FREDERIC BARR

Summary

Affiliation: University of Pennsylvania
Country: USA

Publications

  1. ncbi request reprint Translocations, cancer and the puzzle of specificity
    F G Barr
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia 19104 6082, USA
    Nat Genet 19:121-4. 1998
  2. ncbi request reprint Predominant expression of alternative PAX3 and PAX7 forms in myogenic and neural tumor cell lines
    F G Barr
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia 19104 6082, USA
    Cancer Res 59:5443-8. 1999
  3. ncbi request reprint Gene fusions involving PAX and FOX family members in alveolar rhabdomyosarcoma
    F G Barr
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, 36th Street and Hamilton Walk, Philadelphia, Pennsylvania, PA 19104 6082, USA
    Oncogene 20:5736-46. 2001
  4. pmc Genomic and clinical analyses of 2p24 and 12q13-q14 amplification in alveolar rhabdomyosarcoma: a report from the Children's Oncology Group
    Frederic G Barr
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Genes Chromosomes Cancer 48:661-72. 2009
  5. pmc Molecular diagnosis of ewing family tumors: too many fusions... ?
    Frederic G Barr
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, 505C Stellar Chance Laboratories, 422 Curie Blvd, Philadelphia, PA 19104 6100, USA
    J Mol Diagn 9:437-40. 2007
  6. pmc Examination of gene fusion status in archival samples of alveolar rhabdomyosarcoma entered on the Intergroup Rhabdomyosarcoma Study-III trial: a report from the Children's Oncology Group
    Frederic G Barr
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA 19104 6100, USA
    J Mol Diagn 8:202-8. 2006
  7. ncbi request reprint Genetic heterogeneity in the alveolar rhabdomyosarcoma subset without typical gene fusions
    Frederic G Barr
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
    Cancer Res 62:4704-10. 2002
  8. doi request reprint Identification of PAX3-FKHR-regulated genes differentially expressed between alveolar and embryonal rhabdomyosarcoma: focus on MYCN as a biologically relevant target
    Gabriela E Mercado
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Genes Chromosomes Cancer 47:510-20. 2008
  9. pmc High expression of the PAX3-FKHR oncoprotein is required to promote tumorigenesis of human myoblasts
    Shujuan J Xia
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104 6082, USA
    Am J Pathol 175:2600-8. 2009
  10. ncbi request reprint Inducible short-term and stable long-term cell culture systems reveal that the PAX3-FKHR fusion oncoprotein regulates CXCR4, PAX3, and PAX7 expression
    Oana Tomescu
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA, USA
    Lab Invest 84:1060-70. 2004

