Mechanism of co-operation of mutant IL-7R α and mutant NRAS in acute lymphoblastic leukemia: role of MYC

Haematologica. 2024 Jun 1;109(6):1726-1740. doi: 10.3324/haematol.2023.283559.

Abstract

Acute lymphoblastic leukemia (ALL) is an aggressive leukemia which can be derived from either T-cell or B-cell precursors. With current treatments, the survival rate is high, but the treatments are highly toxic with severe side effects. Individual mutations in IL7Ra and RAS pathways have been previously shown to be prevalent in ALL, and especially in relapsed patients. The relationship of IL-7Ra and RAS was investigated by transducing immature mouse thymocytes with the combination of these mutants. The resultant ALL cells were analyzed to identify the regulators and the oncoproteins that are up-regulated or down-regulated by the combination of IL7Ra with NRAS. Leukemia cells showed a significant increase in IL7Ra-mediated BCL2 expression, and an increase in MYC protein levels was mainly induced by NRAS signaling. MYC was both necessary and sufficient to replace mutant NRAS, and drugs targeting the MYC pathway showed a therapeutic benefit in IL-7Ra/NRAS T-ALL. We suggest that MYC protein stability can be regulated by PLK-1 kinase, which was increased mainly by the NRAS signal. These studies identify novel pathways of oncogenesis and new targets for intervention that could lead to better therapeutic development.

Publication types

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

MeSH terms

  • Animals
  • Cell Line, Tumor
  • GTP Phosphohydrolases* / genetics
  • GTP Phosphohydrolases* / metabolism
  • Gene Expression Regulation, Leukemic
  • Humans
  • Membrane Proteins / genetics
  • Membrane Proteins / metabolism
  • Mice
  • Monomeric GTP-Binding Proteins
  • Mutation*
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma* / genetics
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma* / metabolism
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma* / pathology
  • Proto-Oncogene Proteins c-bcl-2 / genetics
  • Proto-Oncogene Proteins c-bcl-2 / metabolism
  • Proto-Oncogene Proteins c-myc* / genetics
  • Proto-Oncogene Proteins c-myc* / metabolism
  • Receptors, Interleukin-7 / genetics
  • Receptors, Interleukin-7 / metabolism
  • Signal Transduction

Substances

  • Proto-Oncogene Proteins c-myc
  • GTP Phosphohydrolases
  • Receptors, Interleukin-7
  • Membrane Proteins
  • Nras protein, mouse
  • interleukin-7 receptor, alpha chain
  • NRAS protein, human
  • Proto-Oncogene Proteins c-bcl-2
  • Monomeric GTP-Binding Proteins

Grants and funding

Funding: This research was supported by the intramural program of the National Cancer Institute, National Institutes of Health.