Recent advances in the molecular pathogenesis of Ewing's sarcoma View Full Text


Ontology type: schema:ScholarlyArticle      Open Access: True


Article Info

DATE

2010-06-14

AUTHORS

E C Toomey, J D Schiffman, S L Lessnick

ABSTRACT

Tumor development is a complex process resulting from interplay between mutations in oncogenes and tumor suppressors, host susceptibility factors, and cellular context. Great advances have been made by studying rare tumors with unique clinical, genetic, or molecular features. Ewing's sarcoma serves as an excellent paradigm for understanding tumorigenesis because it exhibits some very useful and important characteristics. For example, nearly all cases of Ewing's sarcoma contain the (11;22)(q24;q12) chromosomal translocation that encodes the EWS/FLI oncoprotein. Besides the t(11;22), however, many cases have otherwise simple karyotypes with no other demonstrable abnormalities. Furthermore, it seems that an underlying genetic susceptibility to Ewing's sarcoma, if it exists, must be rare. These two features suggest that EWS/FLI is the primary mutation that drives the development of this tumor. Finally, Ewing's sarcoma is an aggressive tumor that requires aggressive treatment. Thus, improved understanding of the pathogenesis of this tumor will not only be of academic interest, but may also lead to new therapeutic approaches for individuals afflicted with this disease. The purpose of this review is to highlight recent advances in understanding the molecular pathogenesis of Ewing's sarcoma, while considering the questions surrounding this disease that still remain and how this knowledge may be applied to developing new treatments for patients with this highly aggressive disease. More... »

PAGES

4504-4516

References to SciGraph publications

  • 2009-01-14. Combined use of expression and CGH arrays pinpoints novel candidate genes in Ewing sarcoma family of tumors in BMC CANCER
  • 2002-06-07. Prognostic impact of chromosomal aberrations in Ewing tumours in BRITISH JOURNAL OF CANCER
  • 2002-03-01. Effectiveness of insulin-like growth factor I receptor antisense strategy against Ewing's sarcoma cells in CANCER GENE THERAPY
  • 2001-03-29. Phosphorylation of the EWS IQ domain regulates transcriptional activity of the EWS/ATF1 and EWS/FLI1 fusion proteins in ONCOGENE
  • 1999-10. Repression of the gene encoding the TGF-β type II receptor is a major target of the EWS-FLI1 oncoprotein in NATURE GENETICS
  • 1999-09-30. Divergent Ewing's sarcoma EWS/ETS fusions confer a common tumorigenic phenotype on NIH3T3 cells in ONCOGENE
  • 2008-05-28. Induced ncRNAs allosterically modify RNA-binding proteins in cis to inhibit transcription in NATURE
  • 2002-12. Radiation-Induced Changes of Telomerase Activity in a Human Ewing Xenograft Tumor in STRAHLENTHERAPIE UND ONKOLOGIE
  • 2009-01-19. O-GlcNAcylation is involved in the transcriptional activity of EWS-FLI1 in Ewing's sarcoma in ONCOGENE
  • 2007-10-22. Array CGH and gene-expression profiling reveals distinct genomic instability patterns associated with DNA repair and cell-cycle checkpoint pathways in Ewing's sarcoma in ONCOGENE
  • 2001-11. The ETS-domain transcription factor family in NATURE REVIEWS MOLECULAR CELL BIOLOGY
  • 2006-04-20. Genome-wide profiling of PRC1 and PRC2 Polycomb chromatin binding in Drosophila melanogaster in NATURE GENETICS
  • 1998-08-06. Oncogenic EWS-Fli1 interacts with hsRPB7, a subunit of human RNA polymerase II in ONCOGENE
  • 2009-06-02. Stable interference of EWS–FLI1 in an Ewing sarcoma cell line impairs IGF-1/IGF-1R signalling and reveals TOPK as a new target in BRITISH JOURNAL OF CANCER
  • 2009-08-31. GSTM4 is a microsatellite-containing EWS/FLI target involved in Ewing's sarcoma oncogenesis and therapeutic resistance in ONCOGENE
  • 1997-10-30. Among genes involved in the RB dependent cell cycle regulatory cascade, the p16 tumor suppressor gene is frequently lost in the Ewing family of tumors in ONCOGENE
  • 2007-12-17. The EWS/FLI1 oncogenic transcription factor deregulates GLI1 in ONCOGENE
  • 1997-05. Recurrent gains of 1q, 8 and 12 in the Ewing family of tumours by comparative genomic hybridization in BRITISH JOURNAL OF CANCER
  • 1992-09. Gene fusion with an ETS DNA-binding domain caused by chromosome translocation in human tumours in NATURE
  • 1994-02. A second Ewing's sarcoma translocation, t(21;22), fuses the EWS gene to another ETS–family transcription factor, ERG in NATURE GENETICS
  • 1992-04. Type I and type II insulin-like growth factor receptors and their function in human Ewing's sarcoma cells in JOURNAL OF CANCER RESEARCH AND CLINICAL ONCOLOGY
  • 2007-05-29. Chromosome 9p21 gene copy number and prognostic significance of p16 in ESFT in BRITISH JOURNAL OF CANCER
  • 1997-12. EWS/FLI1-induced manic fringe renders NIH 3T3 cells tumorigenic in NATURE GENETICS
  • 2009-07-05. A small molecule blocking oncogenic protein EWS-FLI1 interaction with RNA helicase A inhibits growth of Ewing's sarcoma in NATURE MEDICINE
  • 2000-06. Ewing’s sarcoma in CURRENT TREATMENT OPTIONS IN ONCOLOGY
  • 2001-10-11. Loss of p16 pathways stabilizes EWS/FLI1 expression and complements EWS/FLI1 mediated transformation in ONCOGENE
  • 1997-03-13. A new member of the ETS family fused to EWS in Ewing tumors in ONCOGENE
  • 2007-11-25. Isolation and directed differentiation of neural crest stem cells derived from human embryonic stem cells in NATURE BIOTECHNOLOGY
  • 2002-07-01. The phosphatidylinositol 3-Kinase–AKT pathway in human cancer in NATURE REVIEWS CANCER
  • Identifiers

