C/EBPalpha in leukemogenesis: a matter of being in the right place with the right signals
Program in Gene Function and Expression
Medical Subject Headings
Animals; CCAAT-Enhancer-Binding Protein-alpha; Gene Expression Profiling; Leukemia, Myelomonocytic, Acute; Mice; Mutant Proteins; Myeloid Progenitor Cells; Neoplastic Stem Cells; Protein Isoforms
Cancer Biology | Genetics and Genomics
Leukemia-initiating cells can originate from hematopoietic progenitor cells that have acquired self-renewal capacity upon transformation with leukemic fusion genes. In this issue of Cancer Cell, Kirstetter and colleagues describe a mouse model for the frequent CEBPA mutations in human acute myeloid leukemia that result in the synthesis of only the 30kDa isoform, but not the 42kDa isoform of C/EBPalpha. This mutation uncouples C/EBPalpha's roles in myeloid differentiation and proliferation control. Furthermore, this mutation activates self-renewal in committed myeloid progenitor cells and induces myeloid malignancy with complete penetrance that is sustained by leukemia-initiating cells with a committed myeloid molecular signature.
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Citation: Cancer Cell. 2008 Apr;13(4):289-91. Link to article on publisher's site