{"id":"https://openalex.org/W7221084902","doi":"https://doi.org/10.64898/2026.10.02.756202","title":"A PATHOGENIC NFKB2 VARIANT CAUSES COMBINED IMMUNODEFICIENCY WITH THYMIC AND PERIPHERAL LYMPHOID DEFECTS IN RATS","display_name":"A PATHOGENIC NFKB2 VARIANT CAUSES COMBINED IMMUNODEFICIENCY WITH THYMIC AND PERIPHERAL LYMPHOID DEFECTS IN RATS","publication_year":2026,"publication_date":"2026-10-08","ids":{"openalex":"https://openalex.org/W7221084902","doi":"https://doi.org/10.64898/2026.10.02.756202"},"language":"en","primary_location":{"id":"doi:10.64898/2026.10.02.756202","doi":"https://doi.org/10.64898/2026.10.02.756202","is_oa":true,"landing_page_url":"https://doi.org/10.64898/2026.10.02.756202","pdf_url":"https://www.biorxiv.org/content/biorxiv/early/2026/10/08/2026.10.02.756202.full.pdf","source":{"id":"https://openalex.org/S4306402567","display_name":"bioRxiv (Cold Spring Harbor Laboratory)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I2750212522","host_organization_name":"Cold Spring Harbor Laboratory","host_organization_lineage":["https://openalex.org/I2750212522"],"host_organization_lineage_names":[],"type":"repository"},"license":"cc-by-nc-nd","license_id":"https://openalex.org/licenses/cc-by-nc-nd","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"bioRxiv","raw_type":"posted-content"},"volume":null,"type":"preprint","is_traditional":true,"indexed_in":["crossref"],"open_access":{"is_oa":true,"oa_status":"green","oa_url":"https://www.biorxiv.org/content/biorxiv/early/2026/10/08/2026.10.02.756202.full.pdf","any_repository_has_fulltext":true},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5065006567","display_name":"S\u00e9verine Remy","orcid":"https://orcid.org/0000-0002-5241-880X","observed_orcids":["https://orcid.org/0000-0002-5241-880X"]},"institutions":[{"id":"https://openalex.org/I154526488","display_name":"Inserm","ror":"https://ror.org/02vjkv261","country_code":"FR","type":"government","lineage":["https://openalex.org/I154526488"]},{"id":"https://openalex.org/I3019204325","display_name":"Centre Hospitalier Universitaire de Nantes","ror":"https://ror.org/05c1qsg97","country_code":"FR","type":"healthcare","lineage":["https://openalex.org/I3019204325"]},{"id":"https://openalex.org/I4392021198","display_name":"Center for Research in Transplantation and Translational Immunology","ror":"https://ror.org/01165k395","country_code":"FR","type":"facility","lineage":["https://openalex.org/I154526488","https://openalex.org/I4392021198","https://openalex.org/I97188460"]},{"id":"https://openalex.org/I97188460","display_name":"Nantes Universit\u00e9","ror":"https://ror.org/03gnr7b55","country_code":"FR","type":"education","lineage":["https://openalex.org/I97188460"]}],"countries":["FR"],"is_corresponding":true,"raw_author_name":"S\u00e9verine Remy","raw_affiliation_strings":["Nantes Universit\u00e9, INSERM, CHU Nantes, Center for Research in Transplantation and Translational Immunology, UMR 1064, F-44000 Nantes, France","Nantes Universit\u00e9, INSERM, CHU Nantes, Center for Research in Transplantation and Translational Immunology, UMR 1064, Transgenic Rats ImmunoPhenomic, F-44000 Nantes, France"],"raw_orcid":"https://orcid.org/0000-0002-5241-880X","affiliations":[{"raw_affiliation_string":"Nantes Universit\u00e9, INSERM, CHU Nantes, Center for Research in Transplantation and Translational Immunology, UMR 1064, F-44000 Nantes, France","institution_ids":["https://openalex.org/I154526488","https://openalex.org/I3019204325","https://openalex.org/I4392021198","https://openalex.org/I97188460"]},{"raw_affiliation_string":"Nantes Universit\u00e9, INSERM, CHU Nantes, Center for Research in Transplantation and Translational Immunology, UMR 1064, Transgenic Rats ImmunoPhenomic, F-44000 Nantes, France","institution_ids":["https://openalex.org/I154526488","https://openalex.org/I3019204325","https://openalex.org/I4392021198","https://openalex.org/I97188460"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5010751986","display_name":"Laurent Tesson","orcid":"https://orcid.org/0000-0002-7317-8921","observed_orcids":["https://orcid.org/0000-0002-7317-8921"]},"institutions":[{"id":"https://openalex.org/I154526488","display_name":"Inserm","ror":"https://ror.org/02vjkv261","country_code":"FR","type":"government","lineage":["https://openalex.org/I154526488"]},{"id":"https://openalex.org/I3019204325","display_name":"Centre Hospitalier Universitaire de Nantes","ror":"https://ror.org/05c1qsg97","country_code":"FR","type":"healthcare","lineage":["https://openalex.org/I3019204325"]},{"id":"https://openalex.org/I4392021198","display_name":"Center for Research in Transplantation and Translational Immunology","ror":"https://ror.org/01165k395","country_code":"FR","type":"facility","lineage":["https://openalex.org/I154526488","https://openalex.org/I4392021198","https://openalex.org/I97188460"]},{"id":"https://openalex.org/I97188460","display_name":"Nantes