    URI

    http://scigraph.springernature.com/pub.10.1038/onc.2010.205

    DOI

    http://dx.doi.org/10.1038/onc.2010.205

    DIMENSIONS

    https://app.dimensions.ai/details/publication/pub.1050872467

    PUBMED

    https://www.ncbi.nlm.nih.gov/pubmed/20543858


    Indexing Status Check whether this publication has been indexed by Scopus and Web Of Science using the SN Indexing Status Tool
    Incoming Citations Browse incoming citations for this publication using opencitations.net

    JSON-LD is the canonical representation for SciGraph data.

    TIP: You can open this SciGraph record using an external JSON-LD service: JSON-LD Playground Google SDTT

    [
      {
        "@context": "https://springernature.github.io/scigraph/jsonld/sgcontext.json", 
        "about": [
          {
            "id": "http://purl.org/au-research/vocabulary/anzsrc-for/2008/11", 
            "inDefinedTermSet": "http://purl.org/au-research/vocabulary/anzsrc-for/2008/", 
            "name": "Medical and Health Sciences", 
            "type": "DefinedTerm"
          }, 
          {
            "id": "http://purl.org/au-research/vocabulary/anzsrc-for/2008/1112", 
            "inDefinedTermSet": "http://purl.org/au-research/vocabulary/anzsrc-for/2008/", 
            "name": "Oncology and Carcinogenesis", 
            "type": "DefinedTerm"
          }, 
          {
            "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
            "name": "Animals", 
            "type": "DefinedTerm"
          }, 
          {
            "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
            "name": "Cell Transformation, Neoplastic", 
            "type": "DefinedTerm"
          }, 
          {
            "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
            "name": "Gene Dosage", 
            "type": "DefinedTerm"
          }, 
          {
            "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
            "name": "Humans", 
            "type": "DefinedTerm"
          }, 
          {
            "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
            "name": "Mutation", 
            "type": "DefinedTerm"
          }, 
          {
            "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
            "name": "RNA-Binding Protein EWS", 
            "type": "DefinedTerm"
          }, 
          {
            "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
            "name": "Sarcoma, Ewing", 
            "type": "DefinedTerm"
          }, 
          {
            "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
            "name": "Transcription, Genetic", 
            "type": "DefinedTerm"
          }
        ], 
        "author": [
          {
            "affiliation": {
              "alternateName": "Department of Oncological Sciences and Center for Children's Cancer Research, Huntsman Cancer Institute, Salt Lake City, UT, USA", 
              "id": "http://www.grid.ac/institutes/grid.479969.c", 
              "name": [
                "Department of Oncological Sciences and Center for Children's Cancer Research, Huntsman Cancer Institute, Salt Lake City, UT, USA"
              ], 
              "type": "Organization"
            }, 
            "familyName": "Toomey", 
            "givenName": "E C", 
            "id": "sg:person.0745625531.55", 
            "sameAs": [
              "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.0745625531.55"
            ], 
            "type": "Person"
          }, 
          {
            "affiliation": {
              "alternateName": "Division of Pediatric Hematology/Oncology, University of Utah School of Medicine, Salt Lake City, UT, USA", 
              "id": "http://www.grid.ac/institutes/grid.223827.e", 
              "name": [
                "Department of Oncological Sciences and Center for Children's Cancer Research, Huntsman Cancer Institute, Salt Lake City, UT, USA", 
                "Division of Pediatric Hematology/Oncology, University of Utah School of Medicine, Salt Lake City, UT, USA"
              ], 
              "type": "Organization"
            }, 
            "familyName": "Schiffman", 
            "givenName": "J D", 
            "id": "sg:person.01216376523.70", 
            "sameAs": [
              "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01216376523.70"
            ], 
            "type": "Person"
          }, 
          {
            "affiliation": {
              "alternateName": "Division of Pediatric Hematology/Oncology, University of Utah School of Medicine, Salt Lake City, UT, USA", 
              "id": "http://www.grid.ac/institutes/grid.223827.e", 
              "name": [
                "Department of Oncological Sciences and Center for Children's Cancer Research, Huntsman Cancer Institute, Salt Lake City, UT, USA", 
                "Division of Pediatric Hematology/Oncology, University of Utah School of Medicine, Salt Lake City, UT, USA"
              ], 
              "type": "Organization"
            }, 
            "familyName": "Lessnick", 
            "givenName": "S L", 
            "id": "sg:person.01345530760.42", 
            "sameAs": [
              "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01345530760.42"
            ], 
            "type": "Person"
          }
        ], 
        "citation": [
          {
            "id": "sg:pub.10.1007/s11864-000-0036-7", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1040537163", 
