Abstract
The expression of both Notch3 and pre-T-cell-receptor (pre-TCR) invariant chain appears to be a common feature of all T-cell acute lymphoblastic leukemias (T-ALL). Notch genes, and other genes that are dysregulated in some T-ALL subgroups, encode factors that play a crucial role in both T-cell development and leukemogenesis. A complex network of signals, involving Notchs, pre-TCR, nuclear factor kappaB and E2A, appears to be responsible for the leukemogenesis process. Thus, T-ALL is a paradigm for developmental pathways that underlie the pathogenesis of this disease.
Publication types
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Research Support, Non-U.S. Gov't
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Review
MeSH terms
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Animals
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Basic Helix-Loop-Helix Transcription Factors
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DNA-Binding Proteins / metabolism
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Humans
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Leukemia, T-Cell / genetics*
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Leukemia, T-Cell / metabolism*
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Membrane Proteins / genetics
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Membrane Proteins / metabolism*
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Models, Biological
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NF-kappa B / metabolism
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Precursor Cell Lymphoblastic Leukemia-Lymphoma / genetics*
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Precursor Cell Lymphoblastic Leukemia-Lymphoma / metabolism*
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Proto-Oncogene Proteins / genetics
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Proto-Oncogene Proteins / metabolism*
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Receptor, Notch1
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Receptor, Notch3
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Receptors, Cell Surface*
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Receptors, Notch
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Signal Transduction
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Transcription Factors / metabolism
Substances
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Basic Helix-Loop-Helix Transcription Factors
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DNA-Binding Proteins
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Membrane Proteins
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NF-kappa B
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NOTCH1 protein, human
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NOTCH3 protein, human
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Proto-Oncogene Proteins
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Receptor, Notch1
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Receptor, Notch3
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Receptors, Cell Surface
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Receptors, Notch
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TCF3 protein, human
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Transcription Factors