Universit\u00e9","ror":"https://ror.org/03gnr7b55","country_code":"FR","type":"education","lineage":["https://openalex.org/I97188460"]}],"countries":["FR"],"is_corresponding":false,"raw_author_name":"Laurent Tesson","raw_affiliation_strings":["Nantes Universit\u00e9, INSERM, CHU Nantes, Center for Research in Transplantation and Translational Immunology, UMR 1064, F-44000 Nantes, France","Nantes Universit\u00e9, INSERM, CHU Nantes, Center for Research in Transplantation and Translational Immunology, UMR 1064, Transgenic Rats ImmunoPhenomic, F-44000 Nantes, France"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Nantes Universit\u00e9, INSERM, CHU Nantes, Center for Research in Transplantation and Translational Immunology, UMR 1064, F-44000 Nantes, France","institution_ids":["https://openalex.org/I154526488","https://openalex.org/I3019204325","https://openalex.org/I4392021198","https://openalex.org/I97188460"]},{"raw_affiliation_string":"Nantes Universit\u00e9, INSERM, CHU Nantes, Center for Research in Transplantation and Translational Immunology, UMR 1064, Transgenic Rats ImmunoPhenomic, F-44000 Nantes, France","institution_ids":["https://openalex.org/I154526488","https://openalex.org/I3019204325","https://openalex.org/I4392021198","https://openalex.org/I97188460"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5075580138","display_name":"Claire Usal","orcid":"https://orcid.org/0000-0003-3913-202X","observed_orcids":["https://orcid.org/0000-0003-3913-202X"]},"institutions":[{"id":"https://openalex.org/I154526488","display_name":"Inserm","ror":"https://ror.org/02vjkv261","country_code":"FR","type":"government","lineage":["https://openalex.org/I154526488"]},{"id":"https://openalex.org/I3019204325","display_name":"Centre Hospitalier Universitaire de Nantes","ror":"https://ror.org/05c1qsg97","country_code":"FR","type":"healthcare","lineage":["https://openalex.org/I3019204325"]},{"id":"https://openalex.org/I4392021198","display_name":"Center for Research in Transplantation and Translational Immunology","ror":"https://ror.org/01165k395","country_code":"FR","type":"facility","lineage":["https://openalex.org/I154526488","https://openalex.org/I4392021198","https://openalex.org/I97188460"]},{"id":"https://openalex.org/I97188460","display_name":"Nantes Universit\u00e9","ror":"https://ror.org/03gnr7b55","country_code":"FR","type":"education","lineage":["https://openalex.org/I97188460"]}],"countries":["FR"],"is_corresponding":false,"raw_author_name":"Claire Usal","raw_affiliation_strings":["Nantes Universit\u00e9, INSERM, CHU Nantes, Center for Research in Transplantation and Translational Immunology, UMR 1064, F-44000 Nantes, France","Nantes Universit\u00e9, INSERM, CHU Nantes, Center for Research in Transplantation and Translational Immunology, UMR 1064, Transgenic Rats ImmunoPhenomic, F-44000 Nantes, France"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Nantes Universit\u00e9, INSERM, CHU Nantes, Center for Research in Transplantation and Translational Immunology, UMR 1064, F-44000 Nantes, France","institution_ids":["https://openalex.org/I154526488","https://openalex.org/I3019204325","https://openalex.org/I4392021198","https://openalex.org/I97188460"]},{"raw_affiliation_string":"Nantes Universit\u00e9, INSERM, CHU Nantes, Center for Research in Transplantation and Translational Immunology, UMR 1064, Transgenic Rats ImmunoPhenomic, F-44000 Nantes, France","institution_ids":["https://openalex.org/I154526488","https://openalex.org/I3019204325","https://openalex.org/I4392021198","https://openalex.org/I97188460"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5138969354","display_name":"Marie Massoda","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I154526488","display_name":"Inserm","ror":"https://ror.org/02vjkv261","country_code":"FR","type":"government","lineage":["https://openalex.org/I154526488"]},{"id":"https://openalex.org/I3019204325","display_name":"Centre Hospitalier Universitaire de Nantes","ror":"https://ror.org/05c1qsg97","country_code":"FR","type":"healthcare","lineage":["https://openalex.org/I3019204325"]},{"id":"https://openalex.org/I4392021198","display_name":"Center for Research in Transplantation and Translational Immunology","ror":"https://ror.org/01165k395","country_code":"FR","type":"facility","lineage":["https://openalex.org/I154526488","https://openalex.org/I4392021198","https://openalex.org/I97188460"]},{"id":"https://openalex.org/I97188460","display_name":"Nantes Universit\u00e9","ror":"https://ror.org/03gnr7b55","country_code":"FR","type":"education","lineage":["https://openalex.org/I97188460"]}],"countries":["FR"],"is_corresponding":false,"raw_author_name":"Marie Massoda","raw_affiliation_strings":["Nantes Universit\u00e9, INSERM, CHU Nantes, Center for Research in Transplantation and Translational Immunology, UMR 1064, F-44000 Nantes, France"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Nantes Universit\u00e9, INSERM, CHU Nantes, Center for Research in Transplantation and Translational Immunology, UMR 1064, F-44000 