 1 1 Honjo, Kumamoto 860 8556, Japan
J Neurol 259:1039-44. 2012..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...
- Accumulation of insoluble forms of FUS protein correlates with toxicity in DrosophilaLaetitia Miguel
UMR Inserm U614, IFRMP23, Rouen Institute for Medical Research and Innovation, Faculty of Medicine, University of Rouen, Rouen, France
Neurobiol Aging 33:1008.e1-15. 2012Recently, 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 ..
- Screening of the FUS gene in familial and sporadic amyotrophic lateral sclerosis patients of Chinese originZ Y Zou
Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
Eur J Neurol 19:977-83. 2012According 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...
- TLS/FUS (translocated in liposarcoma/fused in sarcoma) regulates target gene transcription via single-stranded DNA response elementsAdelene Y Tan
Department of Biological Sciences, Columbia University, New York, NY 10027, USA
Proc Natl Acad Sci U S A 109:6030-5. 2012b>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...
- 1H NMR STUDIES OF NON-HODGKINS LYMPHOMAJerry D Glickson; Fiscal Year: 2012..Here we are developing much more sensitive 1H NMR methods that can be used for early detection of response in all NHL patients. The same methods should be applicable to other prevalent forms of human cancer. ..
- Trial of mesna to prevent doxorubicin-induced plasma protein oxidation and TNFa rJeffrey A Moscow; Fiscal Year: 2012....
- Prognostic Models in Diffuse Large B-Cell LymphomaIzidore S Lossos; Fiscal Year: 2010..Further, establishment of a paraffin-based RNA prognostic model using the new methodology of RNA extraction could serve as a paradigm for other lymphomas and tumors. ..
- JULIE MARIE VOSE; Fiscal Year: 2014..This would represent a significant advance in the use of PET imaging for personalized therapy with an expectedly positive impact on patient management and/or outcome of patients with DLBCL. ..
- DARYL ANGELA BOSCO; Fiscal Year: 2016DESCRIPTION (provided by applicant): Mutations in the gene encoding Fused in Sarcoma/Translocated in Liposarcoma (FUS/TLS or FUS) were recently linked to amyotrophic lateral sclerosis (ALS). ALS is the most common motor neuron disorder...
- Jacob C Schwartz; Fiscal Year: 2014Project Summary/Abstract: Fused in Sarcoma (FUS) is a human RNA-binding protein, mutations in which can cause amyotrophic lateral sclerosis (ALS). ALS is a devastating neurodegenerative disease affecting 1 in 50000 people in the U.S...
- Sogol Mostoufi-Moab; Fiscal Year: 2016....
- Regulation of transcription by the RNA-binding protein FUSJacob C Schwartz; Fiscal Year: 2013This proposal will investigate RNA-directed regulation of transcription by the RNA- binding protein FUS. FUS has been shown to be translocated in liposarcomas, sarcomas and acute myeloid leukemia (AML)...
- Qinghua Sun; Fiscal Year: 2015....
- Robert H Brown; Fiscal Year: 2015..dominantly inherited familial ALS (fALS): Cu,Zn superoxide dismutase-1 (SOD1), the first identified ALS gene, and FUS/TLS. Respectively, these account for 20% and 5% of fALS cases...
- Molecular library screen for suppressors of FUS proteinopathyFrank P Shewmaker; Fiscal Year: 2013..Recently, the DNA/RNA-binding proteins FUS (Fused in Sarcoma) and TDP-43 (TAR DNA-binding protein) were implicated in these diseases...
- Developing in vivo and in vitro Models for ALS Caused by Mutant FUSXugang Xia; Fiscal Year: 2011..A recent advance in ALS research is the discovery of pathogenic mutations in the Fused in Sarcoma (FUS) gene in ALS. FUS is a highly conserved ribonucleoprotein encoded by the FUS gene of 15 exons...
- Randal J Kaufman; Fiscal Year: 2014..The proposed studies on protein synthesis will provide vital information that should encourage the development of novel therapeutics to treat diabetes. ..
- Udai B Pandey; Fiscal Year: 2016..b>FUS and TDP-43 are DNA/RNA binding proteins found to be mutated in both sporadic and familial forms of ALS...
- James L Manley; Fiscal Year: 2015..Lastly, the function of the Translocated in Liposarcoma (TLS) protein in coupling transcription-splicing will be investigated...
- HELAINA LEHRER; Fiscal Year: 2014..Mutations in two RNA binding proteins, TAR DNA binding protein 43 (TDP43) and Fused in sarcoma/translocated in liposarcoma (FUS/TLS or FUS), cause some forms of familial ALS...
