MLL chimeric protein activation renders cells vulnerable to chromosomal damage: an explanation for the very short latency of infant leukemia

Genes Chromosomes Cancer. 2006 Aug;45(8):754-60. doi: 10.1002/gcc.20338.

Abstract

MLL fusion genes are a predominant feature of acute leukemias in infants and in secondary acute myeloid leukemia (AML) associated with prior chemotherapy with topo-II poisons. The former is considered to possibly arise in utero via transplacental chemical exposure. A striking feature of these leukemias is their malignancy and remarkably brief latencies implying the rapid acquisition of any necessary additional mutations. We have suggested that these coupled features might be explained if MLL fusion gene encoded proteins rendered cells more vulnerable to further DNA damage and mutation in the presence of chronic exposure to the agent(s) that induced the MLL fusion itself. We have tested this idea by exploiting a hormone regulated MLL-ENL (MLLT1) activation system and show that MLL-ENL function in normal murine progenitor cells substantially increases the incidence of chromosomal abnormalities in proliferating cells that survive exposure to etoposide VP-16. This phenotype is associated with an altered pattern of cell cycle arrest and/or apoptosis.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Cell Line, Transformed
  • Chromosome Aberrations*
  • DNA Damage* / genetics
  • Estrogen Antagonists / metabolism
  • Estrogen Antagonists / pharmacology
  • Humans
  • In Situ Hybridization, Fluorescence
  • Infant
  • Leukemia / genetics*
  • Mice
  • Myeloid-Lymphoid Leukemia Protein / genetics
  • Myeloid-Lymphoid Leukemia Protein / metabolism*
  • Oncogene Proteins, Fusion / genetics
  • Oncogene Proteins, Fusion / metabolism*
  • Reaction Time / drug effects
  • Stem Cells / drug effects
  • Stem Cells / metabolism
  • Tamoxifen / analogs & derivatives
  • Tamoxifen / metabolism
  • Tamoxifen / pharmacology

Substances

  • Estrogen Antagonists
  • MLL-ENL oncoprotein, human
  • Oncogene Proteins, Fusion
  • Tamoxifen
  • Myeloid-Lymphoid Leukemia Protein
  • afimoxifene