{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,16]],"date-time":"2026-02-16T17:43:42Z","timestamp":1771263822868,"version":"3.50.1"},"reference-count":37,"publisher":"Proceedings of the National Academy of Sciences","issue":"5","content-domain":{"domain":["www.pnas.org"],"crossmark-restriction":true},"short-container-title":["Proc. Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[2005,2]]},"abstract":"<jats:p>\n            Nijmegen breakage syndrome (NBS) is a rare autosomal recessive disorder characterized by predisposition to hematopoietic malignancy, cell-cycle checkpoint defects, and ionizing radiation sensitivity. NBS is caused by a hypomorphic mutation of the\n            <jats:italic>NBS1<\/jats:italic>\n            gene, encoding nibrin, which forms a protein complex with Mre11 and Rad50, both involved in DNA repair. Nibrin localizes to chromosomal sites of class switching, and B cells from NBS patients show an enhanced presence of microhomologies at the sites of switch recombination. Because nibrin is crucial for embryonic survival, direct demonstration by targeted deletion that nibrin functions in class switch recombination has been lacking. Here, we show by cell-type-specific conditional inactivation of\n            <jats:italic>Nbn<\/jats:italic>\n            , the murine homologue of\n            <jats:italic>NBS1<\/jats:italic>\n            , that nibrin plays a role in the repair of \u03b3-irradiation damage, maintenance of chromosomal stability, and the recombination of Ig constant region genes in B lymphocytes.\n          <\/jats:p>","DOI":"10.1073\/pnas.0409191102","type":"journal-article","created":{"date-parts":[[2005,1,25]],"date-time":"2005-01-25T01:26:07Z","timestamp":1106616367000},"page":"1584-1589","update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":88,"title":["Nibrin functions in Ig class-switch recombination"],"prefix":"10.1073","volume":"102","author":[{"given":"Sven","family":"Kracker","sequence":"first","affiliation":[{"name":"German Rheumatism Research Center, Schumannstrasse 21-22, 10117 Berlin, Germany; Institute of Human Genetics, Charit\u00e9 Medical University, Augustenburger Platz 1, 13353 Berlin, Germany; International Agency for Research on Cancer, Lyon 150 Cours Albert Thomas, 69372 Lyon Cedex 08, France; Amaxa GmbH, Nattermannallee 1, 50829 Cologne, Germany; and Experimental Rheumatology, Charit\u00e9, Humboldt University, Schumannstrasse 21-22, 10117 Berlin, Germany"}]},{"given":"Yvonne","family":"Bergmann","sequence":"additional","affiliation":[{"name":"German Rheumatism Research Center, Schumannstrasse 21-22, 10117 Berlin, Germany; Institute of Human Genetics, Charit\u00e9 Medical University, Augustenburger Platz 1, 13353 Berlin, Germany; International Agency for Research on Cancer, Lyon 150 Cours Albert Thomas, 69372 Lyon Cedex 08, France; Amaxa GmbH, Nattermannallee 1, 50829 Cologne, Germany; and Experimental Rheumatology, Charit\u00e9, Humboldt University, Schumannstrasse 21-22, 10117 Berlin, Germany"}]},{"given":"Ilja","family":"Demuth","sequence":"additional","affiliation":[{"name":"German Rheumatism Research Center, Schumannstrasse 21-22, 10117 Berlin, Germany; Institute of Human Genetics, Charit\u00e9 Medical University, Augustenburger Platz 1, 13353 Berlin, Germany; International Agency for Research on Cancer, Lyon 150 Cours Albert Thomas, 69372 Lyon Cedex 08, France; Amaxa GmbH, Nattermannallee 1, 50829 Cologne, Germany; and Experimental Rheumatology, Charit\u00e9, Humboldt University, Schumannstrasse 21-22, 10117 Berlin, Germany"}]},{"given":"Pierre-Olivier","family":"Frappart","sequence":"additional","affiliation":[{"name":"German Rheumatism Research Center, Schumannstrasse 21-22, 10117 Berlin, Germany; Institute of Human Genetics, Charit\u00e9 Medical University, Augustenburger Platz 1, 13353 Berlin, Germany; International Agency for Research on Cancer, Lyon 150 Cours Albert Thomas, 69372 Lyon Cedex 08, France; Amaxa GmbH, Nattermannallee 1, 50829 Cologne, Germany; and Experimental Rheumatology, Charit\u00e9, Humboldt University, Schumannstrasse 21-22, 10117 Berlin, Germany"}]},{"given":"Gabriele","family":"Hildebrand","sequence":"additional","affiliation":[{"name":"German Rheumatism Research Center, Schumannstrasse 21-22, 10117 Berlin, Germany; Institute of Human Genetics, Charit\u00e9 Medical University, Augustenburger Platz 1, 13353 Berlin, Germany; International Agency for Research on Cancer, Lyon 150 Cours Albert Thomas, 69372 Lyon Cedex 08, France; Amaxa GmbH, Nattermannallee 