- Zuoshang Xu; Fiscal Year: 2014..to cause familial ALS, which include mutations in SOD1, ALS2, VAPB, senataxin, dynactin, angiogenin, TDP-43, FUS, UBQLN2 and c9orf72 genes...
- Cellular and Animal Models of FUS Mutations in ALSEric J Huang; Fiscal Year: 2013..we propose to establish both cellular and animal models that will allow us to characterize the mechanism of FUS/TLS mutations...
- Teepu Siddique; Fiscal Year: 2016..The genetic causes of FALS have been linked to mutations in several genes such as superoxide dismutase, TDP-43, FUS, optineurin, ubiquilin 2 and C9ORF72. The etiology of SALS, however, remains elusive...
- Role of High Density Lipoprotein Particles in amyotrophic lateral sclerosisTeepu Siddique; Fiscal Year: 2012..The genetic causes of FALS have been linked to mutations in several genes such as superoxide dismutase, TDP-43, FUS, and optineurin. The etiology of SALS, however, remains elusive...
- Development of mouse models of optineurin-linked ALSHan Xiang Deng; Fiscal Year: 2013..Our data, together with those from previous studies of TDP43 and FUS provide important evidence supporting the hypothesis that: (I) most of ALS, including sporadic ALS and SOD1-..
- Scott R Barnum; Fiscal Year: 2014..are familial, associated with mutations in one of several genes including Cu,Zn SOD-1, Alsin, Senataxin, TDP-43 and FUS/TLS...
- Zhiyong Zhao; Fiscal Year: 2016..In Specific Aim 3, we will investigate whether TRAF1 triggers DISC formation by binding to TRADD and other DISC factors. We will, then, investigate whether TRAF1 causes NTDs in diabetic embryopathy using a traf1 knockout model. ..
- GLUTAMATE TRANSPORTERS IN THE CNSMICHAEL BYRNE ROBINSON; Fiscal Year: 2011..The name of the principal investigator/program director must be provided at the top of each printed page and each continuation page. RESEARCH GRANT TABLE OF CONTENTS Page Numbers Face Page i_ Description, ..
- ALS therapies and genomics for mutant TDP-43 and TLS/FUSDon W Cleveland; Fiscal Year: 2010..that mutations in two strikingly similar DNA/RNA binding proteins, TAR DNA-binding protein (TDP-43) and Fused in sarcoma (FUS/TLS), are also primary causes of ALS...
- Avoiding toxicity associated with MTP ablationM Mahmood Hussain; Fiscal Year: 2013..Proposed studies may lead to new therapeutic modalities for the treatment of various disorders associated with high plasma lipids. ..
- The role of human DNA polymerase eta in the mutagenic response to oxidative stresSCOTT DAVID MCCULLOCH; Fiscal Year: 2013..Translesion synthesis (TLS) by specialized polymerases of the Y-family is therefore a necessary component of DNA replication...
- Hesham M Amin; Fiscal Year: 2015..Our long-term goal is to understand IGF-IR-dependent signaling in this aggressive lymphoma in order to devise novel therapeutic strategies. Tactics that antagonize IGF-IR could potentially provide a cure for NPM-ALK+ ALCL patients. ..
- The Unfolded Protein Response in Human Airway and Alveolar Epithelial CellsSalim Merali; Fiscal Year: 2010..The results obtained are expected to improve our understanding of cigarette smoke-related lung disease and contribute to the development of new and better treatments. (End of Abstract) ..
- Haining Zhu; Fiscal Year: 2014..as their mutation can lead to familial ALS, including two genes encoding RNA processing proteins TDP-43 and fused in sarcoma/translocated in liposarcoma (FUS/TLS). FUS is a ubiquitously expressed multi-domain RNA-binding protein...
- Role of aberrant organelle stress responses in alcohol-induced liver injuryCheng Ji; Fiscal Year: 2012..This research is to understand how alcohol promotes injury processes in the two organelles and how organelle damages contribute to liver disease which will open new avenues for preventing and treating liver disease. ..
- Frank A Anania; Fiscal Year: 2016..Additional data indicate that exendin-4 suppresses CHOP expression both in vitro and in mice fed a high-fat diet, but in CHOP knockout mice fed an identical diet, fat-laden hepatocytes are protected from apoptotic death. ..
- Marina Gorbatyuk; Fiscal Year: 2014..This approach may overcome the genetic diversity of this disease and reveal the pathways of cell death that lead from mutation to retinal degeneration. ..