              "https://doi.org/10.1007/s11864-000-0036-7"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1038/ng1792", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1037918885", 
              "https://doi.org/10.1038/ng1792"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1038/bjc.1997.242", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1008000905", 
              "https://doi.org/10.1038/bjc.1997.242"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1038/sj.bjc.6605104", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1010283261", 
              "https://doi.org/10.1038/sj.bjc.6605104"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1038/sj.onc.1201964", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1005008704", 
              "https://doi.org/10.1038/sj.onc.1201964"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1038/13854", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1041243219", 
              "https://doi.org/10.1038/13854"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1038/359162a0", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1008302153", 
              "https://doi.org/10.1038/359162a0"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1038/sj.onc.1204268", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1019375730", 
              "https://doi.org/10.1038/sj.onc.1204268"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1038/sj.onc.1202928", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1046466161", 
              "https://doi.org/10.1038/sj.onc.1202928"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1038/onc.2008.484", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1031191918", 
              "https://doi.org/10.1038/onc.2008.484"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1007/bf01208615", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1036396171", 
              "https://doi.org/10.1007/bf01208615"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1186/1471-2407-9-17", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1001912147", 
              "https://doi.org/10.1186/1471-2407-9-17"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1038/nm.1983", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1021241680", 
              "https://doi.org/10.1038/nm.1983"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1038/nrc839", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1014023118", 
              "https://doi.org/10.1038/nrc839"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1038/nature06992", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1032941966", 
              "https://doi.org/10.1038/nature06992"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1038/nbt1365", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1039916118", 
              "https://doi.org/10.1038/nbt1365"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1038/sj.onc.1201397", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1004676244", 
              "https://doi.org/10.1038/sj.onc.1201397"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1038/sj.bjc.6600332", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1048032216", 
              "https://doi.org/10.1038/sj.bjc.6600332"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1038/sj.onc.1210845", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1025623018", 
              "https://doi.org/10.1038/sj.onc.1210845"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1038/sj.onc.1200933", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1019736256", 
              "https://doi.org/10.1038/sj.onc.1200933"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1038/onc.2009.262", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1029181355", 
              "https://doi.org/10.1038/onc.2009.262"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1007/s00066-002-0992-x", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1034091831", 
              "https://doi.org/10.1007/s00066-002-0992-x"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1038/sj.bjc.6603819", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1038603468", 
              "https://doi.org/10.1038/sj.bjc.6603819"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1038/sj.onc.1210991", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1029345731", 
              "https://doi.org/10.1038/sj.onc.1210991"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1038/35099076", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1022308098", 
              "https://doi.org/10.1038/35099076"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1038/sj.onc.1204875", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1032408374", 
              "https://doi.org/10.1038/sj.onc.1204875"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1038/sj.cgt.7700442", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1032510451", 
              "https://doi.org/10.1038/sj.cgt.7700442"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1038/ng1297-495", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1020405463", 
              "https://doi.org/10.1038/ng1297-495"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1038/ng0294-146", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1012944240", 
              "https://doi.org/10.1038/ng0294-146"
            ], 
            "type": "CreativeWork"
          }
        ], 
        "datePublished": "2010-06-14", 
        "datePublishedReg": "2010-06-14", 