1, 50829 Cologne, Germany; and Experimental Rheumatology, Charit\u00e9, Humboldt University, Schumannstrasse 21-22, 10117 Berlin, Germany"}]},{"given":"Rainer","family":"Christine","sequence":"additional","affiliation":[{"name":"German Rheumatism Research Center, Schumannstrasse 21-22, 10117 Berlin, Germany; Institute of Human Genetics, Charit\u00e9 Medical University, Augustenburger Platz 1, 13353 Berlin, Germany; International Agency for Research on Cancer, Lyon 150 Cours Albert Thomas, 69372 Lyon Cedex 08, France; Amaxa GmbH, Nattermannallee 1, 50829 Cologne, Germany; and Experimental Rheumatology, Charit\u00e9, Humboldt University, Schumannstrasse 21-22, 10117 Berlin, Germany"}]},{"given":"Zhao-Qi","family":"Wang","sequence":"additional","affiliation":[{"name":"German Rheumatism Research Center, Schumannstrasse 21-22, 10117 Berlin, Germany; Institute of Human Genetics, Charit\u00e9 Medical University, Augustenburger Platz 1, 13353 Berlin, Germany; International Agency for Research on Cancer, Lyon 150 Cours Albert Thomas, 69372 Lyon Cedex 08, France; Amaxa GmbH, Nattermannallee 1, 50829 Cologne, Germany; and Experimental Rheumatology, Charit\u00e9, Humboldt University, Schumannstrasse 21-22, 10117 Berlin, Germany"}]},{"given":"Karl","family":"Sperling","sequence":"additional","affiliation":[{"name":"German Rheumatism Research Center, Schumannstrasse 21-22, 10117 Berlin, Germany; Institute of Human Genetics, Charit\u00e9 Medical University, Augustenburger Platz 1, 13353 Berlin, Germany; International Agency for Research on Cancer, Lyon 150 Cours Albert Thomas, 69372 Lyon Cedex 08, France; Amaxa GmbH, Nattermannallee 1, 50829 Cologne, Germany; and Experimental Rheumatology, Charit\u00e9, Humboldt University, Schumannstrasse 21-22, 10117 Berlin, Germany"}]},{"given":"Martin","family":"Digweed","sequence":"additional","affiliation":[{"name":"German Rheumatism Research Center, Schumannstrasse 21-22, 10117 Berlin, Germany; Institute of Human Genetics, Charit\u00e9 Medical University, Augustenburger Platz 1, 13353 Berlin, Germany; International Agency for Research on Cancer, Lyon 150 Cours Albert Thomas, 69372 Lyon Cedex 08, France; Amaxa GmbH, Nattermannallee 1, 50829 Cologne, Germany; and Experimental Rheumatology, Charit\u00e9, Humboldt University, Schumannstrasse 21-22, 10117 Berlin, Germany"}]},{"given":"Andreas","family":"Radbruch","sequence":"additional","affiliation":[{"name":"German Rheumatism Research Center, Schumannstrasse 21-22, 10117 Berlin, Germany; Institute of Human Genetics, Charit\u00e9 Medical University, Augustenburger Platz 1, 13353 Berlin, Germany; International Agency for Research on Cancer, Lyon 150 Cours Albert Thomas, 69372 Lyon Cedex 08, France; Amaxa GmbH, Nattermannallee 1, 50829 Cologne, Germany; and Experimental Rheumatology, Charit\u00e9, Humboldt University, Schumannstrasse 21-22, 10117 Berlin, Germany"}]}],"member":"341","published-online":{"date-parts":[[2005,1,24]]},"reference":[{"key":"e_1_3_2_1_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(00)81174-5"},{"key":"e_1_3_2_2_2","doi-asserted-by":"publisher","DOI":"10.1038\/nature01125"},{"key":"e_1_3_2_3_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0960-9822(01)00019-7"},{"key":"e_1_3_2_4_2","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/25.15.2985"},{"key":"e_1_3_2_5_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.96.13.7376"},{"key":"e_1_3_2_6_2","doi-asserted-by":"publisher","DOI":"10.1038\/86920"},{"key":"e_1_3_2_7_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M308705200"},{"key":"e_1_3_2_8_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0198-8859(01)00345-7"},{"key":"e_1_3_2_9_2","doi-asserted-by":"publisher","DOI":"10.1002\/1521-4141(200205)32:5<1300::AID-IMMU1300>3.0.CO;2-L"},{"key":"e_1_3_2_10_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M312796200"},{"key":"e_1_3_2_11_2","doi-asserted-by":"publisher","DOI":"10.1093\/emboj\/21.6.1447"},{"key":"e_1_3_2_12_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0960-9822(02)00763-7"},{"key":"e_1_3_2_13_2","doi-asserted-by":"publisher","DOI":"10.1038\/414660a"},{"key":"e_1_3_2_14_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddh278"},{"key":"e_1_3_2_15_2","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/25.6.1317"},{"key":"e_1_3_2_16_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.032068699"},{"key":"e_1_3_2_17_2","first-page":"149","volume":"271","year":"2004","unstructured":"Kracker, S. & Radbruch, A. 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