MCL-1 but not BCL-XL is critical for the development and sustained expansion of thymic lymphoma in p53-deficient mice

Blood. 2014 Dec 18;124(26):3939-46. doi: 10.1182/blood-2014-09-601567. Epub 2014 Nov 3.

Abstract

Apoptosis plays a role in normal lymphopoiesis and lymphoid malignancies. Pro-survival MCL-1 is essential for survival of T-cell progenitors, BCL-XL for immature thymocytes, and BCL-2 for mature T cells. Conversely, little is known about the regulators that are required for the survival of T-cell lymphomas. We used constitutive and conditionally gene-targeted mice to investigate which pro-survival BCL-2 family member is required for the sustained survival of thymic lymphomas initiated by loss of p53. Constitutive loss of a single Mcl-1 allele delayed tumor onset. In contrast, lymphomas emerging in p53(-/-) mice in which Mcl-1 could be conditionally deleted had been selected for retention of MCL-1 expression. In contrast, complete loss of BCL-XL had no impact on lymphoma development in p53(-/-) mice. These results demonstrate that thymic lymphomas elicited by loss of p53 must arise from cancer-initiating cells that require MCL-1 for their survival. Acute deletion of both Mcl-1 alleles abrogated the expansion of p53(-/-) lymphomas in mice, whereas inducible loss of BCL-XL had little impact. This reveals that MCL-1 is essential for the sustained survival of these malignant cells and suggests that targeting MCL-1 may be an attractive strategy for the treatment of T-cell lymphoma.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Alleles
  • Animals
  • Apoptosis
  • Cell Survival
  • Cell Transplantation
  • Gene Deletion
  • Gene Expression Regulation, Neoplastic*
  • Genes, p53*
  • Genotype
  • Lymphoma, T-Cell / metabolism*
  • Mice
  • Mice, Inbred C57BL
  • Mutation
  • Myeloid Cell Leukemia Sequence 1 Protein / genetics
  • Myeloid Cell Leukemia Sequence 1 Protein / physiology*
  • Stem Cells / cytology
  • Thymus Neoplasms / metabolism*
  • bcl-X Protein / genetics
  • bcl-X Protein / physiology*

Substances

  • Bcl2l1 protein, mouse
  • Mcl1 protein, mouse
  • Myeloid Cell Leukemia Sequence 1 Protein
  • bcl-X Protein