dc.contributor.author | Wolff, Anette Susanne Bøe | |
dc.contributor.author | Braun, Sarah | |
dc.contributor.author | Husebye, Eystein Sverre | |
dc.contributor.author | Oftedal, Bergithe Eikeland | |
dc.date.accessioned | 2022-04-20T09:57:04Z | |
dc.date.available | 2022-04-20T09:57:04Z | |
dc.date.created | 2021-10-07T00:06:19Z | |
dc.date.issued | 2021 | |
dc.identifier.issn | 2227-9059 | |
dc.identifier.uri | https://hdl.handle.net/11250/2991567 | |
dc.description.abstract | Autoimmune polyendocrine syndrome type 1 (APS-1) is a rare but severe monogenetic autoimmune endocrine disease caused by failure of the Autoimmune Regulator (AIRE). AIRE regulates the negative selection of T cells in the thymus, and the main pathogenic mechanisms are believed to be T cell-mediated, but little is known about the role of B cells. Here, we give an overview of the role of B cells in thymic and peripheral tolerance in APS-1 patients and different AIRE-deficient mouse models. We also look closely into which autoantibodies have been described for this disorder, and their implications. Based on what is known about B cell therapy in other autoimmune disorders, we outline the potential of B cell therapies in APS-1 and highlight the unresolved research questions to be answered. | en_US |
dc.language.iso | eng | en_US |
dc.publisher | MDPI | en_US |
dc.rights | Navngivelse 4.0 Internasjonal | * |
dc.rights.uri | http://creativecommons.org/licenses/by/4.0/deed.no | * |
dc.title | B cells and autoantibodies in AIRE deficiency | en_US |
dc.type | Journal article | en_US |
dc.type | Peer reviewed | en_US |
dc.description.version | publishedVersion | en_US |
dc.rights.holder | Copyright 2021 The Author(s) | en_US |
dc.source.articlenumber | 1274 | en_US |
cristin.ispublished | true | |
cristin.fulltext | original | |
cristin.qualitycode | 1 | |
dc.identifier.doi | 10.3390/biomedicines9091274 | |
dc.identifier.cristin | 1943972 | |
dc.source.journal | Biomedicines | en_US |
dc.relation.project | Norges forskningsråd: 262677 | en_US |
dc.relation.project | Norges forskningsråd: 288022 | en_US |
dc.relation.project | Stiftelsen Kristian Gerhard Jebsen: KGJ senter for autoimmune sykdommer | en_US |
dc.identifier.citation | Biomedicines. 2021, 9 (9), 1274. | en_US |
dc.source.volume | 9 | en_US |
dc.source.issue | 9 | en_US |