Nantes, France","institution_ids":["https://openalex.org/I154526488","https://openalex.org/I3019204325","https://openalex.org/I4392021198","https://openalex.org/I97188460"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5102714754","display_name":"S\u00e9verine M\u00e9noret","orcid":"https://orcid.org/0000-0002-3234-1382","observed_orcids":["https://orcid.org/0000-0002-3234-1382","https://orcid.org/0000-0002-9200-4927"]},"institutions":[{"id":"https://openalex.org/I1294671590","display_name":"Centre National de la Recherche Scientifique","ror":"https://ror.org/02feahw73","country_code":"FR","type":"government","lineage":["https://openalex.org/I1294671590"]},{"id":"https://openalex.org/I154526488","display_name":"Inserm","ror":"https://ror.org/02vjkv261","country_code":"FR","type":"government","lineage":["https://openalex.org/I154526488"]},{"id":"https://openalex.org/I3019204325","display_name":"Centre Hospitalier Universitaire de Nantes","ror":"https://ror.org/05c1qsg97","country_code":"FR","type":"healthcare","lineage":["https://openalex.org/I3019204325"]},{"id":"https://openalex.org/I4392021198","display_name":"Center for Research in Transplantation and Translational Immunology","ror":"https://ror.org/01165k395","country_code":"FR","type":"facility","lineage":["https://openalex.org/I154526488","https://openalex.org/I4392021198","https://openalex.org/I97188460"]},{"id":"https://openalex.org/I4400008889","display_name":"UMS BioCore","ror":"https://ror.org/04r5nwv94","country_code":"FR","type":"facility","lineage":["https://openalex.org/I1294671590","https://openalex.org/I154526488","https://openalex.org/I3019204325","https://openalex.org/I4210096427","https://openalex.org/I4400008889","https://openalex.org/I97188460"]},{"id":"https://openalex.org/I4400009061","display_name":"Structure F\u00e9d\u00e9rative de Recherche Bonamy","ror":"https://ror.org/03p6ach34","country_code":"FR","type":"facility","lineage":["https://openalex.org/I154526488","https://openalex.org/I4392021198","https://openalex.org/I4400009061","https://openalex.org/I97188460"]},{"id":"https://openalex.org/I97188460","display_name":"Nantes Universit\u00e9","ror":"https://ror.org/03gnr7b55","country_code":"FR","type":"education","lineage":["https://openalex.org/I97188460"]}],"countries":["FR"],"is_corresponding":false,"raw_author_name":"S\u00e9verine M\u00e9noret","raw_affiliation_strings":["Nantes Universit\u00e9, CHU Nantes, CNRS, INSERM, BioCore, US16, SFR Bonamy, F-44000 Nantes, France","Nantes Universit\u00e9, INSERM, CHU Nantes, Center for Research in Transplantation and Translational Immunology, UMR 1064, F-44000 Nantes, France","Nantes Universit\u00e9, INSERM, CHU Nantes, Center for Research in Transplantation and Translational Immunology, UMR 1064, Transgenic Rats ImmunoPhenomic, F-44000 Nantes, France"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Nantes Universit\u00e9, CHU Nantes, CNRS, INSERM, BioCore, US16, SFR Bonamy, F-44000 Nantes, France","institution_ids":["https://openalex.org/I1294671590","https://openalex.org/I154526488","https://openalex.org/I3019204325","https://openalex.org/I4400008889","https://openalex.org/I4400009061","https://openalex.org/I97188460"]},{"raw_affiliation_string":"Nantes Universit\u00e9, INSERM, CHU Nantes, Center for Research in Transplantation and Translational Immunology, UMR 1064, F-44000 Nantes, France","institution_ids":["https://openalex.org/I154526488","https://openalex.org/I3019204325","https://openalex.org/I4392021198","https://openalex.org/I97188460"]},{"raw_affiliation_string":"Nantes Universit\u00e9, INSERM, CHU Nantes, Center for Research in Transplantation and Translational Immunology, UMR 1064, Transgenic Rats ImmunoPhenomic, F-44000 Nantes, France","institution_ids":["https://openalex.org/I154526488","https://openalex.org/I3019204325","https://openalex.org/I4392021198","https://openalex.org/I97188460"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5039237490","display_name":"Irina Proekt","orcid":"https://orcid.org/0000-0003-3818-1102","observed_orcids":["https://orcid.org/0000-0003-3818-1102"]},"institutions":[{"id":"https://openalex.org/I180670191","display_name":"University of California, San Francisco","ror":"https://ror.org/043mz5j54","country_code":"US","type":"education","lineage":["https://openalex.org/I180670191"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Irina Proekt","raw_affiliation_strings":["UCSF Diabetes Center, University of California, San Francisco, United States"],"raw_orcid":"https://orcid.org/0000-0003-3818-1102","affiliations":[{"raw_affiliation_string":"UCSF Diabetes Center, University of California, San Francisco, United States","institution_ids":["https://openalex.org/I180670191"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5135840641","display_name":"David Yu","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I180670191","display_name":"University of California, San Francisco","ror":"https://ror.org/043mz5j54","country_code":"US","type":"education","lineage":["https://openalex.org/I180670191"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"David