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Dissecting Epigenetic Dysregulation of Primary Antibody Deficiencies

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Abstract

Primary antibody deficiencies (PADs), the most prevalent inherited primary immunodeficiencies (PIDs), are associated with a wide range of genetic alterations (both monogenic or polygenic) in B cell-specific genes. However, correlations between the genotype and clinical manifestations are not evident in all cases indicating that genetic interactions, environmental and epigenetic factors may have a role in PAD pathogenesis. The recent identification of key defects in DNA methylation in common variable immunodeficiency as well as the multiple evidences on the role of epigenetic control during B cell differentiation, activation and during antibody formation highlight the importance of investing research efforts in dissecting the participation of epigenetic defects in this group of diseases. This review focuses on the role of epigenetic control in B cell biology which can provide clues for the study of potential novel pathogenic defects involved in PADs.

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Acknowledgments

This work was supported by grant SAF2014-55942-R from the Instituto de Salud Carlos III, organisms ascribed to the Ministerio de Economía y Competitividad and cofunded by FEDER funds/ European Regional Development Fund (ERDF) - a way to build Europe.

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Authors and Affiliations

  1. Chromatin and Disease Group, Cancer Epigenetics and Biology Programme (PEBC), Bellvitge Biomedical Research Institute (IDIBELL), 08908 L’Hospitalet de Llobregat, Barcelona, Spain

    Virginia C. Rodríguez-Cortez, Javier Rodríguez-Ubreva & Esteban Ballestar

  2. Clinical Immunology Department, University Hospital La Paz, Paseo de la Castellana 261, 28046, Madrid, Spain

    Lucia del Pino-Molina & Eduardo López-Granados

  3. Physiopathology of Lymphocytes in Immunodeficiencies Group, IdiPAZ Institute for Health Research, Paseo de la Castellana 261, 28046, Madrid, Spain

    Lucia del Pino-Molina & Eduardo López-Granados

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  1. Virginia C. Rodríguez-Cortez
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Rodríguez-Cortez, V.C., del Pino-Molina, L., Rodríguez-Ubreva, J. et al. Dissecting Epigenetic Dysregulation of Primary Antibody Deficiencies. J Clin Immunol 36 (Suppl 1), 48–56 (2016). https://doi.org/10.1007/s10875-016-0267-4

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