Abstract
About half of patients with diffuse large B-cell lymphoma (DLBCL) do not respond to or relapse soon after the standard chemotherapy, indicating a critical need to better understand the specific pathways perturbed in DLBCL for developing effective therapeutic approaches. Mice deficient in the E3 ubiquitin ligase Smurf2 spontaneously develop B-cell lymphomas that resemble human DLBCL with molecular features of germinal centre or post-germinal centre B cells. Here we show that Smurf2 mediates ubiquitination and degradation of YY1, a key germinal centre transcription factor. Smurf2 deficiency enhances YY1-mediated transactivation of c-Myc and B-cell proliferation. Furthermore, Smurf2 expression is significantly decreased in primary human DLBCL samples, and low levels of Smurf2 expression correlate with inferior survival in DLBCL patients. The Smurf2-YY1-c-Myc regulatory axis represents a novel pathway perturbed in DLBCL that suppresses B-cell proliferation and lymphomagenesis, suggesting pharmaceutical targeting of Smurf2 as a new therapeutic paradigm for DLBCL.
Publication types
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Research Support, N.I.H., Extramural
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Research Support, Non-U.S. Gov't
MeSH terms
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Animals
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B-Lymphocytes / metabolism
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B-Lymphocytes / pathology
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Cell Line, Tumor
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Cell Proliferation
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Gene Expression Regulation, Neoplastic*
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Germinal Center / metabolism
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Germinal Center / pathology
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Humans
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Lymphocyte Activation
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Lymphoma, Large B-Cell, Diffuse / genetics*
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Lymphoma, Large B-Cell, Diffuse / metabolism
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Lymphoma, Large B-Cell, Diffuse / pathology
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Mice
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Mice, Knockout
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Proteasome Endopeptidase Complex / metabolism
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Proteolysis
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Proto-Oncogene Proteins c-myc / genetics*
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Proto-Oncogene Proteins c-myc / metabolism
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Signal Transduction
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Transcription, Genetic
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Ubiquitin-Protein Ligases / deficiency
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Ubiquitin-Protein Ligases / genetics*
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Ubiquitin-Protein Ligases / metabolism
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Ubiquitination
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YY1 Transcription Factor / genetics*
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YY1 Transcription Factor / metabolism
Substances
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Myc protein, mouse
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Proto-Oncogene Proteins c-myc
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YY1 Transcription Factor
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Yy1 protein, mouse
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SMURF2 protein, human
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Smurf2 protein, mouse
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Ubiquitin-Protein Ligases
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Proteasome Endopeptidase Complex