        "description": "Tumor development is a complex process resulting from interplay between mutations in oncogenes and tumor suppressors, host susceptibility factors, and cellular context. Great advances have been made by studying rare tumors with unique clinical, genetic, or molecular features. Ewing's sarcoma serves as an excellent paradigm for understanding tumorigenesis because it exhibits some very useful and important characteristics. For example, nearly all cases of Ewing's sarcoma contain the (11;22)(q24;q12) chromosomal translocation that encodes the EWS/FLI oncoprotein. Besides the t(11;22), however, many cases have otherwise simple karyotypes with no other demonstrable abnormalities. Furthermore, it seems that an underlying genetic susceptibility to Ewing's sarcoma, if it exists, must be rare. These two features suggest that EWS/FLI is the primary mutation that drives the development of this tumor. Finally, Ewing's sarcoma is an aggressive tumor that requires aggressive treatment. Thus, improved understanding of the pathogenesis of this tumor will not only be of academic interest, but may also lead to new therapeutic approaches for individuals afflicted with this disease. The purpose of this review is to highlight recent advances in understanding the molecular pathogenesis of Ewing's sarcoma, while considering the questions surrounding this disease that still remain and how this knowledge may be applied to developing new treatments for patients with this highly aggressive disease.", 
        "genre": "article", 
        "id": "sg:pub.10.1038/onc.2010.205", 
        "inLanguage": "en", 
        "isAccessibleForFree": true, 
        "isFundedItemOf": [
          {
            "id": "sg:grant.2438849", 
            "type": "MonetaryGrant"
          }, 
          {
            "id": "sg:grant.2480504", 
            "type": "MonetaryGrant"
          }, 
          {
            "id": "sg:grant.2605244", 
            "type": "MonetaryGrant"
          }
        ], 
        "isPartOf": [
          {
            "id": "sg:journal.1097543", 
            "issn": [
              "0950-9232", 
              "1476-5594"
            ], 
            "name": "Oncogene", 
            "publisher": "Springer Nature", 
            "type": "Periodical"
          }, 
          {
            "issueNumber": "32", 
            "type": "PublicationIssue"
          }, 
          {
            "type": "PublicationVolume", 
            "volumeNumber": "29"
          }
        ], 
        "keywords": [
          "Ewing's sarcoma", 
          "molecular pathogenesis", 
          "new therapeutic approaches", 
          "host susceptibility factors", 
          "aggressive treatment", 
          "demonstrable abnormality", 
          "aggressive disease", 
          "rare tumor", 
          "aggressive tumors", 
          "therapeutic approaches", 
          "sarcoma", 
          "new treatments", 
          "genetic susceptibility", 
          "tumors", 
          "susceptibility factors", 
          "tumor development", 
          "pathogenesis", 
          "simple karyotypes", 
          "EWS/FLI", 
          "disease", 
          "primary mutations", 
          "chromosomal translocations", 
          "molecular features", 
          "tumor suppressor", 
          "treatment", 
          "recent advances", 
          "patients", 
          "mutations", 
          "abnormalities", 
          "great advances", 
          "tumorigenesis", 
          "excellent paradigm", 
          "cases", 
          "oncoprotein", 
          "oncogene", 
          "cellular context", 
          "advances", 
          "review", 
          "suppressor", 
          "FLI", 
          "karyotype", 
          "susceptibility", 
          "individuals", 
          "translocation", 
          "factors", 
          "development", 
          "complex process", 
          "features", 
          "purpose", 
          "knowledge", 
          "understanding", 
          "characteristics", 
          "questions", 
          "interplay", 
          "paradigm", 
          "important characteristics", 
          "interest", 
          "approach", 
          "context", 
          "academic interest", 
          "process", 
          "example"
        ], 
        "name": "Recent advances in the molecular pathogenesis of Ewing's sarcoma", 
        "pagination": "4504-4516", 
        "productId": [
          {
            "name": "dimensions_id", 
            "type": "PropertyValue", 
            "value": [
              "pub.1050872467"
            ]
          }, 
          {
            "name": "doi", 
            "type": "PropertyValue", 
            "value": [
              "10.1038/onc.2010.205"
            ]
          }, 
          {
            "name": "pubmed_id", 
            "type": "PropertyValue", 
            "value": [
              "20543858"
            ]
          }
        ], 
        "sameAs": [
          "https://doi.org/10.1038/onc.2010.205", 
          "https://app.dimensions.ai/details/publication/pub.1050872467"
        ], 
        "sdDataset": "articles", 
        "sdDatePublished": "2022-06-01T22:07", 
        "sdLicense": "https://scigraph.springernature.com/explorer/license/", 
        "sdPublisher": {
          "name": "Springer Nature - SN SciGraph project", 
          "type": "Organization"
        }, 
        "sdSource": "s3://com-springernature-scigraph/baseset/20220601/entities/gbq_results/article/article_518.jsonl", 
        "type": "ScholarlyArticle", 
        "url": "https://doi.org/10.1038/onc.2010.205"
      }
    ]
     

