Fusion-protein truncation provides new insights into leukemogenesis

摘要

AML1-ETO (also known as RUNX1-ETO) is the fusion-protein transcription factor product of the 8;21 transloca-tion. The translocation is present in up to 40% of leukemias of the French-American-British M2 subtype and is one of the most common events associated with myeloid leukemia. Clarifying the role of AML1-ETO in leukemogenesis has been difficult because the expression of the oncoprotein is not sufficient to cause disease. The challenge is reflected by the number of models that have been devised to study AML1-ETO in mice. In this issue of PNAS, Zhang and colleagues (1) advance the understanding of AML1-ETO another step with the discovery that a C-terminal truncation mutation produces penetrant leukemia.