Abstract
The identification of clonal chromosomal translocations in human leukemias provided one of the first insights into the underlying pathogenesis of this clinically heterogeneous disease. Over the last decade a large number of these chromosomal rearrangements have been molecularly cloned and the involved genes identified. A surprising finding that has emerged from this work is that many of these chromosomal alterations target the genes encoding the AML1/CBFbeta transcription factor complex, a critical regulator of normal hematopoiesis. In this review, we summarize our present understanding of the mechanisms through which alterations of AML1/CBFbeta contribute to leukemogenesis.
Publication types
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Research Support, Non-U.S. Gov't
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Research Support, U.S. Gov't, P.H.S.
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Review
MeSH terms
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Acute Disease
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Animals
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Core Binding Factor Alpha 2 Subunit
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DNA-Binding Proteins / genetics*
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DNA-Binding Proteins / metabolism
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Gene Expression Regulation, Leukemic
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Hematopoiesis
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Hematopoietic Stem Cells / physiology*
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Humans
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Leukemia / etiology*
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Leukemia / genetics
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Leukemia, Myeloid / genetics
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Oncogene Proteins, Fusion / genetics*
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Oncogene Proteins, Fusion / metabolism
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Point Mutation
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Proto-Oncogene Proteins*
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RUNX1 Translocation Partner 1 Protein
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Transcription Factor AP-2
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Transcription Factors / genetics*
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Transcription Factors / metabolism
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Translocation, Genetic*
Substances
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Core Binding Factor Alpha 2 Subunit
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DNA-Binding Proteins
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Oncogene Proteins, Fusion
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Proto-Oncogene Proteins
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RUNX1 Translocation Partner 1 Protein
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RUNX1 protein, human
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RUNX1T1 protein, human
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Transcription Factor AP-2
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Transcription Factors