Collaborators

Detail Information

Publications36

  1. ncbi request reprint Translocations, cancer and the puzzle of specificity
    F G Barr
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia 19104 6082, USA
    Nat Genet 19:121-4. 1998
    ..A dynamic relationship between the gene fusion and the cellular environment is proposed in which the environment influences the selection of oncogenic fusions and the oncogenic fusion in turn influences the cellular environment...
  2. ncbi request reprint Predominant expression of alternative PAX3 and PAX7 forms in myogenic and neural tumor cell lines
    F G Barr
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia 19104 6082, USA
    Cancer Res 59:5443-8. 1999
    ..However, the findings of nearly identical COOH-terminal regions within the corresponding genes of the avian and fish genomes suggest conserved functional roles for these regions that require further investigation...
  3. ncbi request reprint Gene fusions involving PAX and FOX family members in alveolar rhabdomyosarcoma
    F G Barr
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, 36th Street and Hamilton Walk, Philadelphia, Pennsylvania, PA 19104 6082, USA
    Oncogene 20:5736-46. 2001
    ..This aberrant gene expression program is then hypothesized to contribute to tumorigenic behavior by impacting on the control of growth, apoptosis, differentiation and motility...
  4. pmc Genomic and clinical analyses of 2p24 and 12q13-q14 amplification in alveolar rhabdomyosarcoma: a report from the Children's Oncology Group
    Frederic G Barr
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Genes Chromosomes Cancer 48:661-72. 2009
    ....
  5. pmc Molecular diagnosis of ewing family tumors: too many fusions... ?
    Frederic G Barr
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, 505C Stellar Chance Laboratories, 422 Curie Blvd, Philadelphia, PA 19104 6100, USA
    J Mol Diagn 9:437-40. 2007
  6. pmc Examination of gene fusion status in archival samples of alveolar rhabdomyosarcoma entered on the Intergroup Rhabdomyosarcoma Study-III trial: a report from the Children's Oncology Group
    Frederic G Barr
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA 19104 6100, USA
    J Mol Diagn 8:202-8. 2006
    ....
  7. ncbi request reprint Genetic heterogeneity in the alveolar rhabdomyosarcoma subset without typical gene fusions
    Frederic G Barr
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
    Cancer Res 62:4704-10. 2002
    ....
  8. doi request reprint Identification of PAX3-FKHR-regulated genes differentially expressed between alveolar and embryonal rhabdomyosarcoma: focus on MYCN as a biologically relevant target
    Gabriela E Mercado
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Genes Chromosomes Cancer 47:510-20. 2008
    ..In conclusion, we identified a selected set of biologically relevant genes modulated by PAX3-FKHR, and demonstrated that PAX3-FKHR contributes to the expression of MYCN and in turn MYCN collaborates with PAX3-FKHR in tumorigenesis...
  9. pmc High expression of the PAX3-FKHR oncoprotein is required to promote tumorigenesis of human myoblasts
    Shujuan J Xia
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104 6082, USA
    Am J Pathol 175:2600-8. 2009
    ..These findings support the premise that high PAX3-FKHR-expressing cells are selected during tumorigenesis...
  10. ncbi request reprint Inducible short-term and stable long-term cell culture systems reveal that the PAX3-FKHR fusion oncoprotein regulates CXCR4, PAX3, and PAX7 expression
    Oana Tomescu
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA, USA
    Lab Invest 84:1060-70. 2004
    ..These findings of genes regulated by PAX3-FKHR will direct future biological and clinical investigation to important pathways contributing to ARMS tumorigenesis and progression...
  11. ncbi request reprint PDGF-A, PDGF-Rbeta, TGFbeta3 and bone morphogenic protein-4 in desmoplastic small round cell tumors with EWS-WT1 gene fusion product and their role in stromal desmoplasia: an immunohistochemical study
    Paul J Zhang
    University of Pennsylvania Medical Center, Philadelphia, PA 19147, USA
    Mod Pathol 18:382-7. 2005
    ..Other targets of the EWS-WT1 transcription factor other than PDGF-A may be directly responsible for the prominent tumor-associated desmoplasia seen in desmoplastic small round cell tumor...
  12. ncbi request reprint Molecular pathogenesis of rhabdomyosarcoma
    Shujuan J Xia
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, 36th Street and Hamilton Walk, Philadelphia, Pennsylvania 19104 6082, USA
    Cancer Biol Ther 1:97-104. 2002
    ..Therefore, despite similarities in the downstream targets of these genetic alterations, the striking cytogenetic and molecular differences between ARMS and ERMS indicate distinct molecular etiologies in these two subtypes...
  13. ncbi request reprint Immunophenotype of desmoplastic small round cell tumors as detected in cases with EWS-WT1 gene fusion product
    Paul J Zhang
    Anatomic Pathology, 6 Founders Pavilion, University of Pennsylvania Medical Center, Philadelphia, Pennsylvania 19147, USA
    Mod Pathol 16:229-35. 2003
    ..Detection of EWS-WT1 fusion becomes critical for the diagnosis when the characteristic divergent phenotype cannot be detected immunohistochemically...
  14. doi request reprint Embryonal and alveolar rhabdomyosarcoma of parameningeal sites in adults: a report of 13 cases
    Kathleen T Montone
    Department of Pathology and Laboratory Medicine, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA
    Int J Surg Pathol 17:22-30. 2009
    ..Disease can occur in the elderly. Desmin and myogenin can aid in the diagnosis but cytokeratin reactivity can be seen and care must be taken not to diagnose carcinoma...
  15. ncbi request reprint Chromosomal translocations and sarcomas
    Jeannette L Bennicelli
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
    Curr Opin Oncol 14:412-9. 2002
    ..These and other emerging models of tumorigenesis will help identify new therapeutic targets...
  16. ncbi request reprint Co-expression of alternatively spliced forms of PAX3, PAX7, PAX3-FKHR and PAX7-FKHR with distinct DNA binding and transactivation properties in rhabdomyosarcoma
    Shouying Du
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Int J Cancer 115:85-92. 2005
    ..In conclusion, these studies reveal that PAX3, PAX7 and their fusions with FKHR are each expressed in RMS tumors as a consistent mixture of functionally distinct isoforms...
  17. ncbi request reprint Fusions involving PAX and FOX genes in the molecular pathogenesis of alveolar rhabdomyosarcoma: recent advances
    Gabriela E Mercado
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Curr Mol Med 7:47-61. 2007
    ....
  18. ncbi request reprint Analysis of genetic events that modulate the oncogenic and growth suppressive activities of the PAX3-FKHR fusion oncoprotein
    Shujuan J Xia
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Lab Invest 87:318-25. 2007
    ....
