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Thus, the generation of antigen (Ag)-specific memory cells and PCs underlies long-lived serological immunity, contributing to the success of most vaccines. Our understanding of immunity is often derived from animal models. However, analysis of individuals with monogenic defects that disrupt immune cell function are unprecedented models to link genotypes to clinical phenotypes, establish mechanisms of disease pathogenesis, and elucidate critical pathways for immune cell development and differentiation. Here, we review fundamental breakthroughs in unraveling the complexities of humoral immunity in humans that have come from the discovery of inborn errors disrupting B cell function.<\/jats:p>","DOI":"10.1084\/jem.20221105","type":"journal-article","created":{"date-parts":[[2023,5,16]],"date-time":"2023-05-16T02:21:08Z","timestamp":1684203668000},"update-policy":"https:\/\/doi.org\/10.1084\/jem.crossmarkpolicy","source":"Crossref","is-referenced-by-count":61,"title":["Inborn errors of human B cell development, differentiation, and function"],"prefix":"10.1084","volume":"220","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5360-5180","authenticated-orcid":true,"given":"Stuart G.","family":"Tangye","sequence":"first","affiliation":[{"id":[{"id":"https:\/\/ror.org\/01b3dvp57","id-type":"ROR","asserted-by":"publisher"}],"name":"Garvan Institute of Medical Research 1 , Darlinghurst, Australia"},{"name":"School of Clinical Medicine, Faculty of Medicine and Health, UNSW Sydney 2 , Sydney, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0709-3636","authenticated-orcid":true,"given":"Tina","family":"Nguyen","sequence":"additional","affiliation":[{"id":[{"id":"https:\/\/ror.org\/01b3dvp57","id-type":"ROR","asserted-by":"publisher"}],"name":"Garvan Institute of Medical Research 1 , Darlinghurst, Australia"},{"name":"School of Clinical Medicine, Faculty of Medicine and Health, UNSW Sydney 2 , Sydney, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9271-0004","authenticated-orcid":true,"given":"Elissa K.","family":"Deenick","sequence":"additional","affiliation":[{"id":[{"id":"https:\/\/ror.org\/01b3dvp57","id-type":"ROR","asserted-by":"publisher"}],"name":"Garvan Institute of Medical Research 1 , Darlinghurst, Australia"},{"name":"School of Clinical Medicine, Faculty of Medicine and Health, UNSW Sydney 2 , Sydney, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4697-7410","authenticated-orcid":true,"given":"Vanessa L.","family":"Bryant","sequence":"additional","affiliation":[{"id":[{"id":"https:\/\/ror.org\/01b6kha49","id-type":"ROR","asserted-by":"publisher"}],"name":"Immunology Division, Walter & Eliza Hall Institute 3 , Parkville, Australia"},{"name":"University of Melbourne 4 Department of Medical Biology, , Parkville, Australia"},{"name":"The Royal Melbourne Hospital 5 Department of Clinical Immunology & Allergy, , Parkville, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5387-8413","authenticated-orcid":true,"given":"Cindy S.","family":"Ma","sequence":"additional","affiliation":[{"id":[{"id":"https:\/\/ror.org\/01b3dvp57","id-type":"ROR","asserted-by":"publisher"}],"name":"Garvan Institute of Medical Research 1 , Darlinghurst, Australia"},{"name":"School of Clinical Medicine, Faculty of Medicine and Health, UNSW Sydney 2 , Sydney, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"291","published-online":{"date-parts":[[2023,6,5]]},"reference":[{"key":"2023060513525017800_bib1","doi-asserted-by":"publisher","first-page":"1391","DOI":"10.1007\/s10875-021-01049-9","article-title":"TCF3 dominant negative variant causes an early block in B-lymphopoiesis and agammaglobulinemia","volume":"41","author":"Al Sheikh","year":"2021","journal-title":"J. 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