Ectopic expression of wild-type FGFR3 cooperates with MYC to accelerate development of B-cell lineage neoplasms

Leukemia. 2010 Jun;24(6):1171-8. doi: 10.1038/leu.2010.50. Epub 2010 Apr 15.

Abstract

The t(4;14) translocation in multiple myeloma (MM) simultaneously dysregulates two apparent oncogenes: fibroblast growth factor receptor 3 (FGFR3) controlled by the 3' immunoglobulin heavy chain enhancer on der(14) and MMSET controlled by the intronic Emu enhancer on der(4). Although all MM tumors and cell lines with a t(4;14) translocation have dysregulated MMSET, about 25% do not express FGFR3. Therefore, the function of dysregulated wild-type (WT) FGFR3 in the pathogenesis of MM remains unclear. We developed a murine transgenic (TG) model in which WT FGFR3 is overexpressed in B lymphoid cells. Although high levels of FGFR3 resulted in lymphoid hyperplasia in about one-third of older mice, no increase in tumorigenesis was observed. However, double TG FGFR3/Myc mice develop mature B lymphoma tumors that occur with a higher penetrance and shorter latency than in single TG Myc mice (P=0.006). We conclude that expression of high levels of WT FGFR3 can be oncogenic and cooperate with MYC to generate B lymphoid tumors. This suggests that dysregulated FGFR3 expression is likely to be essential at least for the early stages of pathogenesis of MM tumors that have a t(4;14) translocation.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, N.I.H., Intramural

MeSH terms

  • Animals
  • Biomarkers, Tumor / genetics
  • Biomarkers, Tumor / metabolism
  • Blotting, Southern
  • Blotting, Western
  • Female
  • Gene Expression Profiling
  • Genes, Immunoglobulin
  • Humans
  • Immunoenzyme Techniques
  • Immunophenotyping
  • Immunoprecipitation
  • Lymphoma, B-Cell / etiology*
  • Lymphoma, B-Cell / pathology
  • Male
  • Mice
  • Mice, Transgenic
  • Multiple Myeloma / etiology*
  • Multiple Myeloma / pathology
  • Oligonucleotide Array Sequence Analysis
  • Proto-Oncogene Proteins c-myc / physiology*
  • RNA, Messenger / genetics
  • RNA, Messenger / metabolism
  • Receptor, Fibroblast Growth Factor, Type 3 / physiology*
  • Reverse Transcriptase Polymerase Chain Reaction

Substances

  • Biomarkers, Tumor
  • Myc protein, mouse
  • Proto-Oncogene Proteins c-myc
  • RNA, Messenger
  • FGFR3 protein, human
  • Receptor, Fibroblast Growth Factor, Type 3