- Transcriptional coactivators and arginine methylationJennifer Butler; Fiscal Year: 2004..Two proteins, an RNA shuttling protein called TLS (translocated in liposarcoma) and PRMT2 (protein arginine methyltransferase 2), were identified as novel CBP-..
- Michelle Denburg; Fiscal Year: 2016....
- Matthew M Lavail; Fiscal Year: 2014..abstract_text> ..
- Stuart H Friess; Fiscal Year: 2014..The goal of this proposal is to develop new and effective therapies to treat acute traumatic brain injury in children utilizing an immature large animal model. ..
- Makoto Miyazaki; Fiscal Year: 2016..Specific Aim 3: Elucidate molecular mechanisms by which ATF4 regulates osteoblastic differentiation and mineralization of VSMCs. ..
- Craig L Bennett; Fiscal Year: 2014..However, the recent discovery of the involvement of TDP-43 and FUS in familial and sporadic ALS (FALS and SALS) reemphasizes the role of altered RNA processing in ALS...
- DECISION SUPPORT SYSTEM TO GUIDE PEDIATRIC PHARMACOTHERAPYJEFFREY SCOTT BARRETT; Fiscal Year: 2010..The solution (PPDSS dashboards and installation guidance) will be provided to all who request as part of our data-sharing plan. ..
- Podocyte TRB in Diabetic NephropathyROBYN A CUNARD; Fiscal Year: 2012..Accordingly, we believe that TRB3 may prove to be an important therapeutic target in diabetic nephropathy. ..
- Rachana Shah; Fiscal Year: 2015....
- KELLY ANN DOUGHERTY; Fiscal Year: 2014..Well-designed trials are urgently needed to determine the clinical impact of vit A supplementation and this study is the next step. ..
- Mahlon Collins; Fiscal Year: 2014..in motor neurons similar in appearance to those containing the other ALS-associated RNA binding proteins TDP-43 and FUS. We now seek to extend these preliminary observations by studying the normal function of RBM45 as well as its ..
- Transgenic Mouse Models of FUS/TLS-Mediated Amyotrophic Lateral SclerosisZuoshang Xu; Fiscal Year: 2010..Recently mutations in a new gene, FUS/TLS, have been identified to cause ~5% of familial ALS cases...
- Alexis A Topjian; Fiscal Year: 2016....
- Paolo Casali; Fiscal Year: 2016..We also argue that the translesion DNA synthesis (TLS) polymerase (pol) 8 and pol7 are critical in SHM of (auto)antibodies, by repairing DNA lesions after being ..
- Role of Ncb5or in Insulin ProductionHao Zhu; Fiscal Year: 2010..These studies may provide new insights into the pathogenesis and treatment of diabetes. ..
- Full Human Genome Sequencing in ALSRobert H Brown; Fiscal Year: 2010..participated in reports of additional ALS genes including alsin, VAPB, and most recently FIG4, ELP3 and FUS. This group led the multinational genome analysis that identified variants in KIFAP3 as modifiers of survival in ..
- William J Snell; Fiscal Year: 2016..Pre- fusion attachment between gamete membranes is governed by a species-specific plus gamete-specific protein FUS1, and subsequent membrane merger depends on a broadly conserved, minus gamete-specific protein, HAP2...
- High Resolution Peripheral Quantitative CTMARY BETH LEONARD; Fiscal Year: 2013..We have a detailed and ready-to-execute plan for operation that includes technical expertise and support, and full institutional commitment. ..
- Combination immunotherapy with gene modified CD4 and CD8 T cells and stem cellsCarl H June; Fiscal Year: 2013....
- Richard J Price; Fiscal Year: 2016..guided drug-delivery approach that couples magnetic resonance (MR)-targeted BBB opening via focused ultrasound (FUS) and microbubbles (MBs) with drug-loaded nanoparticles that have been engineered with extremely dense polyethylene ..
- Sensimmer Virtual Phaco Trainer for Cataract SurgeryP Pat Banerjee; Fiscal Year: 2011..Following STTR Phases I and II, the SENSIMMER Virtual Phaco Trainer will include well developed modules for the two most difficult part-tasks of cataract surgery: capsulorrhexis and phacoemulsification. ..
- MICHAEL BYRNE ROBINSON; Fiscal Year: 2015..5 million from NICHD). The Center includes an excess of 70 Penn faculty at 15 departments at the Schools of Medicine, Veterinary Medicine, Nursing, the Wistar Institute, and the College of Arts and Sciences. ..
