A Specific Anti-Aire Antibody Reveals Aire Expression Is Restricted to Medullary Thymic Epithelial Cells and Not Expressed in Periphery

Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy is an autoimmune disorder caused by mutations in the autoimmune regulator gene AIRE. We examined the expression of Aire in different organs (thymus, spleen, and lymph nodes) in C57BL/6 mice, using a novel rat mAb, specific for murine Air...

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Bibliographic Details
Published in:	The Journal of immunology (1950) Vol. 180; no. 6; pp. 3824 - 3832
Main Authors:	Hubert, Francois-Xavier, Kinkel, Sarah A, Webster, Kylie E, Cannon, Ping, Crewther, Pauline E, Proeitto, Anna I, Wu, Li, Heath, William R, Scott, Hamish S
Format:	Journal Article
Language:	English
Published:	United States Am Assoc Immnol 15-03-2008
Subjects:	AIRE Protein Amino Acid Sequence Animals Antibodies, Monoclonal - analysis Antibodies, Monoclonal - biosynthesis Antibody Specificity Antigen-Antibody Reactions Cell Nucleus - immunology Cell Nucleus - metabolism Epithelial Cells - immunology Epithelial Cells - metabolism Immunophenotyping Mice Mice, Inbred C57BL Mice, Knockout Mice, Transgenic Molecular Sequence Data Quantum Dots Rats Thymus Gland - cytology Thymus Gland - immunology Thymus Gland - metabolism Transcription Factors - biosynthesis Transcription Factors - deficiency Transcription Factors - genetics Transcription Factors - immunology
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Summary:	Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy is an autoimmune disorder caused by mutations in the autoimmune regulator gene AIRE. We examined the expression of Aire in different organs (thymus, spleen, and lymph nodes) in C57BL/6 mice, using a novel rat mAb, specific for murine Aire. Using flow cytometry, directly fluorochrome-labeled mAb revealed Aire expression in a rare thymic cellular subset that was CD45(-), expressed low levels of Ly51, and was high for MHC-II and EpCam. This subset also expressed a specific pattern of costimulatory molecules, including CD40, CD80, and PD-L1. Immunohistochemical analysis revealed that Aire(+) cells were specifically localized to the thymus or, more precisely, to the cortico-medulla junction and medulla, correlating with the site of negative selection. Although in agreement with previous studies, low levels of Aire mRNA was detected in all dendritic cell subtypes however lacZ staining, immunohistochemistry and flow cytometry failed to detect Aire protein. At a cellular level, Aire was expressed in perinuclear speckles within the nucleus. This report provides the first detailed analysis of Aire protein expression, highlighting the precise location at both the tissue and cellular level.
Bibliography:	ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23
ISSN:	0022-1767 1550-6606
DOI:	10.4049/jimmunol.180.6.3824