Defects of class-switch recombination

The Journal of Allergy and Clinical Immunology
Luigi NotarangeloAnne Durandy

Abstract

Shaping of the secondary antibody repertoire is generated by means of class-switch recombination (CSR), which replaces IgM with other isotypes, and somatic hypermutation (SHM), which allows production of high-affinity antibodies. However, the molecular mechanisms underlying these important processes have long remained obscure. Immunodeficiency with hyper-IgM comprises a group of genetically heterogeneous defects of CSR variably associated with defects of SHM. The study of these patients has allowed us to recognize that both T-cell-B-cell interaction (resulting in CD40-mediated signaling) and intrinsic B-cell mechanisms are involved in CSR and SHM. Elucidation of the molecular defects underlying these disorders has been essential to better understand the molecular basis of Ig diversification and has offered the opportunity to define the clinical spectrum of these diseases and to prompt more accurate diagnostic and therapeutic approaches.

Citations

Feb 5, 2008·Journal of Clinical Immunology·Adriana A JesusJoão B Oliveira
Jul 4, 2009·Journal of Clinical Immunology·Asghar AghamohammadiLennart Hammarström
Jun 24, 2010·Current Allergy and Asthma Reports·Itai M Pessach
Oct 6, 2007·Immunologic Research·Luigi D NotarangeloSilvia Giliani
Oct 26, 2007·Nature Reviews. Immunology·László Maródi, Luigi D Notarangelo
May 16, 2007·Bone Marrow Transplantation·E MazzolariN Kutukculer
Jul 15, 2009·Clinical Microbiology Reviews·Ari J Fried, Francisco A Bonilla
May 15, 2010·Allergy, Asthma, and Clinical Immunology : Official Journal of the Canadian Society of Allergy and Clinical Immunology·Theresa S Cole, Andrew J Cant
Sep 21, 2012·Italian Journal of Pediatrics·Silvia MontellaFrancesca Santamaria
Jul 9, 2013·Clinical Reviews in Allergy & Immunology·M Teresa de la Morena, Robert P Nelson
Jun 16, 2014·Blood Cells, Molecules & Diseases·Manisha MadkaikarKanjaksha Ghosh
Mar 13, 2014·Journal of Clinical Immunology·John RoutesTroy Torgerson
Jan 7, 2014·Journal of Critical Care·Raquel AlmansaJesús F Bermejo-Martin
Jan 18, 2012·Journal of the Formosan Medical Association = Taiwan Yi Zhi·Wen-I LeeCheng-Hsun Chiu
Nov 6, 2010·The Journal of Allergy and Clinical Immunology·Dominique Gatto, Robert Brink
Nov 9, 2010·Advances in Pediatrics·E Richard StiehmHeather Lehman
Apr 14, 2009·Journal of Autoimmunity·Itai M Pessach, Luigi D Notarangelo
Sep 23, 2008·Clinical Immunology : the Official Journal of the Clinical Immunology Society·Melinda ErdosLászló Maródi
Apr 22, 2008·Immunology and Allergy Clinics of North America·Massimo MorraDavid T Miller
Oct 13, 2007·The Journal of Allergy and Clinical Immunology·William T ShearerJennifer M Puck
Sep 16, 2015·The Journal of Allergy and Clinical Immunology·Francisco A BonillaJames W Verbsky
Nov 10, 2013·Immunologic Research·Bonnie B Blomberg, Daniela Frasca
Oct 12, 2015·Immunology and Allergy Clinics of North America·Maryam Saifi, Christian A Wysocki
Feb 12, 2008·Clinical and Experimental Allergy : Journal of the British Society for Allergy and Clinical Immunology·J M Davies, R E O'Hehir
Mar 27, 2012·Scandinavian Journal of Immunology·N E KaracaN Kutukculer
Oct 20, 2012·Pediatric Transplantation·Nima Rezaei, Luigi D Notarangelo
May 27, 2010·Scandinavian Journal of Immunology·G J ArasonB R Ludviksson
Jun 26, 2007·The Journal of Allergy and Clinical Immunology·Javier Chinen, William T Shearer
Sep 18, 2015·Frontiers in Immunology·Marinos KallikourdisFederica Benvenuti
Feb 10, 2011·Biochemical and Biophysical Research Communications·Juan MaHisaya Akiba
Jun 15, 2011·Journal of Autoimmunity·Norbert GleicherDavid H Barad
Jan 17, 2009·Vaccine·Quan-en Yang
Aug 13, 2010·Blood·Gaetana LanziSilvia Giliani
Feb 27, 2014·Journal of Immunoassay & Immunochemistry·Payam MohammadinejadArndt Borkhardt

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