title: The autoimmune regulator (Aire) controls iNKT cell development and maturation.
abstract: The mechanism underlying the autoimmune polyglandular syndrome type-1 (APS1) has been attributed to defective T-cell negative selection resulting from reduced expression and presentation of autoantigens in thymic medullary epithelial cells (MECs). It has also been postulated that Aire is involved in development of regulatory T cells, although supporting evidence is lacking. Here we show that expression of Aire in MECs is required for development of iNKT cells, suggesting a role for iNKT cells in APS1.
authors.affiliation: Center for Biotechnology and Genomic Medicine, Medical College of Georgia, 1120 15th Street, Augusta, Georgia 30912, USA. qmi@mail.mcg.edu
authors.forename: Qing-Sheng
authors.initials: QS
authors.lastname: Mi
authors.forename: Zhong-Bin
authors.initials: ZB
authors.lastname: Deng
authors.forename: Sunil K
authors.initials: SK
authors.lastname: Joshi
authors.forename: Zai-Zhao
authors.initials: ZZ
authors.lastname: Wang
authors.forename: Li
authors.initials: L
authors.lastname: Zhou
authors.forename: Sarah
authors.initials: S
authors.lastname: Eckenrode
authors.forename: Ratanmani
authors.initials: R
authors.lastname: Joshi
authors.forename: Dalam
authors.initials: D
authors.lastname: Ly
authors.forename: Bing
authors.initials: B
authors.lastname: Yi
authors.forename: Terry L
authors.initials: TL
authors.lastname: Delovitch
authors.forename: Jin-Xiong
authors.initials: JX
authors.lastname: She
mesh_terms: D000818:Animals; D015703:Antigens, CD / Q000276:immunology; D002478:Cells, Cultured; D004847:Epithelial Cells / Q000166:cytology / Q000276:immunology; D007694:Killer Cells, Natural / Q000166:cytology / Q000276:immunology / Q000502:physiology*; D008099:Liver / Q000166:cytology; D051379:Mice; D018345:Mice, Knockout; D016884:Polyendocrinopathies, Autoimmune / Q000276:immunology*; D013154:Spleen / Q000166:cytology; D016176:T-Lymphocyte Subsets / Q000166:cytology / Q000276:immunology / Q000502:physiology*; D013950:Thymus Gland / Q000166:cytology; D014157:Transcription Factors / Q000235:genetics / Q000276:immunology*; D000096927:AIRE Protein
chemical_list: D015703:Antigens, CD; D014157:Transcription Factors; D000096927:AIRE Protein