- GENETIC AND MOLECULAR ANALYSIS OF YEAST DNA REPLICATIONRobert A Sclafani; Fiscal Year: 2012..3, we investigate the fidelity of DNA replication and the regulation of mutagenesis by studying the role of DDK in TLS (trans-lesion synthesis)...
- Installation of Energy-Efficient Cage Washers for Childrens Hospital VivariumPhilip R Johnson; Fiscal Year: 2012..These new colonies must first be rederived (creation of pathogen- free mice) prior to transfer to the CTRB barrier. ..
- Jack Roth; Fiscal Year: 2016..Therapeutic Targeting of Telomerase in Lung Cancer;and 5. Translation of the 3p21.3 Gene FUS1 into Pathway- Targeted Molecular Therapy. The Cores are: (A) Administrative;(B) Pathology &Tissue Resources;(C)...
- ROBIN E REED; Fiscal Year: 2015..The protein FUS, which associates with U1 snRNP, SR proteins, and RNA polymerase II, was identified as a strong candidate for ..
- Sara E Pinney; Fiscal Year: 2015....
- Leonard Petrucelli; Fiscal Year: 2014..Our hypothesis is that loss of functional PGRN leads to loss of functional TDP-43, which leads to cell death. ..
- Cyrus Vaziri; Fiscal Year: 2016..tolerance of bulky DNA adducts (such as B[a]P- and UV-induced lesions) via Trans-Lesion Synthesis (TLS) and in repair of Double Strand Break (DSB) that arise during S-phase...
- Peter M Burgers; Fiscal Year: 2015..Translesion synthesis (TLS) in response to DNA damage allows the cell to overcome damage that forms a terminal block for the regular ..
- Role of PDX1 in insulin biosynthesis and ER homeostasisCynthia Khoo; Fiscal Year: 2011..Studying the mechanism for PDXI regulation of insulin secretion and [unreadable] cell survival may lead to targets for treatment of type 2 diabetes. ..
- Metabolic regulation in growth factor-dependent cellsRalph J DeBerardinis; Fiscal Year: 2010....
- Calcineurin Inhibitor Minimization And Foxp3+ Tregs Post-TransplantWAYNE WILLIAM contact HANCOCK; Fiscal Year: 2010..Our studies of the effects of immunosuppression on regulatory T cells may revolutionize immunosuppressive therapy post- transplant and decrease patient morbidity and graft dysfunction long-term. ..
- mRNA Targets for TDP-43 and FUS/TLS: Identifying Key RNA-Processing Errors in ALSMagdalini Polymenidou; Fiscal Year: 2012..of ALS-causing mutations not only in TDP-43, but more recently, also in another RNA-binding protein, namely FUS/TLS, confirmed the primary role of these proteins in the pathogenesis of ALS...
- Role of ER Stress in the Pathogenesis of Primary Open Angle GlaucomaGULAB ZODE; Fiscal Year: 2013..This project will facilitate continued technical, intellectul, and professional training of the candidate, and assist the candidate in the establishment of an independent research laboratory at an academic research institute. ..
- Roles for TDP-43 and FUS in ALS Using Motor Neurons from Embryonic Stem CellsDara Ditsworth; Fiscal Year: 2012..Mutations in two DNA/RNA binding proteins, TAR DNA-binding protein (TDP-43) and Fused in Sarcoma (FUS) have recently been identified as primary causes of inherited ALS and have led to what is likely to be a ..
- REGULATION OF HEPATIC INSULIN EXTRACTIONKenneth S Polonsky; Fiscal Year: 2013....
- Transition Readiness of Adolescent and Young Adult Survivors of Childhood CancerLisa Schwartz; Fiscal Year: 2012..Thus, the proposed project has significant public health implications for advancing the study of transition in childhood cancer survivorship and other conditions to ultimately improve disease management and continuity of care. ..
- Comprehensive Clinical Decision Support for the Primary Care of Premature InfantsRobert W Grundmeier; Fiscal Year: 2010....
- Effect of Aging on Preadipocyte DifferentiationJames L Kirkland; Fiscal Year: 2012..This represents an entirely new way of thinking about mechanisms causing the fat redistribution, diabetes, and their complications so common in old age. ..
- Bypass of DNA lesions by translesion synthesis in yeastLouise Prakash; Fiscal Year: 2013Translesion synthesis (TLS) DNA polymerases (Pols) help maintain the continuity of the replication fork by mediating the bypass of DNA lesions...
