The autoimmune regulator (Aire) controls iNKT cell development and maturation.
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.
Mi
Qing-Sheng
QS
Center for Biotechnology and Genomic Medicine, Medical College of Georgia, 1120 15th Street, Augusta, Georgia 30912, USA. qmi@mail.mcg.edu
Deng
Zhong-Bin
ZB
Joshi
Sunil K
SK
Wang
Zai-Zhao
ZZ
Zhou
Li
L
Eckenrode
Sarah
S
Joshi
Ratanmani
R
Ly
Dalam
D
Yi
Bing
B
Delovitch
Terry L
TL
She
Jin-Xiong
JX
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
D015703:Antigens, CD; D014157:Transcription Factors; D000096927:AIRE Protein