Abstract
AML1 is a transcription factor that is essential for normal hematopoietic development. It is the most frequent target for translocations in acute leukemia. Recently, fluorescence in situ hybridization was used to identify a novel syndrome of radiation-associated secondary acute myelogenous leukemia that had AML1 translocations. Using polymerase chain reaction, the AML1 fusion transcript was isolated from the patient who had a t(19;21) radiation-associated leukemia. The AML1 gene is fused out of frame to chromosome 19 sequences, resulting in a truncated AML protein bearing the DNA binding domain but not the transcriptional activation domain. This fusion AML1 protein functions as an inhibitor of the normal AML1 protein. (Blood. 2001;97:2168-2170)
MeSH terms
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Acute Disease
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Amino Acid Sequence
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Base Sequence
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Binding Sites / genetics
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Chromosomes, Human, Pair 19 / genetics
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Chromosomes, Human, Pair 19 / ultrastructure*
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Chromosomes, Human, Pair 21 / genetics
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Chromosomes, Human, Pair 21 / ultrastructure*
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Core Binding Factor Alpha 2 Subunit
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DNA, Complementary / genetics
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DNA-Binding Proteins / antagonists & inhibitors
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DNA-Binding Proteins / chemistry
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DNA-Binding Proteins / genetics*
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Humans
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In Situ Hybridization
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Leukemia, Myeloid / etiology
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Leukemia, Myeloid / genetics*
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Leukemia, Radiation-Induced / genetics*
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Molecular Sequence Data
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Neoplasm Proteins*
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Oncogene Proteins, Fusion
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Protein Structure, Tertiary
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Proto-Oncogene Proteins*
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Recombinant Fusion Proteins / chemistry
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Recombinant Fusion Proteins / genetics*
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Recombinant Fusion Proteins / pharmacology
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Sequence Deletion
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Transcription Factors / antagonists & inhibitors
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Transcription Factors / chemistry
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Transcription Factors / genetics*
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Transcriptional Activation / genetics*
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Translocation, Genetic / genetics*
Substances
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AML1-AMP19 fusion protein, human
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Core Binding Factor Alpha 2 Subunit
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DNA, Complementary
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DNA-Binding Proteins
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Neoplasm Proteins
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Oncogene Proteins, Fusion
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Proto-Oncogene Proteins
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RUNX1 protein, human
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Recombinant Fusion Proteins
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Transcription Factors