Abstract
T cell acute lymphoblastic leukemia (T-ALL) is an aggressive hematopoietic malignancy of thymocytes affecting preferentially children and adolescents. The disease is heterogeneous and characterized by a large set of chromosomal and genetic alterations that deregulate the growth of maturing thymocytes. The identification of activating point mutations in NOTCH1 in more then 50% of all T-ALL cases highlights the NOTCH1 cascade as a central player of T-ALL pathogenesis. In this review, we summarize and update more recent findings on the molecular mechanisms of T-ALL with a particular emphasis on the oncogenic properties of aberrant NOTCH1 signaling.
Copyright © 2011 Elsevier Ltd. All rights reserved.
Publication types
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Research Support, Non-U.S. Gov't
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Review
MeSH terms
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Adolescent
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Animals
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Cell Differentiation / genetics
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Cell Differentiation / immunology
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Child
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Gene Expression Regulation, Leukemic / immunology*
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Gene Regulatory Networks / immunology*
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Genes, T-Cell Receptor
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Humans
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Mice
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MicroRNAs / immunology
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MicroRNAs / metabolism
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Molecular Targeted Therapy
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Neoplasm Proteins / genetics
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Neoplasm Proteins / immunology
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Neoplasm Proteins / metabolism
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Point Mutation
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Precursor T-Cell Lymphoblastic Leukemia-Lymphoma / immunology*
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Precursor T-Cell Lymphoblastic Leukemia-Lymphoma / pathology
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Precursor T-Cell Lymphoblastic Leukemia-Lymphoma / therapy
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Receptor, Notch1 / genetics
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Receptor, Notch1 / immunology*
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Receptor, Notch1 / metabolism
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Signal Transduction / genetics
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Signal Transduction / immunology*
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Species Specificity
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T-Lymphocytes / cytology
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T-Lymphocytes / immunology
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T-Lymphocytes / metabolism
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Thymocytes / immunology*
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Thymocytes / metabolism
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Thymocytes / pathology
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Translocation, Genetic
Substances
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MicroRNAs
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Neoplasm Proteins
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Receptor, Notch1