    Download the RDF metadata as:  json-ld nt turtle xml License info

    HOW TO GET THIS DATA PROGRAMMATICALLY:

    JSON-LD is a popular format for linked data which is fully compatible with JSON.

    curl -H 'Accept: application/ld+json' 'https://scigraph.springernature.com/pub.10.1038/onc.2010.205'

    N-Triples is a line-based linked data format ideal for batch operations.

    curl -H 'Accept: application/n-triples' 'https://scigraph.springernature.com/pub.10.1038/onc.2010.205'

    Turtle is a human-readable linked data format.

    curl -H 'Accept: text/turtle' 'https://scigraph.springernature.com/pub.10.1038/onc.2010.205'

    RDF/XML is a standard XML format for linked data.

    curl -H 'Accept: application/rdf+xml' 'https://scigraph.springernature.com/pub.10.1038/onc.2010.205'


     

    This table displays all metadata directly associated to this object as RDF triples.

    296 TRIPLES      22 PREDICATES      125 URIs      88 LITERALS      15 BLANK NODES

    Subject Predicate Object
    1 sg:pub.10.1038/onc.2010.205 schema:about N14e4179f256c48919df73a504842f015
    2 N2d5048a743bc4e868303db39afa6dca7
    3 N40d822c23a344be2858a4a5f69674906
    4 N58c3733aa3364ba3b2d5f1cc2c34ec0a
    5 Na3c41ab026c4408286cde1351559c4e7
    6 Nb059af324d4243e0a21657932c889b26
    7 Nc8917d7ae36a4f48a0ad8d95c4962cda
    8 Ncbadba93ebe64902a95a7f4912a645b3
    9 anzsrc-for:11
    10 anzsrc-for:1112
    11 schema:author N0157e3ed7d7e40bbadbb0466c3af40b4
    12 schema:citation sg:pub.10.1007/bf01208615
    13 sg:pub.10.1007/s00066-002-0992-x
    14 sg:pub.10.1007/s11864-000-0036-7
    15 sg:pub.10.1038/13854
    16 sg:pub.10.1038/35099076
    17 sg:pub.10.1038/359162a0
    18 sg:pub.10.1038/bjc.1997.242
    19 sg:pub.10.1038/nature06992
    20 sg:pub.10.1038/nbt1365
    21 sg:pub.10.1038/ng0294-146
    22 sg:pub.10.1038/ng1297-495
    23 sg:pub.10.1038/ng1792
    24 sg:pub.10.1038/nm.1983
    25 sg:pub.10.1038/nrc839
    26 sg:pub.10.1038/onc.2008.484
    27 sg:pub.10.1038/onc.2009.262
    28 sg:pub.10.1038/sj.bjc.6600332
    29 sg:pub.10.1038/sj.bjc.6603819
    30 sg:pub.10.1038/sj.bjc.6605104
    31 sg:pub.10.1038/sj.cgt.7700442
    32 sg:pub.10.1038/sj.onc.1200933
    33 sg:pub.10.1038/sj.onc.1201397
    34 sg:pub.10.1038/sj.onc.1201964
    35 sg:pub.10.1038/sj.onc.1202928
    36 sg:pub.10.1038/sj.onc.1204268
    37 sg:pub.10.1038/sj.onc.1204875
    38 sg:pub.10.1038/sj.onc.1210845
    39 sg:pub.10.1038/sj.onc.1210991
    40 sg:pub.10.1186/1471-2407-9-17
    41 schema:datePublished 2010-06-14
    42 schema:datePublishedReg 2010-06-14
    43 schema:description Tumor development is a complex process resulting from interplay between mutations in oncogenes and tumor suppressors, host susceptibility factors, and cellular context. Great advances have been made by studying rare tumors with unique clinical, genetic, or molecular features. Ewing's sarcoma serves as an excellent paradigm for understanding tumorigenesis because it exhibits some very useful and important characteristics. For example, nearly all cases of Ewing's sarcoma contain the (11;22)(q24;q12) chromosomal translocation that encodes the EWS/FLI oncoprotein. Besides the t(11;22), however, many cases have otherwise simple karyotypes with no other demonstrable abnormalities. Furthermore, it seems that an underlying genetic susceptibility to Ewing's sarcoma, if it exists, must be rare. These two features suggest that EWS/FLI is the primary mutation that drives the development of this tumor. Finally, Ewing's sarcoma is an aggressive tumor that requires aggressive treatment. Thus, improved understanding of the pathogenesis of this tumor will not only be of academic interest, but may also lead to new therapeutic approaches for individuals afflicted with this disease. The purpose of this review is to highlight recent advances in understanding the molecular pathogenesis of Ewing's sarcoma, while considering the questions surrounding this disease that still remain and how this knowledge may be applied to developing new treatments for patients with this highly aggressive disease.
    44 schema:genre article
    45 schema:inLanguage en
    46 schema:isAccessibleForFree true
    47 schema:isPartOf N1d022718038040d08f4cd44a2299ee42
