Acute myeloid leukemia: a central role for the ETS factor ERG

Abstract

Acute myeloid leukemia is a cancer of the myeloid lineage, characterized by the rapid proliferation of immature progenitor cells which accumulate in the bone marrow and interfere with the production of normal blood cells. Aberrant regulation of E-twenty-six (ETS) specific transcription factors such as PU.1 (SPI1) is thought to play a major role in this type of leukemia. The importance of these factors in normal blood development is highlighted by the fact that numerous studies showed a direct link between hematopoietic defects and aberrant regulation of ETS factor expression or expression of mutant forms. Apart from PU.1, another ETS factor, ERG, has emerged as a key player in normal hematopoiesis. Here, the role of this protein in normal and aberrant blood development will be discussed as well as the possibilities to therapeutically target this ETS factor.