Yu","raw_affiliation_strings":["UCSF Diabetes Center, University of California, San Francisco, United States"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"UCSF Diabetes Center, University of California, San Francisco, United States","institution_ids":["https://openalex.org/I180670191"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5050210259","display_name":"Axel You","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I154526488","display_name":"Inserm","ror":"https://ror.org/02vjkv261","country_code":"FR","type":"government","lineage":["https://openalex.org/I154526488"]},{"id":"https://openalex.org/I3019204325","display_name":"Centre Hospitalier Universitaire de Nantes","ror":"https://ror.org/05c1qsg97","country_code":"FR","type":"healthcare","lineage":["https://openalex.org/I3019204325"]},{"id":"https://openalex.org/I4392021198","display_name":"Center for Research in Transplantation and Translational Immunology","ror":"https://ror.org/01165k395","country_code":"FR","type":"facility","lineage":["https://openalex.org/I154526488","https://openalex.org/I4392021198","https://openalex.org/I97188460"]},{"id":"https://openalex.org/I97188460","display_name":"Nantes Universit\u00e9","ror":"https://ror.org/03gnr7b55","country_code":"FR","type":"education","lineage":["https://openalex.org/I97188460"]}],"countries":["FR"],"is_corresponding":false,"raw_author_name":"Axel You","raw_affiliation_strings":["Nantes Universit\u00e9, INSERM, CHU Nantes, Center for Research in Transplantation and Translational Immunology, UMR 1064, F-44000 Nantes, France","Nantes Universit\u00e9, INSERM, CHU Nantes, Center for Research in Transplantation and Translational Immunology, UMR 1064, Transgenic Rats ImmunoPhenomic, F-44000 Nantes, France"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Nantes Universit\u00e9, INSERM, CHU Nantes, Center for Research in Transplantation and Translational Immunology, UMR 1064, F-44000 Nantes, France","institution_ids":["https://openalex.org/I154526488","https://openalex.org/I3019204325","https://openalex.org/I4392021198","https://openalex.org/I97188460"]},{"raw_affiliation_string":"Nantes Universit\u00e9, INSERM, CHU Nantes, Center for Research in Transplantation and Translational Immunology, UMR 1064, Transgenic Rats ImmunoPhenomic, F-44000 Nantes, France","institution_ids":["https://openalex.org/I154526488","https://openalex.org/I3019204325","https://openalex.org/I4392021198","https://openalex.org/I97188460"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5090415277","display_name":"Matthieu Giraud","orcid":"https://orcid.org/0000-0002-1208-9677","observed_orcids":["https://orcid.org/0000-0002-1208-9677"]},"institutions":[{"id":"https://openalex.org/I154526488","display_name":"Inserm","ror":"https://ror.org/02vjkv261","country_code":"FR","type":"government","lineage":["https://openalex.org/I154526488"]},{"id":"https://openalex.org/I3019204325","display_name":"Centre Hospitalier Universitaire de Nantes","ror":"https://ror.org/05c1qsg97","country_code":"FR","type":"healthcare","lineage":["https://openalex.org/I3019204325"]},{"id":"https://openalex.org/I4392021198","display_name":"Center for Research in Transplantation and Translational Immunology","ror":"https://ror.org/01165k395","country_code":"FR","type":"facility","lineage":["https://openalex.org/I154526488","https://openalex.org/I4392021198","https://openalex.org/I97188460"]},{"id":"https://openalex.org/I97188460","display_name":"Nantes Universit\u00e9","ror":"https://ror.org/03gnr7b55","country_code":"FR","type":"education","lineage":["https://openalex.org/I97188460"]}],"countries":["FR"],"is_corresponding":false,"raw_author_name":"Matthieu Giraud","raw_affiliation_strings":["Nantes Universit\u00e9, INSERM, CHU Nantes, Center for Research in Transplantation and Translational Immunology, UMR 1064, F-44000 Nantes, France","Nantes Universit\u00e9, INSERM, CHU Nantes, Center for Research in Transplantation and Translational Immunology, UMR 1064, Transgenic Rats ImmunoPhenomic, F-44000 Nantes, France"],"raw_orcid":"https://orcid.org/0000-0002-1208-9677","affiliations":[{"raw_affiliation_string":"Nantes Universit\u00e9, INSERM, CHU Nantes, Center for Research in Transplantation and Translational Immunology, UMR 1064, F-44000 Nantes, France","institution_ids":["https://openalex.org/I154526488","https://openalex.org/I3019204325","https://openalex.org/I4392021198","https://openalex.org/I97188460"]},{"raw_affiliation_string":"Nantes Universit\u00e9, INSERM, CHU Nantes, Center for Research in Transplantation and Translational Immunology, UMR 1064, Transgenic Rats ImmunoPhenomic, F-44000 Nantes, France","institution_ids":["https://openalex.org/I154526488","https://openalex.org/I3019204325","https://openalex.org/I4392021198","https://openalex.org/I97188460"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5091086178","display_name":"Mark Stuart