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XRCC4\n                    <jats:sup>M61R<\/jats:sup>\n                    protein does not interact with Xlf, thus obliterating XRCC4-Xlf filament formation while preserving the ability to stabilize DNA ligase IV. X4\n                    <jats:sup>M61R<\/jats:sup>\n                    mice, which are DNA repair deficient, phenocopy the\n                    <jats:italic>Nhej1-\/-<\/jats:italic>\n                    (known as\n                    <jats:italic>Xlf<\/jats:italic>\n                    -\/-) setting with a minor impact on the development of the adaptive immune system. The core non-homologous end-joining (NHEJ) DNA repair factor XRCC4 is therefore not mandatory for V(D)J recombination aside from its role in stabilizing DNA ligase IV. In contrast,\n                    <jats:italic>\n                      Xrcc4\n                      <jats:sup>M61R<\/jats:sup>\n                    <\/jats:italic>\n                    mice crossed on\n                    <jats:italic>Paxx-\/-<\/jats:italic>\n                    ,\n                    <jats:italic>Nhej1-\/-<\/jats:italic>\n                    , or\n                    <jats:italic>Atm<\/jats:italic>\n                    -\/- backgrounds are severely immunocompromised, owing to aborted V(D)J recombination as in\n                    <jats:italic>Xlf-Paxx<\/jats:italic>\n                    and\n                    <jats:italic>Xlf-Atm<\/jats:italic>\n                    double Knock Out (DKO) settings. Furthermore, massive apoptosis of post-mitotic neurons causes embryonic lethality of\n                    <jats:italic>\n                      Xrcc4\n                      <jats:sup>M61R<\/jats:sup>\n                      -Nhej1-\/-\n                    <\/jats:italic>\n                    double mutants. These in vivo results reveal new functional interplays between XRCC4 and PAXX, ATM and Xlf in mouse development and provide new insights into the understanding of the clinical manifestations of human\n                    <jats:italic>XRCC4<\/jats:italic>\n                    -deficient condition, in particular its absence of immune deficiency.\n                  <\/jats:p>","DOI":"10.7554\/elife.69353","type":"journal-article","created":{"date-parts":[[2021,9,14]],"date-time":"2021-09-14T08:00:56Z","timestamp":1631606456000},"update-policy":"https:\/\/doi.org\/10.7554\/elife.69353","source":"Crossref","is-referenced-by-count":10,"title":["An XRCC4 mutant mouse, a model for human X4 syndrome, reveals interplays with Xlf, PAXX, and ATM in lymphoid development"],"prefix":"10.7554","volume":"10","author":[{"given":"Benoit","family":"Roch","sequence":"first","affiliation":[{"name":"Universit\u00e9 de Paris, Imagine Institute, Laboratory \u201cGenome Dynamics in the Immune System\u201d, INSERM UMR 1163, F-75015","place":["Paris, France"]},{"name":"Equipe Labellis\u00e9e Ligue Nationale Contre le Cancer, F75015","place":["Paris, France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vincent","family":"Abramowski","sequence":"first","affiliation":[{"name":"Universit\u00e9 de Paris, Imagine Institute, Laboratory \u201cGenome Dynamics in the Immune System\u201d, INSERM UMR 1163, F-75015","place":["Paris, France"]},{"name":"Equipe Labellis\u00e9e Ligue Nationale Contre le Cancer, F75015","place":["Paris, France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olivier","family":"Etienne","sequence":"additional","affiliation":[{"name":"Universit\u00e9 de Paris and Universit\u00e9 Paris-Saclay, Inserm, LRP\/iRCM\/IBFJ CEA, UMR Stabilit\u00e9 G\u00e9n\u00e9tique Cellules Souches et Radiations, F-92265","place":["Fontenay-aux-Roses, France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefania","family":"Musilli","sequence":"additional","affiliation":[{"name":"Universit\u00e9 de Paris, Imagine Institute, Laboratory \u201cGenome Dynamics in the Immune System\u201d, INSERM UMR 1163, F-75015","place":["Paris, France"]},{"name":"Equipe Labellis\u00e9e Ligue Nationale Contre le Cancer, F75015","place":["Paris, France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pierre","family":"David","sequence":"additional","affiliation":[{"name":"Universit\u00e9 de Paris, Imagine Institute, Transgenesis facility, INSERM UMR 1163, F-75015","place":["Paris, France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean-Baptiste","family":"Charbonnier","sequence":"additional","affiliation":[{"name":"Institute for Integrative Biology of the Cell (I2BC), Institute Joliot, CEA, CNRS, Univ. 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