- NEIL ALAN SHNEIDER; Fiscal Year: 2015..Mutations in the gene FUS (or TLS) were recently reported in rare ALS families, and FUS pathology has since been found in sporadic ALS, suggesting ..
- MOUSE MODELS OF DNA REPAIR - DEFECTIVE HUMAN DISEASESErrol Friedberg; Fiscal Year: 2011..This process is called translesion DNA synthesis (TLS)...
- Hamid Bassiri; Fiscal Year: 2015..abstract_text> ..
- Harmeet Malhi; Fiscal Year: 2016..Our results will yield mechanistic insights into regulation of macrophage lipoactivation and lipoapoptosis by individual UPR components, thus identifying potential molecules that can be targeted by therapeutic interventions. ..
- Deciphering RNA based mechanisms of neurodegenerationDaniela C Zarnescu; Fiscal Year: 2013..several RNA binding proteins have been linked to motor neuron disease, including senataxin, angiogenin, TDP-43 and FUS. These findings led to a paradigm shift in the current models for neuronal degeneration mechanisms and suggest that ..
- Mark D Sutton; Fiscal Year: 2016..coli replicase with those of translesion DNA synthesis DNA polymerases (TLS Pols). Our findings successfully challenged the well- established tool belt model...
- Anti-HIV Neuroimmunomodulatory Therapy with Neurokinin-1 (NK1-R) AntagonistsSTEVEN DANIEL DOUGLAS; Fiscal Year: 2013..This NK1RA has activity as an antiviral HIV agent that improves innate immunity (Natural Killer cells) and has positive neurobehavioral effects. NK1RA are a potential novel therapy for neuroAIDS...
- Keshav K Singh; Fiscal Year: 2014..The proposed studies should provide insight in to the mechanism involved in arsenic induced breast tumorigenesis. ..
- Makoto Miyazaki; Fiscal Year: 2016....
- Steven M Willi; Fiscal Year: 2016..and is strongly supported by access to a superb range of institutional resources, including the CHOP CTRC and the UPenn DERC. ..
- Michael E Shy; Fiscal Year: 2016..Dr. McDermott, from Rochester, will be the HNC Biostatistician. The Charcot Marie Tooth Association (CMTA), CMT United Kingdom (CMTUK) and TREAT-NMD organizations will interact extensively with the HNC. ..
- Raghunatha R Yammani; Fiscal Year: 2014....
- Raghu Vemuganti; Fiscal Year: 2014....
- Mortimer Poncz; Fiscal Year: 2016..At the end of the 5 years of support, we believe that important fundamental and clinically useful information will have been generated for a disease that remains highly clinically relevant. ..
- BRADLEY ALAN WARADY; Fiscal Year: 2016....
- Susan L Furth; Fiscal Year: 2016....
- Constantinos Koumenis; Fiscal Year: 2016..It is our supposition that findings stemming from work proposed herein will provide a foundation for the design of novel anti-cancer treatment strategies targeting this pathway. ..
- ANDREW LEWIS FELDMAN; Fiscal Year: 2016..intensified vs. experimental therapy. In the longer term, we believe our results will allow individualized selection of chemotherapy drugs based on the pattern of genetic abnormalities in PTCL tumor tissue. ..
- Chongyi Wei; Fiscal Year: 2015..tested, and attitudes and norms toward HIV testing;and 2) Conduct formative research for time-location sampling (TLS) among Chinese MSM...
- Mark Hedglin; Fiscal Year: 2014..Such a task may be carried out by translesion DNA synthesis (TLS), a unique process by which DNA is replicated past damage without repairing it by specialized TLS pols...
- High Throughput Chemical Genomic screening to identify novel ER stress-inducingANDREW MICHAEL FRIBLEY; Fiscal Year: 2010..The proposed studies will determine the ability of "hits" from this screen to interfere with the growth of or kill head and neck tumor cells. ..
- Identifying Strategies to Increase Engagement in Clinical Trials in Pediatric SCDLamia P Barakat; Fiscal Year: 2010....
- C-Reactive Protein in Human Mucosal ImmunityJane Gould; Fiscal Year: 2005..These results will be tested in vivo using murine models of respiratory tract infection with mice carrying the human CRP transgene. ..
- ALDO-KETO REDUCTASES AS PART OF CHEMICAL STRESS RESPONSEOleg Barski; Fiscal Year: 2002....
- C/EDB BETA AND CHOP IN EPIDERMAL DIFFERENTIATIONEdward Maytin; Fiscal Year: 2002....