Anderson","orcid":"https://orcid.org/0000-0002-3093-4758","observed_orcids":["https://orcid.org/0000-0002-3093-4758"]},"institutions":[{"id":"https://openalex.org/I180670191","display_name":"University of California, San Francisco","ror":"https://ror.org/043mz5j54","country_code":"US","type":"education","lineage":["https://openalex.org/I180670191"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Mark S. Anderson","raw_affiliation_strings":["UCSF Diabetes Center, University of California, San Francisco, United States"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"UCSF Diabetes Center, University of California, San Francisco, United States","institution_ids":["https://openalex.org/I180670191"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5071374620","display_name":"Ignacio Aneg\u00f3n","orcid":"https://orcid.org/0000-0001-8700-5645","observed_orcids":["https://orcid.org/0000-0001-8700-5645"]},"institutions":[{"id":"https://openalex.org/I154526488","display_name":"Inserm","ror":"https://ror.org/02vjkv261","country_code":"FR","type":"government","lineage":["https://openalex.org/I154526488"]},{"id":"https://openalex.org/I3019204325","display_name":"Centre Hospitalier Universitaire de Nantes","ror":"https://ror.org/05c1qsg97","country_code":"FR","type":"healthcare","lineage":["https://openalex.org/I3019204325"]},{"id":"https://openalex.org/I4392021198","display_name":"Center for Research in Transplantation and Translational Immunology","ror":"https://ror.org/01165k395","country_code":"FR","type":"facility","lineage":["https://openalex.org/I154526488","https://openalex.org/I4392021198","https://openalex.org/I97188460"]},{"id":"https://openalex.org/I97188460","display_name":"Nantes Universit\u00e9","ror":"https://ror.org/03gnr7b55","country_code":"FR","type":"education","lineage":["https://openalex.org/I97188460"]}],"countries":["FR"],"is_corresponding":false,"raw_author_name":"Ignacio Anegon","raw_affiliation_strings":["Nantes Universit\u00e9, INSERM, CHU Nantes, Center for Research in Transplantation and Translational Immunology, UMR 1064, F-44000 Nantes, France","Nantes Universit\u00e9, INSERM, CHU Nantes, Center for Research in Transplantation and Translational Immunology, UMR 1064, Transgenic Rats ImmunoPhenomic, F-44000 Nantes, France"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Nantes Universit\u00e9, INSERM, CHU Nantes, Center for Research in Transplantation and Translational Immunology, UMR 1064, F-44000 Nantes, France","institution_ids":["https://openalex.org/I154526488","https://openalex.org/I3019204325","https://openalex.org/I4392021198","https://openalex.org/I97188460"]},{"raw_affiliation_string":"Nantes Universit\u00e9, INSERM, CHU Nantes, Center for Research in Transplantation and Translational Immunology, UMR 1064, Transgenic Rats ImmunoPhenomic, F-44000 Nantes, France","institution_ids":["https://openalex.org/I154526488","https://openalex.org/I3019204325","https://openalex.org/I4392021198","https://openalex.org/I97188460"]}]}],"institutions":[],"countries_distinct_count":2,"institutions_distinct_count":8,"corresponding_author_ids":["https://openalex.org/A5065006567"],"corresponding_institution_ids":["https://openalex.org/I154526488","https://openalex.org/I3019204325","https://openalex.org/I4392021198","https://openalex.org/I97188460"],"apc_list":null,"apc_paid":null,"fwci":null,"has_fulltext":true,"cited_by_count":0,"citation_normalized_percentile":null,"cited_by_percentile_year":null,"biblio":{"volume":null,"issue":null,"first_page":null,"last_page":null},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T11725","display_name":"Immunodeficiency and Autoimmune Disorders","score":0.9717000126838684,"subfield":{"id":"https://openalex.org/subfields/2403","display_name":"Immunology"},"field":{"id":"https://openalex.org/fields/24","display_name":"Immunology and Microbiology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},"topics":[{"id":"https://openalex.org/T11725","display_name":"Immunodeficiency and Autoimmune Disorders","score":0.9717000126838684,"subfield":{"id":"https://openalex.org/subfields/2403","display_name":"Immunology"},"field":{"id":"https://openalex.org/fields/24","display_name":"Immunology and Microbiology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},{"id":"https://openalex.org/T10031","display_name":"T-cell and B-cell Immunology","score":0.02239999920129776,"subfield":{"id":"https://openalex.org/subfields/2403","display_name":"Immunology"},"field":{"id":"https://openalex.org/fields/24","display_name":"Immunology and Microbiology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},{"id":"https://openalex.org/T11591","display_name":"NF-\u03baB Signaling Pathways","score":0.002899999963119626,"subfield":{"id":"https://openalex.org/subfields/1306","display_name":"Cancer Research"},"field":{"id":"https://openalex.org/fields/13","display_name":"Biochemistry, Genetics and Molecular Biology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}}],"keywords":[{"id":"https://openalex.org/keywords/nfkb2","display_name":"NFKB2","score":0.9879999756813049},{"id":"https://openalex.org/keywords/non-canonical-nf-kappab-signaling","display_name":"non-canonical