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Following immune response or infection within the body, activation of T cell\u2010dependent and T cell\u2010independent antigens triggers the activation of activation\u2010induced cytidine deaminase, initiating the CSR process. CSR has the capacity to modify the functional properties of antibodies, thereby contributing to the adaptive immune response in the organism. Ig CSR defects, characterized by an abnormal relative frequency of Ig isotypes, represent a rare form of primary immunodeficiency. Elucidating the molecular basis of Ig diversification is essential for a better understanding of diseases related to Ig CSR defects and could provide clues for clinical diagnosis and therapeutic approaches. Here, we review the most recent insights on the diversification of five Ig isotypes and choose several classic diseases, including hyper\u2010IgM syndrome, Waldenstr\u00f6m macroglobulinemia, hyper\u2010IgD syndrome, selective IgA deficiency, hyper\u2010IgE syndrome, multiple myeloma, and Burkitt lymphoma, to illustrate the mechanism of Ig CSR deficiency. The investigation into the underlying mechanism of Ig CSR holds significant potential for the advancement of increasingly precise diagnostic and therapeutic approaches.<\/jats:p>","DOI":"10.1002\/mco2.662","type":"journal-article","created":{"date-parts":[[2024,8,14]],"date-time":"2024-08-14T02:55:10Z","timestamp":1723604110000},"source":"Crossref","is-referenced-by-count":34,"title":["Immunoglobulin class\u2010switch recombination: Mechanism, regulation, and related diseases"],"prefix":"10.1002","volume":"5","author":[{"given":"Jia\u2010Chen","family":"Liu","sequence":"first","affiliation":[{"name":"Institute of Hematology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology  Wuhan China"},{"name":"Department of Pathogen Biology School of Basic Medicine, Tongji Medical College and State Key Laboratory for Diagnosis and Treatment of Severe Zoonotic Infectious Diseases, Huazhong University of Science and Technology  Wuhan Hubei 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