  19. ncbi request reprint Chromosome translocations in sarcomas and the emergence of oncogenic transcription factors
    Shujuan J Xia
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, 505C Stellar Chance Laboratories, 422 Curie Boulevard, Philadelphia, PA 19104 6082, USA
    Eur J Cancer 41:2513-27. 2005
    ....
  20. ncbi request reprint Looking downstream of sarcoma-associated chimeric transcription factors: when is a target really a target?
    Gabriela E Mercado
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104 6082, USA
    Cancer Biol Ther 4:456-8. 2005
  21. ncbi request reprint Analysis of the transforming and growth suppressive activities of the PAX3-FKHR oncoprotein
    Shujuan J Xia
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Oncogene 23:6864-71. 2004
    ..These findings are consistent with the hypothesis that distinct expression pathways are operative in these opposing phenotypic end points...
  22. ncbi request reprint Detection and diagnostic utilization of placental alkaline phosphatase in muscular tissue and tumors with myogenic differentiation
    Jeffrey D Goldsmith
    Department of Pathology and Laboratory Medicine, University of Pennsylvania Medical Center, 3400 Spruce Street, Philadelphia, PA 19147, USA
    Am J Surg Pathol 26:1627-33. 2002
    ..In addition to its role as a germ cell marker, PLAP may also be used as a myogenic marker in the diagnosis of soft tissue tumors...
  23. ncbi request reprint CXCR4-SDF-1 signaling is active in rhabdomyosarcoma cells and regulates locomotion, chemotaxis, and adhesion
    Jolanta Libura
    Stem Cell Biology Program at the James Graham Brown Cancer Center, University of Louisville, KY 40202, USA
    Blood 100:2597-606. 2002
    ....
  24. ncbi request reprint Genomic gains and losses are similar in genetic and histologic subsets of rhabdomyosarcoma, whereas amplification predominates in embryonal with anaplasia and alveolar subtypes
    Julia A Bridge
    Department of Pathology and Microbiology, University of Nebraska Medical Center, Omaha, NE 68198, USA
    Genes Chromosomes Cancer 33:310-21. 2002
    ....
  25. ncbi request reprint Mediastinal synovial sarcoma: report of two cases with molecular genetic analysis
    Jacqueline K Trupiano
    Department of Anatomic Pathology, The Cleveland Clinic Foundation, Ohio 44195, USA
    Ann Thorac Surg 73:628-30. 2002
    ..Detection of this translocation is of paramount importance to confirm this diagnosis, particularly when this neoplasm arises in atypical locations outside the extremities...
  26. ncbi request reprint Correlation between histology and PAX/FKHR fusion status in alveolar rhabdomyosarcoma: a report from the Children's Oncology Group
    David M Parham
    Department of Pathology, University of Arkansas for Medical Sciences, Little Rock, AR, USA
    Am J Surg Pathol 31:895-901. 2007
    ..No features seemed to predict the presence of a particular fusion type. Our results suggest that histologic assessment of ARMS has limited correlation with PAX/FKHR fusion status...
  27. ncbi request reprint Desmoplastic small round cell tumor of the kidney in childhood
    Larry L Wang
    Department of Pathology, Children s Hospital Boston, Boston, MA 02115, USA
    Am J Surg Pathol 31:576-84. 2007
    ..When presenting within visceral organs, the diagnosis of DSRCT poses significant difficulties...
  28. ncbi request reprint Sclerosing rhabdomyosarcomas in children and adolescents: a clinicopathologic review of 13 cases from the Intergroup Rhabdomyosarcoma Study Group and Children's Oncology Group
    Melissa C Chiles
    Department of Pathology, Arkansas Children s Hospital and University of Arkansas for Medical Sciences, Slot 820, 800 Marshall Street, Little Rock, AR 72202, USA
    Pediatr Dev Pathol 7:583-94. 2004
    ..Sclerosing RMS may arise in children, have mixed ERMS-ARMS histology, originate from the head and neck, and lack strong myogenin staining...
  29. ncbi request reprint Identification of target genes regulated by PAX3 and PAX3-FKHR in embryogenesis and alveolar rhabdomyosarcoma
    Thomas D Barber
    National Institute on Deafness and Other Communication Disorders, National Institutes of Health, 5 Research Court, Rockville, Maryland 20850, USA
    Genomics 79:278-84. 2002
    ....
  30. ncbi request reprint Use of a novel FISH assay on paraffin-embedded tissues as an adjunct to diagnosis of alveolar rhabdomyosarcoma
    Jun Nishio
    Department of Pathology and Microbiology, University of Nebraska Medical Center, 983135 Nebraska Medical Center, Omaha, 68198 3135, USA
    Lab Invest 86:547-56. 2006
    ..The findings also suggest that FISH may be a more sensitive assay than RT-PCR in some settings, capable of revealing variant translocations...
  31. ncbi request reprint Rhabdomyosarcomas utilize developmental, myogenic growth factors for disease advantage: a report from the Children's Oncology Group
    Mary C Blandford
    Department of Pediatrics, Division of Pediatric Hematology Oncology, University of Utah, Salt Lake City, USA
    Pediatr Blood Cancer 46:329-38. 2006
    ..Unresectable or metastatic disease represents the greatest obstacle to cure for children with rhabdomyosarcoma. In this study we sought to identify gene expression signatures of advanced stage and progressive disease...
  32. ncbi request reprint Diagnostic applications of chromosomal translocations in bone and soft tissue sarcomas
    Frederic G Barr
    Expert Rev Mol Diagn 4:579-82. 2004
  33. ncbi request reprint PAX3-FKHR and PAX7-FKHR gene fusions are prognostic indicators in alveolar rhabdomyosarcoma: a report from the children's oncology group
    Poul H B Sorensen
    Department of Pathology, Children s and Women s Hospital of British Columbia, Vancouver, British Columbia, Canada
    J Clin Oncol 20:2672-9. 2002
    ..We wished to confirm the diagnostic specificity of gene fusion detection in a large cohort of RMS patients and to evaluate whether these alterations influence clinical outcome in ARMS...
  34. ncbi request reprint The impact of genetics on sarcoma diagnosis: an evolving science
    Frederic G Barr
    Clin Cancer Res 12:5256-7. 2006
  35. ncbi request reprint Impact of SYT-SSX fusion type on the clinical behavior of synovial sarcoma: a multi-institutional retrospective study of 243 patients
    Marc Ladanyi
    Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, New York 10021, USA
    Cancer Res 62:135-40. 2002
    ..In addition, the associations of SYT-SSX fusion type with patient sex and tumor epithelial differentiation point to interesting mechanistic biological differences...
  36. ncbi request reprint Sclerosing rhabdomyosarcomas in children and adolescents: a clinicopathologic review of 13 cases from the Intergroup Rhabdomyosarcoma Study Group and Children's Oncology Group
    Melissa C Chiles
    Department of Pathology and Pediatrics, Arkansas Children s Hospital and University of Arkansas for Medical Sciences, Slot 820, 800 Marshall Street, Little Rock, AR 72202, USA
    Pediatr Dev Pathol 8:141. 2005

