{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T14:15:37Z","timestamp":1786976137289,"version":"build-2736575974"},"reference-count":74,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2023,4,20]],"date-time":"2023-04-20T00:00:00Z","timestamp":1681948800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100006475","name":"Bergens Forskningsstiftelse","doi-asserted-by":"publisher","award":["BFS2017TMT04"],"award-info":[{"award-number":["BFS2017TMT04"]}],"id":[{"id":"10.13039\/501100006475","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100006475","name":"Bergens Forskningsstiftelse","doi-asserted-by":"publisher","award":["BFS2017TMT08"],"award-info":[{"award-number":["BFS2017TMT08"]}],"id":[{"id":"10.13039\/501100006475","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004257","name":"Helse Vest","doi-asserted-by":"publisher","award":["Career stipend for A Wolff"],"award-info":[{"award-number":["Career stipend for A Wolff"]}],"id":[{"id":"10.13039\/501100004257","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100005416","name":"Norges Forskningsr\u00e5d","doi-asserted-by":"publisher","award":["245979\/F50"],"award-info":[{"award-number":["245979\/F50"]}],"id":[{"id":"10.13039\/501100005416","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100009708","name":"Novo Nordisk Fonden","doi-asserted-by":"publisher","award":["NNF17OC0027492"],"award-info":[{"award-number":["NNF17OC0027492"]}],"id":[{"id":"10.13039\/501100009708","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["febs.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["FEBS Letters"],"published-print":{"date-parts":[[2023,5]]},"abstract":"<jats:p>\n                    Autoimmune polyendocrine syndrome type I (APS\u20101) is caused by mutations in the autoimmune regulator (\n                    <jats:italic>AIRE<\/jats:italic>\n                    ) gene and characterised clinically by multiple autoimmune manifestations and serologically by autoantibodies against tissue proteins and cytokines. We here hypothesised that lack of AIRE expression in thymus affects blood immune cells and performed whole\u2010blood microarray analysis (\n                    <jats:italic>N<\/jats:italic>\n                    \u2009=\u200916 APS\u2010I patients vs 16 controls), qPCR verification, and bioinformatic deconvolution of cell subsets. We identified B cell responses as being downregulated in APS\u20101 patients, which was confirmed by qPCR; these results call for further studies on B cells in this disorder. The type I interferon (IFN\u2010I) pathway was also downregulated in APS\u20101, and the presence of IFN antibodies is the likely reason for this mild overall downregulation of the IFN\u2010I genes in most APS\u20101 patients.\n                  <\/jats:p>","DOI":"10.1002\/1873-3468.14625","type":"journal-article","created":{"date-parts":[[2023,4,13]],"date-time":"2023-04-13T11:30:49Z","timestamp":1681385449000},"page":"1261-1274","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Systemic interferon type I and B cell responses are impaired in autoimmune polyendocrine syndrome type 1"],"prefix":"10.1002","volume":"597","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4248-7737","authenticated-orcid":false,"given":"Bergithe E.","family":"Oftedal","sequence":"first","affiliation":[{"name":"Department of Clinical Science University of Bergen  Bergen Norway"},{"name":"KG Jebsen Center for autoimmune diseases University of Bergen  Bergen Norway"},{"name":"Department of Medicine Haukeland University hospital  Bergen Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicolas","family":"Delaleu","sequence":"additional","affiliation":[{"name":"2cSysBioMed  Contra Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Dolan","sequence":"additional","affiliation":[{"name":"KG Jebsen Center for autoimmune diseases University of Bergen  Bergen Norway"},{"name":"Department of Informatics University of Bergen  Bergen Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anthony","family":"Meager","sequence":"additional","affiliation":[{"name":"Biotherapeutics Group The National Institute for Biological Standards and Control  Potters Bar UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7886-2976","authenticated-orcid":false,"given":"Eystein S.","family":"Husebye","sequence":"additional","affiliation":[{"name":"Department of Clinical Science University of Bergen  Bergen Norway"},{"name":"KG Jebsen Center for autoimmune diseases University of Bergen  Bergen Norway"},{"name":"Department of Medicine Haukeland University hospital  Bergen Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8485-2248","authenticated-orcid":false,"given":"Anette