Abstract
To identify oncogenic pathways in T cell acute lymphoblastic leukemia (T-ALL), we combined expression profiling of 117 pediatric patient samples and detailed molecular-cytogenetic analyses including the Chromosome Conformation Capture on Chip (4C) method. Two T-ALL subtypes were identified that lacked rearrangements of known oncogenes. One subtype associated with cortical arrest, expression of cell cycle genes, and ectopic NKX2-1 or NKX2-2 expression for which rearrangements were identified. The second subtype associated with immature T cell development and high expression of the MEF2C transcription factor as consequence of rearrangements of MEF2C, transcription factors that target MEF2C, or MEF2C-associated cofactors. We propose NKX2-1, NKX2-2, and MEF2C as T-ALL oncogenes that are activated by various rearrangements.
Copyright © 2011 Elsevier Inc. All rights reserved.
Publication types
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Research Support, Non-U.S. Gov't
MeSH terms
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Adolescent
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Cell Proliferation
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Child
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Child, Preschool
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Cluster Analysis
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Female
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Gene Expression Regulation, Leukemic
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Gene Rearrangement
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Genome, Human*
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Homeobox Protein Nkx-2.2
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Homeodomain Proteins / genetics*
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Homeodomain Proteins / physiology
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Humans
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Infant
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MADS Domain Proteins / genetics*
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MADS Domain Proteins / physiology
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MEF2 Transcription Factors
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Male
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Myogenic Regulatory Factors / genetics*
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Myogenic Regulatory Factors / physiology
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Nuclear Proteins / genetics*
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Nuclear Proteins / physiology
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Oncogenes*
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Precursor T-Cell Lymphoblastic Leukemia-Lymphoma / etiology
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Precursor T-Cell Lymphoblastic Leukemia-Lymphoma / genetics*
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Precursor T-Cell Lymphoblastic Leukemia-Lymphoma / pathology
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Thyroid Nuclear Factor 1
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Transcription Factors / genetics*
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Transcription Factors / physiology
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Transcription, Genetic*
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Zebrafish Proteins
Substances
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Homeobox Protein Nkx-2.2
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Homeodomain Proteins
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MADS Domain Proteins
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MEF2 Transcription Factors
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MEF2C protein, human
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Myogenic Regulatory Factors
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NKX2-2 protein, human
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Nuclear Proteins
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Thyroid Nuclear Factor 1
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Transcription Factors
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Zebrafish Proteins
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nkx2.2b protein, zebrafish