RNA and protein expression of the murine autoimmune regulator gene (Aire) in normal, RelB-deficient and in NOD mouse

Maarit Heino, Pärt Peterson, Niko Sillanpää, Sandrine Guérin, Li Wu, Graham Anderson, Hamish S. Scott, Stylianos E. Antonarakis, Jun Kudo, Nobuyoshi Shimizu, Eric J. Jenkinson, Philippe Naquet, Kai J E Krohn

Research output: Contribution to journalArticle

148 Citations (Scopus)

Abstract

Mutations in the putative transcription factor autoimmune regulator (AIRE) gene are responsible for autoimmune polyendocrinopathy-candidiosis-ectodermal dystrophy (APECED; OMIM240300), a monogenic recessively inherited disease characterized by destructive autoimmune diseases of the endocrine organs, chronic candidiosis of mucous membranes and ectodermal dystrophies. In this study the expression of murine homolog for AIRE protein, Aire, was detected in a fraction of thymic medullary epithelial cells. Subcellularly, in thymus the protein appears as concentrated into nuclear dot-like structures, whereas in transfected cells the protein is also bound along a cytosolic fibrillar network. By RT-PCR Aire mRNA was detected in thymus, lymph node, spleen and testis although the second round PCR amplified Aire specific band from most mouse tissues analyzed. Furthermore, the Aire mRNA was detected in dendritic cell (DC) populations isolated from thymus and spleen, representing both myeloid- and lymphoid-related lineages of DC. We also demonstrate that the Aire protein is absent in the thymus of RelB-deficient mouse and in NOD thymus most of the Aire positive cells showed an abnormal morphology. These results suggest that the Aire protein is associated with the normal development and/or action of a subset of thymic medullary stromal cells involved in tolerance induction.

Original languageEnglish
Pages (from-to)1884-1893
Number of pages10
JournalEuropean Journal of Immunology
Volume30
Issue number7
DOIs
Publication statusPublished - 2000
Externally publishedYes

Fingerprint

Inbred NOD Mouse
Regulator Genes
RNA
Thymus Gland
Proteins
Autoimmune Polyendocrinopathies
Dendritic Cells
Spleen
Polymerase Chain Reaction
Messenger RNA
Stromal Cells
Autoimmune Diseases
Testis
Mucous Membrane
Transcription Factors
Lymph Nodes
Epithelial Cells
Mutation

Keywords

  • Autoimmunity
  • Mouse model
  • Thymus epithelial cell

ASJC Scopus subject areas

  • Immunology

Cite this

RNA and protein expression of the murine autoimmune regulator gene (Aire) in normal, RelB-deficient and in NOD mouse. / Heino, Maarit; Peterson, Pärt; Sillanpää, Niko; Guérin, Sandrine; Wu, Li; Anderson, Graham; Scott, Hamish S.; Antonarakis, Stylianos E.; Kudo, Jun; Shimizu, Nobuyoshi; Jenkinson, Eric J.; Naquet, Philippe; Krohn, Kai J E.

In: European Journal of Immunology, Vol. 30, No. 7, 2000, p. 1884-1893.

Research output: Contribution to journalArticle

Heino, M, Peterson, P, Sillanpää, N, Guérin, S, Wu, L, Anderson, G, Scott, HS, Antonarakis, SE, Kudo, J, Shimizu, N, Jenkinson, EJ, Naquet, P & Krohn, KJE 2000, 'RNA and protein expression of the murine autoimmune regulator gene (Aire) in normal, RelB-deficient and in NOD mouse', European Journal of Immunology, vol. 30, no. 7, pp. 1884-1893. https://doi.org/10.1002/1521-4141(200007)30:7<1884::AID-IMMU1884>3.0.CO;2-P
Heino, Maarit ; Peterson, Pärt ; Sillanpää, Niko ; Guérin, Sandrine ; Wu, Li ; Anderson, Graham ; Scott, Hamish S. ; Antonarakis, Stylianos E. ; Kudo, Jun ; Shimizu, Nobuyoshi ; Jenkinson, Eric J. ; Naquet, Philippe ; Krohn, Kai J E. / RNA and protein expression of the murine autoimmune regulator gene (Aire) in normal, RelB-deficient and in NOD mouse. In: European Journal of Immunology. 2000 ; Vol. 30, No. 7. pp. 1884-1893.
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AU - Heino, Maarit

AU - Peterson, Pärt

AU - Sillanpää, Niko

AU - Guérin, Sandrine

AU - Wu, Li

AU - Anderson, Graham

AU - Scott, Hamish S.

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AU - Shimizu, Nobuyoshi

AU - Jenkinson, Eric J.

AU - Naquet, Philippe

AU - Krohn, Kai J E

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