Constitutively activated FGFR3 mutants signal through PLCgamma-dependent and -independent pathways for hematopoietic transformation

Blood. 2005 Jul 1;106(1):328-37. doi: 10.1182/blood-2004-09-3686. Epub 2005 Mar 22.

Abstract

Ectopic expression of fibroblast growth factor receptor 3 (FGFR3) associated with t(4;14) has been implicated in the pathogenesis of human multiple myeloma. Some t(4;14) patients have activating mutations of FGFR3, of which a minority are K650E (thanatophoric dysplasia type II [TDII]). To investigate the role of autophosphorylated tyrosine residues in FGFR3 signal transduction and transformation, we characterized a series of FGFR3 TDII mutants with single or multiple Y-->F substitutions. Phenylalanine substitution of Y760, essential for phospholipase Cgamma (PLCgamma) binding and activation, significantly attenuated FGFR3 TDII-mediated PLCgamma activation, as well as transformation in Ba/F3 cells and a murine bone marrow transplant leukemia model. In contrast, single substitution of Y577, Y724, or Y770 had minimal to moderate effects on TDII-dependent transformation. Substitution of all 4 non-activation loop tyrosine residues significantly attenuated, but did not abolish, TDII transforming activity. Similar observations were obtained in the context of a constitutively activated fusion TEL-FGFR3 associated with t(4;12)(p16;p13) peripheral T-cell lymphomas. Moreover, 2 independent EmuSR-FGFR3 TDII transgenic mouse lines developed a pro-B-cell lymphoma, and PLCgamma was highly activated in primary lymphoma cells as assessed by tyrosine phosphorylation. These data indicate that engagement of multiple signaling pathways, including PLCgamma-dependent and PLCgamma-independent pathways, is required for full hematopoietic transformation by constitutively activated FGFR3 mutants.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Amino Acid Substitution
  • Animals
  • Cell Transformation, Neoplastic
  • Cells, Cultured
  • Gene Expression Regulation, Neoplastic
  • Mice
  • Mice, Transgenic
  • Multiple Myeloma / pathology
  • Multiple Myeloma / physiopathology*
  • Mutagenesis, Site-Directed
  • Oncogene Proteins, Fusion / genetics
  • Phospholipase C gamma
  • Protein-Tyrosine Kinases / genetics*
  • Protein-Tyrosine Kinases / metabolism*
  • Receptor, Fibroblast Growth Factor, Type 3
  • Receptors, Fibroblast Growth Factor / genetics*
  • Receptors, Fibroblast Growth Factor / metabolism*
  • Signal Transduction / physiology*
  • Type C Phospholipases / metabolism*
  • Tyrosine / genetics

Substances

  • Oncogene Proteins, Fusion
  • Receptors, Fibroblast Growth Factor
  • Tyrosine
  • Fgfr3 protein, mouse
  • Protein-Tyrosine Kinases
  • Receptor, Fibroblast Growth Factor, Type 3
  • Type C Phospholipases
  • Phospholipase C gamma