Signal transduction and transforming properties of the TEL-TRKC fusions associated with t(12;15)(p13;q25) in congenital fibrosarcoma and acute myelogenous leukemia

EMBO J. 2000 Apr 17;19(8):1827-38. doi: 10.1093/emboj/19.8.1827.

Abstract

The TEL-TRKC fusion is expressed as a consequence of t(12;15)(p13;q25), and is associated with two human cancers: congenital fibrosarcoma and acute myelogenous leukemia (AML). We report that the T/T(F) and T/T(L) fusion variants associated with congenital fibrosarcoma and AML, respectively, are constitutively tyrosine phosphorylated, and confer factor-independent growth to the murine hematopoietic cell line Ba/F3. Retroviral transduction of T/T(L) causes a rapidly fatal myeloproliferative disease in a murine bone marrow transplant (BMT) model, whereas T/T(F) causes a long-latency, pre-B-cell lymphoblastic lymphoma. TEL-TRKC variants are potent activators of the MAP kinase pathway, but neither variant activates Stat5 or other Stat family members. T/T(L), but not T/T(F), induces tyrosine phosphorylation of phospholipase Cgamma (PLCgamma), phosphoinositol-3 kinase and SHC. However, mutation analysis demonstrates that PLCgamma tyrosine phos phorylation by T/T(L) is dispensable for induction of the myeloproliferative phenotype by T/T(L). Collectively, these data demonstrate that the TEL-TRKC fusion variants are oncoproteins that activate the MAP kinase pathway, and do not require activation of either PLCgamma or Stat5 for efficient induction of a myeloproliferative phenotype in the murine BMT model.

Publication types

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

MeSH terms

  • 3T3 Cells
  • Animals
  • Bone Marrow / metabolism
  • Cell Transformation, Neoplastic
  • Chromosomes, Human, Pair 12*
  • Chromosomes, Human, Pair 15*
  • DNA-Binding Proteins / metabolism*
  • ETS Translocation Variant 6 Protein
  • Fibrosarcoma / congenital
  • Fibrosarcoma / genetics*
  • Hematopoietic Stem Cells / metabolism
  • Humans
  • Isoenzymes / metabolism
  • Leukemia, Myeloid, Acute / genetics*
  • MAP Kinase Signaling System
  • Mice
  • Milk Proteins*
  • Muscle, Skeletal / metabolism
  • Oncogene Proteins, Fusion / metabolism*
  • Phosphatidylinositol 3-Kinases / metabolism
  • Phospholipase C gamma
  • Phosphorylation
  • Proto-Oncogene Proteins c-ets
  • Receptor, trkC / metabolism*
  • Repressor Proteins*
  • STAT5 Transcription Factor
  • Signal Transduction
  • Tissue Distribution
  • Trans-Activators / metabolism
  • Transcription Factors / metabolism*
  • Translocation, Genetic*
  • Tumor Cells, Cultured
  • Type C Phospholipases / metabolism
  • Tyrosine / metabolism

Substances

  • DNA-Binding Proteins
  • Isoenzymes
  • Milk Proteins
  • Oncogene Proteins, Fusion
  • Proto-Oncogene Proteins c-ets
  • Repressor Proteins
  • STAT5 Transcription Factor
  • TEL-TRKC fusion protein, human
  • Trans-Activators
  • Transcription Factors
  • Tyrosine
  • Phosphatidylinositol 3-Kinases
  • Receptor, trkC
  • Type C Phospholipases
  • Phospholipase C gamma