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Immunodeficiencies due to defects of class-switch recombination

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Abstract

Maturation of the antibody response is dependent on class-switch recombination (CSR) and somatic hypermutation (SHM), that modify the structure and the affinity of immunoglobulins, respectively. The cellular and molecular mechanisms involved in these processes have long remained obscure. During the last years, careful investigation of a cohort of rare patients with defective antibody responses have led to the identification of several genes that are critically involved in CSR and SHM. At the same time, recognition that defective maturation of antibody responses may result from different mechanisms, has been essential to better define prognosis and to tailor more appropriate and specific forms of treatment.

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Acknowledgments

This work was supported by grant RBNE0189JJ_003 from the Italian Ministry of Education and Research (MIUR-FIRB).

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Authors and Affiliations

  1. Department of Pediatrics, “Angelo Nocivelli” Institute for Molecular Medicine, University of Brescia, Brescia, Italy

    Luigi D. Notarangelo, Gaetana Lanzi, Paola Toniati & Silvia Giliani

  2. Division of Immunology, Children’s Hospital, Harvard Medical School, Karp Research Building, 9th floor, 1 Blackfan Circle, Boston, MA, 02115, USA

    Luigi D. Notarangelo

Authors
  1. Luigi D. Notarangelo
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  2. Gaetana Lanzi
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  3. Paola Toniati
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  4. Silvia Giliani
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Correspondence to Luigi D. Notarangelo.

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Notarangelo, L.D., Lanzi, G., Toniati, P. et al. Immunodeficiencies due to defects of class-switch recombination. Immunol Res 38, 68–77 (2007). https://doi.org/10.1007/s12026-007-0023-1

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  • DOI: https://doi.org/10.1007/s12026-007-0023-1

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