Research Grants31

  1. IRSG STUDIES OF ALVEOLAR RHABDOMYOSARCOMA GENE FUSIONS
    FREDERIC BARR; Fiscal Year: 2005
    ..These studies will provide a definitive analysis of the occurrence and clinical significance of these genetic alterations in ARMS, and will ultimately impact on the future design of clinical protocols for the treatment of ARMS patients. ..
  2. Cancer Molecular Pathology Training Program
    FREDERIC BARR; Fiscal Year: 2006
    ....
  3. COG studies of gene amplification in rhabdomyosarcoma
    FREDERIC BARR; Fiscal Year: 2007
    ..abstract_text> ..
  4. Studies of the t(2;13) of alveolar rhabdomyosarcoma
    FREDERIC BARR; Fiscal Year: 2007
    ..abstract_text> ..
  5. STUDIES OF THE T (2;13) OF ALVEOLAR RHABDOMYOSARCOMA
    FREDERIC BARR; Fiscal Year: 2002
    ....
  6. REGULATION OF PAX3/PAX7 EXPRESSION IN RHABDOMYOSARCOMA
    FREDERIC BARR; Fiscal Year: 2001
    ....
  7. COG studies of gene amplification in rhabdomyosarcoma
    FREDERIC BARR; Fiscal Year: 2009
    ..abstract_text> ..