NF-\u03baB signaling","score":0.9539999961853027},{"id":"https://openalex.org/keywords/combined-immunodeficiency","display_name":"combined immunodeficiency","score":0.890999972820282},{"id":"https://openalex.org/keywords/common-variable-immunodeficiency","display_name":"common variable immunodeficiency","score":0.9480000138282776},{"id":"https://openalex.org/keywords/thymic-epithelial-cells","display_name":"thymic epithelial cells","score":0.7369999885559082},{"id":"https://openalex.org/keywords/autoimmune-regulator","display_name":"AIRE","score":0.8539999723434448},{"id":"https://openalex.org/keywords/regulatory-t-cells","display_name":"regulatory T cells","score":0.8259999752044678},{"id":"https://openalex.org/keywords/t-cell-development","display_name":"T cell development","score":0.7200000286102295},{"id":"https://openalex.org/keywords/marginal-zone","display_name":"marginal zone","score":0.6240000128746033},{"id":"https://openalex.org/keywords/t-cell-homeostasis","display_name":"T cell homeostasis","score":0.8600000143051147},{"id":"https://openalex.org/keywords/inducible-t-cell-costimulator","display_name":"ICOS","score":0.8859999775886536},{"id":"https://openalex.org/keywords/b-cell-maturation","display_name":"B cell maturation","score":0.9599999785423279},{"id":"https://openalex.org/keywords/humoral-immunity","display_name":"humoral immunity","score":0.7279999852180481},{"id":"https://openalex.org/keywords/autoimmunity","display_name":"autoimmunity","score":0.8629999756813049},{"id":"https://openalex.org/keywords/rat-model","display_name":"rat model","score":0.7850000262260437},{"id":"https://openalex.org/keywords/nonsense-mutation","display_name":"nonsense mutation","score":0.6240000128746033}],"concepts":[{"id":"https://openalex.org/C86803240","wikidata":"https://www.wikidata.org/wiki/Q420","display_name":"Biology","level":0,"score":0.6988999843597412},{"id":"https://openalex.org/C203014093","wikidata":"https://www.wikidata.org/wiki/Q101929","display_name":"Immunology","level":1,"score":0.6944000124931335},{"id":"https://openalex.org/C2777607303","wikidata":"https://www.wikidata.org/wiki/Q641307","display_name":"Immunodeficiency","level":3,"score":0.6891000270843506},{"id":"https://openalex.org/C8891405","wikidata":"https://www.wikidata.org/wiki/Q1059","display_name":"Immune system","level":2,"score":0.6593000292778015},{"id":"https://openalex.org/C2776433325","wikidata":"https://www.wikidata.org/wiki/Q1472818","display_name":"Common variable immunodeficiency","level":3,"score":0.593500018119812},{"id":"https://openalex.org/C2780130043","wikidata":"https://www.wikidata.org/wiki/Q192360","display_name":"Autoimmunity","level":3,"score":0.5767999887466431},{"id":"https://openalex.org/C2776566471","wikidata":"https://www.wikidata.org/wiki/Q2872459","display_name":"Autoimmune regulator","level":4,"score":0.570900022983551},{"id":"https://openalex.org/C127716648","wikidata":"https://www.wikidata.org/wiki/Q104053","display_name":"Phenotype","level":3,"score":0.5432999730110168},{"id":"https://openalex.org/C2778954852","wikidata":"https://www.wikidata.org/wiki/Q7799635","display_name":"Thymocyte","level":4,"score":0.5418000221252441},{"id":"https://openalex.org/C181152851","wikidata":"https://www.wikidata.org/wiki/Q712604","display_name":"Lymphatic system","level":2,"score":0.5004000067710876},{"id":"https://openalex.org/C163764329","wikidata":"https://www.wikidata.org/wiki/Q785022","display_name":"Autoantibody","level":3,"score":0.4456999897956848},{"id":"https://openalex.org/C2779468541","wikidata":"https://www.wikidata.org/wiki/Q1334408","display_name":"Severe combined immunodeficiency","level":3,"score":0.40380001068115234},{"id":"https://openalex.org/C96926380","wikidata":"https://www.wikidata.org/wiki/Q1737968","display_name":"Germinal center","level":4,"score":0.4034000039100647},{"id":"https://openalex.org/C2779019163","wikidata":"https://www.wikidata.org/wiki/Q3043160","display_name":"Primary immunodeficiency","level":3,"score":0.39079999923706055},{"id":"https://openalex.org/C113646796","wikidata":"https://www.wikidata.org/wiki/Q50263283","display_name":"Recombination-activating gene","level":4,"score":0.35989999771118164},{"id":"https://openalex.org/C7386963","wikidata":"https://www.wikidata.org/wiki/Q3954859","display_name":"Negative selection","level":4,"score":0.3594000041484833},{"id":"https://openalex.org/C2778830056","wikidata":"https://www.wikidata.org/wiki/Q606766","display_name":"Central tolerance","level":4,"score":0.3589000105857849},{"id":"https://openalex.org/C2780931953","wikidata":"https://www.wikidata.org/wiki/Q9371","display_name":"Spleen","level":2,"score":0.34880000352859497},{"id":"https://openalex.org/C193419808","wikidata":"https://www.wikidata.org/wiki/Q1645075","display_name":"Acquired immune system","level":3,"score":0.34689998626708984},{"id":"https://openalex.org/C2779921676","wikidata":"https://www.wikidata.org/wiki/Q6760434","display_name":"Marginal