    48 N45b7d2e926a34d649d073dd7c14db8c4
    49 sg:journal.1097543
    50 schema:keywords EWS/FLI
    51 Ewing's sarcoma
    52 FLI
    53 abnormalities
    54 academic interest
    55 advances
    56 aggressive disease
    57 aggressive treatment
    58 aggressive tumors
    59 approach
    60 cases
    61 cellular context
    62 characteristics
    63 chromosomal translocations
    64 complex process
    65 context
    66 demonstrable abnormality
    67 development
    68 disease
    69 example
    70 excellent paradigm
    71 factors
    72 features
    73 genetic susceptibility
    74 great advances
    75 host susceptibility factors
    76 important characteristics
    77 individuals
    78 interest
    79 interplay
    80 karyotype
    81 knowledge
    82 molecular features
    83 molecular pathogenesis
    84 mutations
    85 new therapeutic approaches
    86 new treatments
    87 oncogene
    88 oncoprotein
    89 paradigm
    90 pathogenesis
    91 patients
    92 primary mutations
    93 process
    94 purpose
    95 questions
    96 rare tumor
    97 recent advances
    98 review
    99 sarcoma
    100 simple karyotypes
    101 suppressor
    102 susceptibility
    103 susceptibility factors
    104 therapeutic approaches
    105 translocation
    106 treatment
    107 tumor development
    108 tumor suppressor
    109 tumorigenesis
    110 tumors
    111 understanding
    112 schema:name Recent advances in the molecular pathogenesis of Ewing's sarcoma
    113 schema:pagination 4504-4516
    114 schema:productId N609942317e11476ebc00402b1977af50
    115 N6519fc3ddd22474c933fc9cebbad45a4
    116 Nb87168dfde9a4583b0cd02e271198f5e
    117 schema:sameAs https://app.dimensions.ai/details/publication/pub.1050872467
    118 https://doi.org/10.1038/onc.2010.205
    119 schema:sdDatePublished 2022-06-01T22:07
    120 schema:sdLicense https://scigraph.springernature.com/explorer/license/
    121 schema:sdPublisher N028edce9109f4aaa90d4a07609e5acf6
    122 schema:url https://doi.org/10.1038/onc.2010.205
    123 sgo:license sg:explorer/license/
    124 sgo:sdDataset articles
    125 rdf:type schema:ScholarlyArticle
    126 N0157e3ed7d7e40bbadbb0466c3af40b4 rdf:first sg:person.0745625531.55
    127 rdf:rest N1f3318385db940f6960618c6ef6c8f53
    128 N028edce9109f4aaa90d4a07609e5acf6 schema:name Springer Nature - SN SciGraph project
    129 rdf:type schema:Organization
    130 N14e4179f256c48919df73a504842f015 schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
    131 schema:name Transcription, Genetic
    132 rdf:type schema:DefinedTerm
    133 N1d022718038040d08f4cd44a2299ee42 schema:issueNumber 32
    134 rdf:type schema:PublicationIssue
    135 N1f3318385db940f6960618c6ef6c8f53 rdf:first sg:person.01216376523.70
    136 rdf:rest Nf7a5caad28564fc69c54bc80fd44a746
    137 N2d5048a743bc4e868303db39afa6dca7 schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
    138 schema:name Gene Dosage
    139 rdf:type schema:DefinedTerm
    140 N40d822c23a344be2858a4a5f69674906 schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
    141 schema:name RNA-Binding Protein EWS
    142 rdf:type schema:DefinedTerm
    143 N45b7d2e926a34d649d073dd7c14db8c4 schema:volumeNumber 29
    144 rdf:type schema:PublicationVolume
    145 N58c3733aa3364ba3b2d5f1cc2c34ec0a schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
    146 schema:name Humans
    147 rdf:type schema:DefinedTerm
    148 N609942317e11476ebc00402b1977af50 schema:name pubmed_id
    149 schema:value 20543858
    150 rdf:type schema:PropertyValue
    151 N6519fc3ddd22474c933fc9cebbad45a4 schema:name doi
    152 schema:value 10.1038/onc.2010.205
    153 rdf:type schema:PropertyValue
    154 Na3c41ab026c4408286cde1351559c4e7 schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
    155 schema:name Mutation
    156 rdf:type schema:DefinedTerm
    157 Nb059af324d4243e0a21657932c889b26 schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
    158 schema:name Animals
    159 rdf:type schema:DefinedTerm
    160 Nb87168dfde9a4583b0cd02e271198f5e schema:name dimensions_id
    161 schema:value pub.1050872467
    162 rdf:type schema:PropertyValue
    163 Nc8917d7ae36a4f48a0ad8d95c4962cda schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
