{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,10]],"date-time":"2026-06-10T16:25:00Z","timestamp":1781108700595,"version":"3.54.1"},"reference-count":32,"publisher":"National Academy of Sciences","issue":"22","content-domain":{"domain":["www.pnas.org"],"crossmark-restriction":true},"short-container-title":["Proc. Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[1998,10,27]]},"abstract":"<jats:p>Common Variable Immuno-Deficiency (CVID) is the most common symptomatic primary antibody-deficiency syndrome, but the basic immunologic defects underlying this syndrome are not well defined. We report here that among eight patients studied (six CVID and two hypogammaglobulinemic patients with recurrent infections), there is in two CVID patients a dramatic reduction in Ig V gene somatic hypermutation with 40\u201375% of IgG transcripts totally devoid of mutations in the circulating memory B cell compartment. Functional assays of the T cell compartment point to an intrinsic B cell defect in the process of antibody affinity maturation in these two cases.<\/jats:p>","DOI":"10.1073\/pnas.95.22.13135","type":"journal-article","created":{"date-parts":[[2002,7,26]],"date-time":"2002-07-26T10:42:40Z","timestamp":1027680160000},"page":"13135-13140","update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":80,"title":["Defect in IgV gene somatic hypermutation in Common Variable Immuno-Deficiency syndrome"],"prefix":"10.1073","volume":"95","author":[{"given":"Yves","family":"Levy","sequence":"first","affiliation":[{"name":"Unit\u00e9 d\u2019immunologie clinique, H\u00f4pital Henri Mondor, 51, avenue du Mar\u00e9chal de Lattre de Tassigny, 94010 Cr\u00e9teil Cedex 10, France; Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale Unit\u00e9 373, Facult\u00e9 de M\u00e9decine Necker-Enfants Malades, 156, rue de Vaugirard, 75730 Paris Cedex 15, France; and Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale Unit\u00e9 429, H\u00f4pital Necker-Enfants Malades, 149, rue de S\u00e8vres, 75730 Paris Cedex 15, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Neetu","family":"Gupta","sequence":"additional","affiliation":[{"name":"Unit\u00e9 d\u2019immunologie clinique, H\u00f4pital Henri Mondor, 51, avenue du Mar\u00e9chal de Lattre de Tassigny, 94010 Cr\u00e9teil Cedex 10, France; Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale Unit\u00e9 373, Facult\u00e9 de M\u00e9decine Necker-Enfants Malades, 156, rue de Vaugirard, 75730 Paris Cedex 15, France; and Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale Unit\u00e9 429, H\u00f4pital Necker-Enfants Malades, 149, rue de S\u00e8vres, 75730 Paris Cedex 15, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fran\u00e7oise","family":"Le Deist","sequence":"additional","affiliation":[{"name":"Unit\u00e9 d\u2019immunologie clinique, H\u00f4pital Henri Mondor, 51, avenue du Mar\u00e9chal de Lattre de Tassigny, 94010 Cr\u00e9teil Cedex 10, France; Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale Unit\u00e9 373, Facult\u00e9 de M\u00e9decine Necker-Enfants Malades, 156, rue de Vaugirard, 75730 Paris Cedex 15, France; and Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale Unit\u00e9 429, H\u00f4pital Necker-Enfants Malades, 149, rue de S\u00e8vres, 75730 Paris Cedex 15, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Corinne","family":"Garcia","sequence":"additional","affiliation":[{"name":"Unit\u00e9 d\u2019immunologie clinique, H\u00f4pital Henri Mondor, 51, avenue du Mar\u00e9chal de Lattre de Tassigny, 94010 Cr\u00e9teil Cedex 10, France; Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale Unit\u00e9 373, Facult\u00e9 de M\u00e9decine Necker-Enfants Malades, 156, rue de Vaugirard, 75730 Paris Cedex 15, France; and Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale Unit\u00e9 429, H\u00f4pital Necker-Enfants Malades, 149, rue de S\u00e8vres, 75730 Paris Cedex 15, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alain","family":"Fischer","sequence":"additional","affiliation":[{"name":"Unit\u00e9 d\u2019immunologie clinique, H\u00f4pital Henri Mondor, 51, avenue du Mar\u00e9chal de Lattre de Tassigny, 94010 Cr\u00e9teil Cedex 10, France; Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale Unit\u00e9 373, Facult\u00e9 de M\u00e9decine Necker-Enfants Malades, 156, rue de Vaugirard, 75730 Paris Cedex 15, France; and Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale Unit\u00e9 429, H\u00f4pital Necker-Enfants Malades, 149, rue de S\u00e8vres, 75730 Paris Cedex 15, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean-Claude","family":"Weill","sequence":"additional","affiliation":[{"name":"Unit\u00e9 d\u2019immunologie clinique, H\u00f4pital Henri Mondor, 51, avenue du Mar\u00e9chal de Lattre de Tassigny, 94010 Cr\u00e9teil Cedex 10, France; Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale Unit\u00e9 373, Facult\u00e9 de M\u00e9decine Necker-Enfants Malades, 156, rue de Vaugirard, 75730 Paris Cedex 15, France; and Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale