The role of BCL6 in lymphomas and routes to therapy

Summary BCL6 is a transcription factor that has essential B‐cell and T‐cell roles in normal antibody responses. It is involved in chromosomal translocations in diffuse large B‐cell lymphoma (DBCL; including primary mediastinal B‐cell lymphoma) and nodular lymphocyte predominant Hodgkin lymphoma, and...

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Bibliographic Details
Published in:British journal of haematology 2011-01, Vol.152 (1), p.3-12
Main Authors: Wagner, Simon D., Ahearne, Matthew, Ferrigno, Paul Ko
Format: Article
Language:English
Subjects:
Animals
Antineoplastic Agents - pharmacology
BCL6
Biological and medical sciences
Chromosome aberrations
DNA-Binding Proteins - antagonists & inhibitors
DNA-Binding Proteins - genetics
DNA-Binding Proteins - physiology
Drug Design
germinal centre
Hematologic and hematopoietic diseases
Humans
Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis
lymphoma
Lymphoma - drug therapy
Lymphoma - genetics
Lymphoma - metabolism
Medical genetics
Medical sciences
Mice
Neoplasm Proteins - physiology
Proto-Oncogene Proteins c-bcl-6
transcription factor
translocation
Translocation, Genetic
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Summary:Summary BCL6 is a transcription factor that has essential B‐cell and T‐cell roles in normal antibody responses. It is involved in chromosomal translocations in diffuse large B‐cell lymphoma (DBCL; including primary mediastinal B‐cell lymphoma) and nodular lymphocyte predominant Hodgkin lymphoma, and is expressed in follicular lymphoma and Burkitt's lymphoma. The neoplastic T‐cells of angioimmunoblastic T‐cell lymphoma also express BCL6. BCL6 prevents terminal B‐cell differentiation largely through repression of PRDM1. In the “cell of origin” classification of DLBCL BCL6 is associated with the germinal centre subtype, which carries a good response to modern treatments. More recently, specific BCL6 antagonists, including small molecule inhibitors, have been developed. These antagonists have demonstrated that DLBCL cells, in which BCL6 is transcriptionally active, are dependent on this gene for survival. BCL6 antagonists are active against primary DLBCL and may find future application in the treatment of lymphomas.
ISSN:0007-1048
1365-2141
DOI:10.1111/j.1365-2141.2010.08420.x