    164 schema:name Cell Transformation, Neoplastic
    165 rdf:type schema:DefinedTerm
    166 Ncbadba93ebe64902a95a7f4912a645b3 schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
    167 schema:name Sarcoma, Ewing
    168 rdf:type schema:DefinedTerm
    169 Nf7a5caad28564fc69c54bc80fd44a746 rdf:first sg:person.01345530760.42
    170 rdf:rest rdf:nil
    171 anzsrc-for:11 schema:inDefinedTermSet anzsrc-for:
    172 schema:name Medical and Health Sciences
    173 rdf:type schema:DefinedTerm
    174 anzsrc-for:1112 schema:inDefinedTermSet anzsrc-for:
    175 schema:name Oncology and Carcinogenesis
    176 rdf:type schema:DefinedTerm
    177 sg:grant.2438849 http://pending.schema.org/fundedItem sg:pub.10.1038/onc.2010.205
    178 rdf:type schema:MonetaryGrant
    179 sg:grant.2480504 http://pending.schema.org/fundedItem sg:pub.10.1038/onc.2010.205
    180 rdf:type schema:MonetaryGrant
    181 sg:grant.2605244 http://pending.schema.org/fundedItem sg:pub.10.1038/onc.2010.205
    182 rdf:type schema:MonetaryGrant
    183 sg:journal.1097543 schema:issn 0950-9232
    184 1476-5594
    185 schema:name Oncogene
    186 schema:publisher Springer Nature
    187 rdf:type schema:Periodical
    188 sg:person.01216376523.70 schema:affiliation grid-institutes:grid.223827.e
    189 schema:familyName Schiffman
    190 schema:givenName J D
    191 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01216376523.70
    192 rdf:type schema:Person
    193 sg:person.01345530760.42 schema:affiliation grid-institutes:grid.223827.e
    194 schema:familyName Lessnick
    195 schema:givenName S L
    196 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01345530760.42
    197 rdf:type schema:Person
    198 sg:person.0745625531.55 schema:affiliation grid-institutes:grid.479969.c
    199 schema:familyName Toomey
    200 schema:givenName E C
    201 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.0745625531.55
    202 rdf:type schema:Person
    203 sg:pub.10.1007/bf01208615 schema:sameAs https://app.dimensions.ai/details/publication/pub.1036396171
    204 https://doi.org/10.1007/bf01208615
    205 rdf:type schema:CreativeWork
    206 sg:pub.10.1007/s00066-002-0992-x schema:sameAs https://app.dimensions.ai/details/publication/pub.1034091831
    207 https://doi.org/10.1007/s00066-002-0992-x
    208 rdf:type schema:CreativeWork
    209 sg:pub.10.1007/s11864-000-0036-7 schema:sameAs https://app.dimensions.ai/details/publication/pub.1040537163
    210 https://doi.org/10.1007/s11864-000-0036-7
    211 rdf:type schema:CreativeWork
    212 sg:pub.10.1038/13854 schema:sameAs https://app.dimensions.ai/details/publication/pub.1041243219
    213 https://doi.org/10.1038/13854
    214 rdf:type schema:CreativeWork
    215 sg:pub.10.1038/35099076 schema:sameAs https://app.dimensions.ai/details/publication/pub.1022308098
    216 https://doi.org/10.1038/35099076
    217 rdf:type schema:CreativeWork
    218 sg:pub.10.1038/359162a0 schema:sameAs https://app.dimensions.ai/details/publication/pub.1008302153
    219 https://doi.org/10.1038/359162a0
    220 rdf:type schema:CreativeWork
    221 sg:pub.10.1038/bjc.1997.242 schema:sameAs https://app.dimensions.ai/details/publication/pub.1008000905
    222 https://doi.org/10.1038/bjc.1997.242
    223 rdf:type schema:CreativeWork
    224 sg:pub.10.1038/nature06992 schema:sameAs https://app.dimensions.ai/details/publication/pub.1032941966
    225 https://doi.org/10.1038/nature06992
    226 rdf:type schema:CreativeWork
    227 sg:pub.10.1038/nbt1365 schema:sameAs https://app.dimensions.ai/details/publication/pub.1039916118
    228 https://doi.org/10.1038/nbt1365
    229 rdf:type schema:CreativeWork
    230 sg:pub.10.1038/ng0294-146 schema:sameAs https://app.dimensions.ai/details/publication/pub.1012944240
    231 https://doi.org/10.1038/ng0294-146
    232 rdf:type schema:CreativeWork
    233 sg:pub.10.1038/ng1297-495 schema:sameAs https://app.dimensions.ai/details/publication/pub.1020405463
    234 https://doi.org/10.1038/ng1297-495
    235 rdf:type schema:CreativeWork
    236 sg:pub.10.1038/ng1792 schema:sameAs https://app.dimensions.ai/details/publication/pub.1037918885
    237 https://doi.org/10.1038/ng1792
