Implication des genes du CBF dans la leucemogenese

The AML1 gene encodes one of the alpha subunit of the transcriptionnal factor core binding factor (CBF). This gene is frequently involved in chromosomal translocations observed in various human hematopoietic malignancies. The gene coding for the other CBF subunit, CBFβ, is also rearranged as a result of chromosomal abnormalities observed in a subtype of acute myeloid leukemias. In addition to fusion protein-specific effects, the CBF subunit fusion oncoproteins exhibit dominant negative properties with respect to normal CBF functions. Recent data from murin models indicate that a second hit is needed to obtain a malignant phenotype. The aim of this manuscript is to summarize the present knowledge regarding normal and leukemic oncoproteins.