Leukemogenesis by CBF oncoproteins.
The AML1 and CBFbeta subunits of core binding factor (CBF) are involved in several chromosomal abnormalities frequently associated with acute leukemias. As a result, the CBFbeta-SMMHC, AML1-ETO and AML1-MDS1/EVI1 fusion proteins are expressed in subsets of acute myeloid leukemia, and TEL-AML1 is expressed in B-lineage acute lymphocytic leukemia. These CBF oncoproteins likely contribute to leukemogenesis in part by inhibiting endogenous CBF. As a result they are expected to inhibit differentiation and perhaps apoptosis. In addition, the domains unique to each fusion protein may also contribute to leukemogenesis via unique mechanisms.
Friedman
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Division of Pediatric Oncology, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
D017209:Apoptosis; D050676:Core Binding Factor Alpha 2 Subunit; D004268:DNA-Binding Proteins / Q000502:physiology*; D006801:Humans; D007938:Leukemia / Q000209:etiology*; D000074008:MDS1 and EVI1 Complex Locus Protein; D009363:Neoplasm Proteins; D015514:Oncogene Proteins, Fusion / Q000502:physiology*; D011506:Proteins / Q000502:physiology; D011518:Proto-Oncogene Proteins; D011519:Proto-Oncogenes; D000075142:RUNX1 Translocation Partner 1 Protein; D050656:Transcription Factor AP-2; D014157:Transcription Factors / Q000502:physiology*
D050676:Core Binding Factor Alpha 2 Subunit; D004268:DNA-Binding Proteins; D000074008:MDS1 and EVI1 Complex Locus Protein; C063575:MECOM protein, human; D009363:Neoplasm Proteins; D015514:Oncogene Proteins, Fusion; D011506:Proteins; D011518:Proto-Oncogene Proteins; D000075142:RUNX1 Translocation Partner 1 Protein; C493728:RUNX1 protein, human; C082222:RUNX1T1 protein, human; D050656:Transcription Factor AP-2; D014157:Transcription Factors