S. B.","family":"Wolff","sequence":"additional","affiliation":[{"name":"Department of Clinical Science University of Bergen  Bergen Norway"},{"name":"KG Jebsen Center for autoimmune diseases University of Bergen  Bergen Norway"},{"name":"Department of Medicine Haukeland University hospital  Bergen Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,4,20]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra1713301"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2006-1873"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.1075958"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.1038\/ni906"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2010.08.019"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.1084\/jem.20091669"},{"key":"e_1_2_11_8_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pmed.0030289"},{"key":"e_1_2_11_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clim.2008.07.002"},{"key":"e_1_2_11_10_1","doi-asserted-by":"publisher","DOI":"10.1084\/jem.20091983"},{"key":"e_1_2_11_11_1","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2017.00976"},{"key":"e_1_2_11_12_1","doi-asserted-by":"publisher","DOI":"10.1038\/nri2136-c1"},{"key":"e_1_2_11_13_1","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.178.2.1208"},{"key":"e_1_2_11_14_1","article-title":"AIRE expression controls the peripheral selection of autoreactive B cells","volume":"12","author":"Sng J","year":"2019","journal-title":"Sci Immunol"},{"key":"e_1_2_11_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-3083.2010.02397.x"},{"key":"e_1_2_11_16_1","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.1100212"},{"key":"e_1_2_11_17_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0808205105"},{"key":"e_1_2_11_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaut.2009.07.002"},{"key":"e_1_2_11_19_1","doi-asserted-by":"publisher","DOI":"10.1515\/JPEM.2005.18.2.155"},{"key":"e_1_2_11_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.imbio.2016.09.001"},{"key":"e_1_2_11_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cellimm.2009.01.012"},{"key":"e_1_2_11_22_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2249.2009.03873.x"},{"key":"e_1_2_11_23_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00109-008-0374-7"},{"key":"e_1_2_11_24_1","doi-asserted-by":"publisher","DOI":"10.1002\/eji.200535240"},{"key":"e_1_2_11_25_1","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2016-1821"},{"key":"e_1_2_11_26_1","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/gkv007"},{"key":"e_1_2_11_27_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0506580102"},{"key":"e_1_2_11_28_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0013984"},{"key":"e_1_2_11_29_1","doi-asserted-by":"publisher","DOI":"10.1186\/ar4362"},{"key":"e_1_2_11_30_1","unstructured":"Consortium TC(2019)Cytoscape.https:\/\/cytoscape.org\/"},{"key":"e_1_2_11_31_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-011-9617-4"},{"key":"e_1_2_11_32_1","doi-asserted-by":"publisher","DOI":"10.1128\/MMBR.00024-13"},{"key":"e_1_2_11_33_1","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2019.00325"},{"key":"e_1_2_11_34_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.immuni.2006.08.007"},{"key":"e_1_2_11_35_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.6166984"},{"key":"e_1_2_11_36_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.63.4.1102"},{"key":"e_1_2_11_37_1","doi-asserted-by":"publisher","DOI":"10.2337\/db13-1777"},{"key":"e_1_2_11_38_1","doi-asserted-by":"publisher","DOI":"10.3389\/fendo.2017.00232"},{"key":"e_1_2_11_39_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ecl.2007.07.001"},{"key":"e_1_2_11_40_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41598-021-91124-3"},{"key":"e_1_2_11_41_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41598-019-52733-1"},{"key":"e_1_2_11_42_1","article-title":"Deciphering the transcriptomic heterogeneity of duodenal coeliac disease biopsies","volume":"4","author":"Wolf J","year":"2021","journal-title":"Int J Mol Sci"},{"key":"e_1_2_11_43_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9343(96)00259-8"},{"key":"e_1_2_11_44_1","doi-asserted-by":"publisher","DOI":"10.1530\/eje.0.1320587"},{"key":"e_1_2_11_45_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2008-03-144634"},{"key":"e_1_2_11_46_1","doi-asserted-by":"publisher","DOI":"10.1586\/eci.12.106"},{"key":"e_1_2_11_47_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2016.06.024"},{"key":"e_1_2_11_48_1","doi-asserted-by":"publisher","DOI":"10.3390\/ijms17071106"},{"key":"e_1_2_11_49_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.xcrm.2022.100903"},{"key":"e_1_2_11_50_1","article-title":"Preexisting