zone","level":4,"score":0.34619998931884766},{"id":"https://openalex.org/C2779341262","wikidata":"https://www.wikidata.org/wiki/Q182581","display_name":"Immunity","level":3,"score":0.3447999954223633},{"id":"https://openalex.org/C2777761686","wikidata":"https://www.wikidata.org/wiki/Q715347","display_name":"Lymphocyte","level":2,"score":0.3382999897003174},{"id":"https://openalex.org/C2910905871","wikidata":"https://www.wikidata.org/wiki/Q641307","display_name":"Immunodeficiency Syndrome","level":4,"score":0.334199994802475},{"id":"https://openalex.org/C2779720271","wikidata":"https://www.wikidata.org/wiki/Q179422","display_name":"Lymph","level":2,"score":0.32260000705718994},{"id":"https://openalex.org/C2780194698","wikidata":"https://www.wikidata.org/wiki/Q6005429","display_name":"Immune dysregulation","level":3,"score":0.31869998574256897},{"id":"https://openalex.org/C2780648854","wikidata":"https://www.wikidata.org/wiki/Q2745824","display_name":"Immunopathology","level":2,"score":0.3140999972820282},{"id":"https://openalex.org/C104317684","wikidata":"https://www.wikidata.org/wiki/Q7187","display_name":"Gene","level":2,"score":0.2987000048160553},{"id":"https://openalex.org/C2780007613","wikidata":"https://www.wikidata.org/wiki/Q546523","display_name":"Bone marrow","level":2,"score":0.2962999939918518},{"id":"https://openalex.org/C2781376893","wikidata":"https://www.wikidata.org/wiki/Q18031023","display_name":"RAG2","level":4,"score":0.2671999931335449},{"id":"https://openalex.org/C2778453870","wikidata":"https://www.wikidata.org/wiki/Q188930","display_name":"B cell","level":3,"score":0.2669999897480011},{"id":"https://openalex.org/C2777016536","wikidata":"https://www.wikidata.org/wiki/Q7168713","display_name":"Peripheral tolerance","level":4,"score":0.26660001277923584},{"id":"https://openalex.org/C2992881475","wikidata":"https://www.wikidata.org/wiki/Q193529","display_name":"T lymphocyte","level":3,"score":0.2603999972343445},{"id":"https://openalex.org/C13514818","wikidata":"https://www.wikidata.org/wiki/Q5552256","display_name":"Germline mutation","level":4,"score":0.2572999894618988},{"id":"https://openalex.org/C2776090121","wikidata":"https://www.wikidata.org/wiki/Q193529","display_name":"T cell","level":3,"score":0.25609999895095825},{"id":"https://openalex.org/C136449434","wikidata":"https://www.wikidata.org/wiki/Q428253","display_name":"Innate immune system","level":3,"score":0.2529999911785126},{"id":"https://openalex.org/C501734568","wikidata":"https://www.wikidata.org/wiki/Q42918","display_name":"Mutation","level":3,"score":0.25270000100135803}],"mesh":[],"locations_count":1,"locations":[{"id":"doi:10.64898/2026.10.02.756202","doi":"https://doi.org/10.64898/2026.10.02.756202","is_oa":true,"landing_page_url":"https://doi.org/10.64898/2026.10.02.756202","pdf_url":"https://www.biorxiv.org/content/biorxiv/early/2026/10/08/2026.10.02.756202.full.pdf","source":{"id":"https://openalex.org/S4306402567","display_name":"bioRxiv (Cold Spring Harbor Laboratory)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I2750212522","host_organization_name":"Cold Spring Harbor Laboratory","host_organization_lineage":["https://openalex.org/I2750212522"],"host_organization_lineage_names":[],"type":"repository"},"license":"cc-by-nc-nd","license_id":"https://openalex.org/licenses/cc-by-nc-nd","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"bioRxiv","raw_type":"posted-content"}],"best_oa_location":{"id":"doi:10.64898/2026.10.02.756202","doi":"https://doi.org/10.64898/2026.10.02.756202","is_oa":true,"landing_page_url":"https://doi.org/10.64898/2026.10.02.756202","pdf_url":"https://www.biorxiv.org/content/biorxiv/early/2026/10/08/2026.10.02.756202.full.pdf","source":{"id":"https://openalex.org/S4306402567","display_name":"bioRxiv (Cold Spring Harbor Laboratory)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I2750212522","host_organization_name":"Cold Spring Harbor Laboratory","host_organization_lineage":["https://openalex.org/I2750212522"],"host_organization_lineage_names":[],"type":"repository"},"license":"cc-by-nc-nd","license_id":"https://openalex.org/licenses/cc-by-nc-nd","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"bioRxiv","raw_type":"posted-content"},"sustainable_development_goals":[{"score":0.9922999739646912,"display_name":"Good health and