    238 rdf:type schema:CreativeWork
    239 sg:pub.10.1038/nm.1983 schema:sameAs https://app.dimensions.ai/details/publication/pub.1021241680
    240 https://doi.org/10.1038/nm.1983
    241 rdf:type schema:CreativeWork
    242 sg:pub.10.1038/nrc839 schema:sameAs https://app.dimensions.ai/details/publication/pub.1014023118
    243 https://doi.org/10.1038/nrc839
    244 rdf:type schema:CreativeWork
    245 sg:pub.10.1038/onc.2008.484 schema:sameAs https://app.dimensions.ai/details/publication/pub.1031191918
    246 https://doi.org/10.1038/onc.2008.484
    247 rdf:type schema:CreativeWork
    248 sg:pub.10.1038/onc.2009.262 schema:sameAs https://app.dimensions.ai/details/publication/pub.1029181355
    249 https://doi.org/10.1038/onc.2009.262
    250 rdf:type schema:CreativeWork
    251 sg:pub.10.1038/sj.bjc.6600332 schema:sameAs https://app.dimensions.ai/details/publication/pub.1048032216
    252 https://doi.org/10.1038/sj.bjc.6600332
    253 rdf:type schema:CreativeWork
    254 sg:pub.10.1038/sj.bjc.6603819 schema:sameAs https://app.dimensions.ai/details/publication/pub.1038603468
    255 https://doi.org/10.1038/sj.bjc.6603819
    256 rdf:type schema:CreativeWork
    257 sg:pub.10.1038/sj.bjc.6605104 schema:sameAs https://app.dimensions.ai/details/publication/pub.1010283261
    258 https://doi.org/10.1038/sj.bjc.6605104
    259 rdf:type schema:CreativeWork
    260 sg:pub.10.1038/sj.cgt.7700442 schema:sameAs https://app.dimensions.ai/details/publication/pub.1032510451
    261 https://doi.org/10.1038/sj.cgt.7700442
    262 rdf:type schema:CreativeWork
    263 sg:pub.10.1038/sj.onc.1200933 schema:sameAs https://app.dimensions.ai/details/publication/pub.1019736256
    264 https://doi.org/10.1038/sj.onc.1200933
    265 rdf:type schema:CreativeWork
    266 sg:pub.10.1038/sj.onc.1201397 schema:sameAs https://app.dimensions.ai/details/publication/pub.1004676244
    267 https://doi.org/10.1038/sj.onc.1201397
    268 rdf:type schema:CreativeWork
    269 sg:pub.10.1038/sj.onc.1201964 schema:sameAs https://app.dimensions.ai/details/publication/pub.1005008704
    270 https://doi.org/10.1038/sj.onc.1201964
    271 rdf:type schema:CreativeWork
    272 sg:pub.10.1038/sj.onc.1202928 schema:sameAs https://app.dimensions.ai/details/publication/pub.1046466161
    273 https://doi.org/10.1038/sj.onc.1202928
    274 rdf:type schema:CreativeWork
    275 sg:pub.10.1038/sj.onc.1204268 schema:sameAs https://app.dimensions.ai/details/publication/pub.1019375730
    276 https://doi.org/10.1038/sj.onc.1204268
    277 rdf:type schema:CreativeWork
    278 sg:pub.10.1038/sj.onc.1204875 schema:sameAs https://app.dimensions.ai/details/publication/pub.1032408374
    279 https://doi.org/10.1038/sj.onc.1204875
    280 rdf:type schema:CreativeWork
    281 sg:pub.10.1038/sj.onc.1210845 schema:sameAs https://app.dimensions.ai/details/publication/pub.1025623018
    282 https://doi.org/10.1038/sj.onc.1210845
    283 rdf:type schema:CreativeWork
    284 sg:pub.10.1038/sj.onc.1210991 schema:sameAs https://app.dimensions.ai/details/publication/pub.1029345731
    285 https://doi.org/10.1038/sj.onc.1210991
    286 rdf:type schema:CreativeWork
    287 sg:pub.10.1186/1471-2407-9-17 schema:sameAs https://app.dimensions.ai/details/publication/pub.1001912147
    288 https://doi.org/10.1186/1471-2407-9-17
    289 rdf:type schema:CreativeWork
    290 grid-institutes:grid.223827.e schema:alternateName Division of Pediatric Hematology/Oncology, University of Utah School of Medicine, Salt Lake City, UT, USA
    291 schema:name Department of Oncological Sciences and Center for Children's Cancer Research, Huntsman Cancer Institute, Salt Lake City, UT, USA
    292 Division of Pediatric Hematology/Oncology, University of Utah School of Medicine, Salt Lake City, UT, USA
    293 rdf:type schema:Organization
    294 grid-institutes:grid.479969.c schema:alternateName Department of Oncological Sciences and Center for Children's Cancer Research, Huntsman Cancer Institute, Salt Lake City, UT, USA
    295 schema:name Department of Oncological Sciences and Center for Children's Cancer Research, Huntsman Cancer Institute, Salt Lake City, UT, USA
    296 rdf:type schema:Organization
     




    Preview window. Press ESC to close (or click here)


    ...