autoantibodies to type I IFNs underlie critical COVID\u201019 pneumonia in patients with APS\u20101","volume":"5","author":"Bastard P","year":"2021","journal-title":"J Exp Med"},{"key":"e_1_2_11_51_1","doi-asserted-by":"publisher","DOI":"10.1007\/s40618-020-01323-4"},{"key":"e_1_2_11_52_1","doi-asserted-by":"publisher","DOI":"10.3390\/genes12020169"},{"key":"e_1_2_11_53_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2021.03.034"},{"key":"e_1_2_11_54_1","article-title":"Autoantibodies against type I IFNs in patients with life\u2010threatening COVID\u201019","volume":"23","author":"Bastard P","year":"2020","journal-title":"Science"},{"key":"e_1_2_11_55_1","article-title":"Patients with autoimmune polyendocrine syndrome type 1 have an increased susceptibility to severe herpesvirus infections","volume":"8","author":"Iivo H","year":"2021","journal-title":"Clin Immunol"},{"key":"e_1_2_11_56_1","first-page":"131","article-title":"Mild COVID\u201019 despite autoantibodies against type I IFNs in autoimmune polyendocrine syndrome type 1","volume":"15","author":"Meisel C","year":"2021","journal-title":"J Clin Invest"},{"key":"e_1_2_11_57_1","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2020.605673"},{"key":"e_1_2_11_58_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.immuni.2015.05.013"},{"key":"e_1_2_11_59_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2016.09.044"},{"key":"e_1_2_11_60_1","doi-asserted-by":"publisher","DOI":"10.1080\/08916930701773941"},{"key":"e_1_2_11_61_1","doi-asserted-by":"publisher","DOI":"10.1189\/jlb.3A0414-213RR"},{"key":"e_1_2_11_62_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1074-7613(00)80418-5"},{"key":"e_1_2_11_63_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00188178"},{"key":"e_1_2_11_64_1","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2018.02988"},{"key":"e_1_2_11_65_1","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2018.02423"},{"key":"e_1_2_11_66_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaut.2011.01.002"},{"key":"e_1_2_11_67_1","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2020.498703"},{"key":"e_1_2_11_68_1","first-page":"21","article-title":"Role of production of type I interferons by B cells in the mechanisms and pathogenesis of systemic lupus erythematosus","volume":"25","author":"Hamilton JA","year":"2018","journal-title":"Discov Med"},{"key":"e_1_2_11_69_1","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2018.01372"},{"key":"e_1_2_11_70_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.immuni.2016.02.012"},{"key":"e_1_2_11_71_1","article-title":"Aberrant type 1 immunity drives susceptibility to mucosal fungal infections","volume":"15","author":"Break TJ","year":"2021","journal-title":"Science"},{"key":"e_1_2_11_72_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41588-017-0026-3"},{"key":"e_1_2_11_73_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.celrep.2019.07.068"},{"key":"e_1_2_11_74_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41590-020-0622-8"},{"key":"e_1_2_11_75_1","doi-asserted-by":"publisher","DOI":"10.1093\/bioinformatics\/btw535"}],"container-title":["FEBS Letters"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/febs.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/1873-3468.14625","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,29]],"date-time":"2023-10-29T23:34:24Z","timestamp":1698622464000},"score":1,"resource":{"primary":{"URL":"https:\/\/febs.onlinelibrary.wiley.com\/doi\/10.1002\/1873-3468.14625"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,4,20]]},"references-count":74,"journal-issue":{"issue":"9","published-print":{"date-parts":[[2023,5]]}},"alternative-id":["10.1002\/1873-3468.14625"],"URL":"https:\/\/doi.org\/10.1002\/1873-3468.14625","archive":["Portico"],"relation":{"has-review":[{"id-type":"doi","id":"10.1002\/1873-3468.14625\/v1\/review1","asserted-by":"object"},{"id-type":"doi","id":"10.1002\/1873-3468.14625\/v2\/decision1","asserted-by":"object"},{"id-type":"doi","id":"10.1002\/1873-3468.14625\/v2\/response1","asserted-by":"object"},{"id-type":"doi","id":"10.1002\/1873-3468.14625\/v1\/decision1","asserted-by":"object"}]},"ISSN":["0014-5793","1873-3468"],"issn-type":[{"value":"0014-5793","type":"print"},{"value":"1873-3468","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,4,20]]},"assertion":[{"value":"2023-02-01","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-03-24","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-04-20","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}