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Lymphoma

Tumor suppressor TNFAIP3 (A20) is frequently deleted in Sézary syndrome

Leukemia volume 25, pages 1494–1501 (2011)Cite this article

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Abstract

Despite recent therapeutic improvements, the prognosis for patients suffering from Sézary syndrome (SS), a disseminated form of cutaneous T-cell lymphomas, is still poor. We identified bi- and monoallelic deletions of the tumor necrosis factor-α-induced protein 3 gene (TNFAIP3; A20) in a high proportion of SS patients as well as biallelic A20 deletion in the SS-derived cell line SeAx. Furthermore, we demonstrate that inhibition of A20 activates the NF-κB pathway thereby increasing the proliferation of normal T lymphocytes. On the other hand, the reconstitution of A20 expression slowed down the cell cycle in SeAx cells. Recently A20 inactivation has been reported in various B-cell lymphomas. In this study, we show that A20 is also a putative tumor suppressor in the T-cell malignancy—SS.

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Acknowledgements

This work was supported by the German José Carreras Leukemia Foundation (C.A.S.), Ministry of Science and Higher Education, Poland (GKP). Funding was provided in part by Framework VII EU (European Union/BMBF-0315207A) grant. The excellent technical assistance of Kathrin Assmus (Klinik für Innere Medizin C, Universität Greifswald) is gratefully acknowledged.

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Author notes

  1. F C M Braun and P Grabarczyk: These authors contributed equally to this study.

Authors and Affiliations

  1. Clinic for Internal Medicine C, University Greifswald, Greifswald, Germany

    F C M Braun, P Grabarczyk, F Busse, J Schröder, M Delin, G K Przybylski & C A Schmidt

  2. Department of Dermatology and Allergy, Skin Cancer Center, Charité—Humboldt University Berlin, Berlin, Germany

    M Möbs, F K Braun, J Eberle, M Beyer & W Sterry

  3. Department of Molecular Pathology, Institute of Human Genetics, Polish Academy of Sciences, Poznan, Poland

    G K Przybylski

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  1. F C M Braun
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Correspondence to C A Schmidt.

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Braun, F., Grabarczyk, P., Möbs, M. et al. Tumor suppressor TNFAIP3 (A20) is frequently deleted in Sézary syndrome. Leukemia 25, 1494–1501 (2011). https://doi.org/10.1038/leu.2011.101

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