Unit\u00e9 429, H\u00f4pital Necker-Enfants Malades, 149, rue de S\u00e8vres, 75730 Paris Cedex 15, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claude-Agn\u00e8s","family":"Reynaud","sequence":"additional","affiliation":[{"name":"Unit\u00e9 d\u2019immunologie clinique, H\u00f4pital Henri Mondor, 51, avenue du Mar\u00e9chal de Lattre de Tassigny, 94010 Cr\u00e9teil Cedex 10, France; Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale Unit\u00e9 373, Facult\u00e9 de M\u00e9decine Necker-Enfants Malades, 156, rue de Vaugirard, 75730 Paris Cedex 15, France; and Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale Unit\u00e9 429, H\u00f4pital Necker-Enfants Malades, 149, rue de S\u00e8vres, 75730 Paris Cedex 15, France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"341","published-online":{"date-parts":[[1998,10,27]]},"reference":[{"key":"e_1_3_3_1_2","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-118-9-199305010-00011"},{"key":"e_1_3_3_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0167-5699(97)01086-4"},{"key":"e_1_3_3_3_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.86.20.8015"},{"key":"e_1_3_3_4_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.89.22.10653"},{"key":"e_1_3_3_5_2","doi-asserted-by":"publisher","DOI":"10.1038\/2281045a0"},{"key":"e_1_3_3_6_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-065X.1988.tb00763.x"},{"key":"e_1_3_3_7_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-065X.1986.tb01484.x"},{"key":"e_1_3_3_8_2","doi-asserted-by":"publisher","DOI":"10.1038\/354389a0"},{"key":"e_1_3_3_9_2","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(91)90289-B"},{"key":"e_1_3_3_10_2","doi-asserted-by":"publisher","DOI":"10.1084\/jem.172.6.1681"},{"key":"e_1_3_3_11_2","doi-asserted-by":"publisher","DOI":"10.1016\/S1074-7613(00)80240-X"},{"key":"e_1_3_3_12_2","doi-asserted-by":"publisher","DOI":"10.1084\/jem.172.6.1717"},{"key":"e_1_3_3_13_2","doi-asserted-by":"publisher","DOI":"10.1084\/jem.177.2.409"},{"key":"e_1_3_3_14_2","doi-asserted-by":"publisher","DOI":"10.1084\/jem.173.2.395"},{"key":"e_1_3_3_15_2","doi-asserted-by":"publisher","DOI":"10.1002\/eji.1830250926"},{"key":"e_1_3_3_16_2","doi-asserted-by":"publisher","DOI":"10.1016\/0167-4781(92)90134-L"},{"key":"e_1_3_3_17_2","doi-asserted-by":"publisher","DOI":"10.1002\/eji.1830231232"},{"key":"e_1_3_3_18_2","doi-asserted-by":"publisher","DOI":"10.1093\/intimm\/8.5.701"},{"key":"e_1_3_3_19_2","doi-asserted-by":"publisher","DOI":"10.1084\/jem.187.1.59"},{"key":"e_1_3_3_20_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.95.5.2463"},{"key":"e_1_3_3_21_2","doi-asserted-by":"publisher","DOI":"10.1084\/jem.180.4.1383"},{"key":"e_1_3_3_22_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V89.4.1288"},{"key":"e_1_3_3_23_2","doi-asserted-by":"publisher","DOI":"10.1016\/0167-5699(92)90199-H"},{"key":"e_1_3_3_24_2","doi-asserted-by":"publisher","DOI":"10.1038\/367425a0"},{"key":"e_1_3_3_25_2","first-page":"334","volume":"78","author":"Pastorelli G","year":"1989","unstructured":"G Pastorelli, M G Roncarolo, J L Touraine, G Peronne, P A Tovo, J E De Vries Clin Exp Immunol 78, 334\u2013340 (1989).","journal-title":"Clin Exp Immunol"},{"key":"e_1_3_3_26_2","doi-asserted-by":"crossref","first-page":"3762","DOI":"10.4049\/jimmunol.144.10.3762","volume":"144","author":"Sneller M C","year":"1990","unstructured":"M C Sneller, W Strober J Immunol 144, 3762\u20133769 (1990).","journal-title":"J Immunol"},{"key":"e_1_3_3_27_2","doi-asserted-by":"publisher","DOI":"10.1016\/0140-6736(93)92272-U"},{"key":"e_1_3_3_28_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.91.3.1099"},{"key":"e_1_3_3_29_2","doi-asserted-by":"publisher","DOI":"10.1093\/emboj\/16.13.3987"},{"key":"e_1_3_3_30_2","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(94)90316-6"},{"key":"e_1_3_3_31_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-065X.1997.tb00958.x"},{"key":"e_1_3_3_32_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199410063311405"}],"container-title":["Proceedings of the National Academy of Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/pnas.org\/doi\/pdf\/10.1073\/pnas.95.22.13135","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,4,22]],"date-time":"2023-04-22T08:30:31Z","timestamp":1682152231000},"score":1,"resource":{"primary":{"URL":"https:\/\/pnas.org\/doi\/full\/10.1073\/pnas.95.22.13135"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1998,10,27]]},"references-count":32,"journal-issue":{"issue":"22","published-print":{"date-parts":[[1998,10,27]]}},"alternative-id":["10.1073\/pnas.95.22.13135"],"URL":"https:\/\/doi.org\/10.1073\/pnas.95.22.13135","relation":{},"ISSN":["0027-8424","1091-6490"],"issn-type":[{"value":"0027-8424","type":"print"},{"value":"1091-6490","type":"electronic"}],"subject":[],"published":{"date-parts":[[1998,10,27]]},"assertion":[{"value":"1998-06-08","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"1998-10-27","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}