RUNX1-EVI1 induces dysplastic hematopoiesis and acute leukemia of the megakaryocytic lineage in mice

The t(3;21)(q26;q22) translocation is a recurrent chromosomal abnormality found in chronic myelogenous leukemia in blastic crisis, myelodysplastic syndromes (MDS)-derived leukemia and de novo acute megakaryoblastic leukemia (AMKL) [1]. The translocation creates the RUNX1-EVI1 fusion gene whose resultant molecules contain the N-terminus of RUNX1 and almost the entire region of EVI1 [2]. RUNX1-EVI1 exhibits two main molecular functions [3]. One is a dominant-negative function over normal RUNX1 function, and the other is an aberrant EVI1 expression leading to repression of TGF β signaling [4], inhibition of CEBPA [5] and stimulation of AP-1 activity [6].
Source: Leukemia Research - Category: Hematology Authors: Tags: Research paper Source Type: research