well-being","id":"https://metadata.un.org/sdg/3"}],"study_designs":[],"awards":[],"funders":[],"has_content":{"pdf":true,"grobid_xml":true},"content_urls":{"pdf":"https://content.openalex.org/works/W7221084902.pdf","grobid_xml":"https://content.openalex.org/works/W7221084902.grobid-xml"},"referenced_works_count":49,"referenced_works":["https://openalex.org/W1558901013","https://openalex.org/W1564210308","https://openalex.org/W1573828236","https://openalex.org/W1601981272","https://openalex.org/W1668508243","https://openalex.org/W1832300219","https://openalex.org/W1904721555","https://openalex.org/W1919878195","https://openalex.org/W1922016811","https://openalex.org/W1970444129","https://openalex.org/W1984508378","https://openalex.org/W1987531976","https://openalex.org/W1988974789","https://openalex.org/W2011680474","https://openalex.org/W2015301752","https://openalex.org/W2018718597","https://openalex.org/W2033810895","https://openalex.org/W2035099738","https://openalex.org/W2046749021","https://openalex.org/W2068467005","https://openalex.org/W2073145905","https://openalex.org/W2074787353","https://openalex.org/W2086697922","https://openalex.org/W2098912300","https://openalex.org/W2103944436","https://openalex.org/W2138486090","https://openalex.org/W2139771438","https://openalex.org/W2147507133","https://openalex.org/W2158992345","https://openalex.org/W2172579093","https://openalex.org/W2257197315","https://openalex.org/W2528688876","https://openalex.org/W2586071800","https://openalex.org/W2605044133","https://openalex.org/W2610586533","https://openalex.org/W2620769476","https://openalex.org/W2743432286","https://openalex.org/W2768098631","https://openalex.org/W2810749078","https://openalex.org/W2923669061","https://openalex.org/W2968498407","https://openalex.org/W2975094656","https://openalex.org/W3093745818","https://openalex.org/W3145413671","https://openalex.org/W3153384101","https://openalex.org/W4311587635","https://openalex.org/W4388488683","https://openalex.org/W4403627606","https://openalex.org/W4404641956"],"related_works":["https://openalex.org/W2923669061","https://openalex.org/W2046749021","https://openalex.org/W7203942974","https://openalex.org/W3077137192","https://openalex.org/W2011680474","https://openalex.org/W2959493656","https://openalex.org/W3087844029","https://openalex.org/W4381735654","https://openalex.org/W2772502727","https://openalex.org/W4251368721"],"abstract_inverted_index":{"Abstract":[0],"The":[1,47,190],"non-canonical":[2,255],"NF-\u03baB":[3],"pathway,":[4],"in":[5,77,95,119,162,243,258],"which":[6],"NFKB2":[7,22,117],"plays":[8],"a":[9,29,67,72,98,211,238],"central":[10],"role,":[11],"is":[12],"critical":[13],"for":[14,250],"lymphoid":[15,150],"organ":[16],"development":[17,142],"and":[18,44,134,143,167,172,181,210,228,262],"immune":[19,52,64,259],"homeostasis.":[20],"Pathogenic":[21],"variants":[23],"have":[24],"been":[25,93],"strongly":[26],"associated":[27,223],"with":[28,97,107,156,196,224],"broad":[30],"spectrum":[31],"of":[32,66,82,101,122,158,169,203,214,231,254],"human":[33],"pathologies,":[34],"notably":[35],"immunodysregulation":[36],"syndromes,":[37],"characterized":[38,62,200],"by":[39,139,201],"recurrent":[40],"infections,":[41],"autoantibody":[42],"production":[43],"endocrine":[45],"abnormalities.":[46],"mechanisms":[48],"underlying":[49],"the":[50,58,63,78,83,123,163,244,252],"resulting":[51],"defects":[53,161],"remain":[54],"incompletely":[55],"understood.":[56],"In":[57],"present":[59],"study,":[60],"we":[61,113],"phenotype":[65,242],"novel":[68],"rat":[69],"model":[70],"harboring":[71],"Nfkb2":[73,87],"non-sense":[74],"mutation":[75],"located":[76],"c-":[79],"terminal":[80],"region":[81],"gene":[84],"(c.2602T>A;":[85],"p.Tyr867*,":[86],"p.Tyr867*":[88],").":[89],"This":[90],"variant":[91],"has":[92],"identified":[94],"patients":[96],"familial":[99],"form":[100],"common":[102],"variable":[103],"immunodeficiency":[104,241],"(CVID)":[105],"presenting":[106],"autoimmunity":[108],"manifestations.":[109],"Using":[110],"this":[111],"model,":[112],"demonstrate":[114],"that":[115],"mutated":[116],"resulted":[118],"profound":[120],"alterations":[121,168],"thymic":[124,131,145],"microenvironment,":[125],"including":[126,177],"reduced":[127,182],"K5":[128],"+":[129,171,174,187,205,216],"medullary":[130],"epithelial":[132],"cells":[133,209],"decreased":[135],"AIRE":[136],"expression,":[137],"accompanied":[138],"altered":[140],"thymocyte":[141],"impaired":[144,178,197,225],"regulatory":[146],"T":[147,188],"populations.":[148],"Peripheral":[149],"organization":[151,261],"was":[152,193],"also":[153],"profoundly":[154],"affected,":[155],"absence":[157,230],"lymph":[159],"nodes,":[160],"splenic":[164],"marginal":[165],"zone,":[166],"CD4":[170,186],"CD8":[173],"T-cell":[175],"homeostasis,":[176],"Treg":[179],"populations":[180],"ICOS":[183],"expression":[184],"on":[185],"cells.":[189,219],"B-cell":[191,198],"compartment":[192],"markedly":[194],"altered,":[195],"maturation,":[199],"accumulation":[202],"IgM":[204],"IgD":[206,215],"\u2212":[207],"transitional":[208],"marked":[212],"reduction":[213],"mature":[217],"B":[218],"These":[220],"abnormalities":[221],"were":[222],"humoral":[226],"immunity":[227],"complete":[229],"circulating":[232],"immunoglobulins.":[233],"Collectively,":[234],"our":[235],"findings":[236],"define":[237],"systemic":[239],"combined":[240],"Nfkb2-mutated":[245],"rat,":[246],"highlighting":[247],"its":[248],"utility":[249],"studying":[251],"role":[253],"NF-kB":[256],"signaling":[257],"system":[260],"adaptative":[263],"immunity.":[264]},"counts_by_year":[],"updated_date":"2026-11-09T13:22:03.289127","created_date":"2026-10-10T00:00:00"}
