Fusion AML1 transcript in a radiation-associated leukemia results in a truncated inhibitory AML1 protein.

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)
Authors	R Hromas, T Busse, A Carroll, D Mack, R Shopnick, D E Zhang, H Nakshatri, K Richkind
Journal	Blood (Blood) Vol. 97 Issue 7 Pg. 2168-70 (Apr 01 2001) ISSN: 0006-4971 [Print] United States
PMID	11264187 (Publication Type: Journal Article)
Chemical References	AML1-AMP19 fusion protein, human Core Binding Factor Alpha 2 Subunit DNA, Complementary DNA-Binding Proteins Neoplasm Proteins Oncogene Proteins, Fusion Proto-Oncogene Proteins RUNX1 protein, human Recombinant Fusion Proteins Transcription Factors
Topics	Acute Disease Amino Acid Sequence Base Sequence Binding Sites (genetics) Chromosomes, Human, Pair 19 (genetics, ultrastructure) Chromosomes, Human, Pair 21 (genetics, ultrastructure) Core Binding Factor Alpha 2 Subunit DNA, Complementary (genetics) DNA-Binding Proteins (antagonists & inhibitors, chemistry, genetics) Humans In Situ Hybridization Leukemia, Myeloid (etiology, genetics) Leukemia, Radiation-Induced (genetics) Molecular Sequence Data Neoplasm Proteins Oncogene Proteins, Fusion Protein Structure, Tertiary Proto-Oncogene Proteins Recombinant Fusion Proteins (chemistry, genetics, pharmacology) Sequence Deletion Transcription Factors (antagonists & inhibitors, chemistry, genetics) Transcriptional Activation (genetics) Translocation, Genetic (genetics)

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