Point mutations within a dimer interface homology domain of c-Mpl induce constitutive receptor activity and tumorigenicity

EMBO J. 1995 Nov 15;14(22):5569-78. doi: 10.1002/j.1460-2075.1995.tb00244.x.

Abstract

c-Mpl, a receptor for thrombopoietin (TPO), belongs to the haemopoietin/cytokine receptor superfamily, a group of cell surface molecules characterized by conserved sequence motifs within their ligand binding domains. A recurring mechanism for the activation of haemopoietin receptors is the formation of functional complexes by receptor subunit oligomerization. Within the growth hormone receptor, a cluster of extracellular amino acids forms a dimer interface domain that stabilizes ligand-induced homodimers. This domain appears to be functionally conserved in the erythropoietin (EPO) receptor because substitution of cysteines for residues in the analogous region causes EPO-independent receptor activation via disulfide-linked homodimerization. This report identifies an homologous domain within the c-Mpl receptor. The substitution of cysteine residues for specific amino acids in the dimer interface homology regions of c-Mpl induced constitutive receptor activity. Factor-dependent FDC-P1 and Ba/F3 cells expressing the active receptor mutants no longer required exogenous factors and proliferated autonomously. The results imply that the normal process of TPO-stimulated Mpl activation occurs through receptor homodimerization and is mediated by a conserved haemopoietin receptor dimer interface domain. Moreover, cells expressing activated mutant Mpl receptors were tumorigenic in transplanted mice. Thus, like v-mpl, its viral counterpart, mutated forms of the cellular mpl gene also have oncogenic potential.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Amino Acid Sequence
  • Animals
  • Base Sequence
  • Cell Count
  • Cell Line
  • Clone Cells
  • Cysteine / metabolism
  • Humans
  • Mice
  • Mice, Inbred BALB C
  • Mice, Inbred DBA
  • Molecular Sequence Data
  • Neoplasm Proteins*
  • Neoplasms, Experimental / etiology*
  • Oligodeoxyribonucleotides
  • Point Mutation
  • Proto-Oncogene Proteins / chemistry
  • Proto-Oncogene Proteins / genetics
  • Proto-Oncogene Proteins / metabolism*
  • Receptors, Cytokine / chemistry
  • Receptors, Cytokine / genetics
  • Receptors, Cytokine / metabolism*
  • Receptors, Thrombopoietin
  • Thrombopoietin* / metabolism

Substances

  • Neoplasm Proteins
  • Oligodeoxyribonucleotides
  • Proto-Oncogene Proteins
  • Receptors, Cytokine
  • Receptors, Thrombopoietin
  • MPL protein, human
  • Thrombopoietin
  • Cysteine