Aire regulates negative selection of organ-specific T cells
Date
2003
Authors
Liston, Adrian
Lesage, Sylvie
Wilson, Judith
Peltonen, L
Goodnow, Christopher
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Nature Publishing Group
Abstract
Autoimmune polyendocrinopathy syndrome type I is a recessive Mendelian disorder resulting from mutations in a novel gene, AIRE, and is characterized by a spectrum of organ-specific autoimmune diseases. It is not known what tolerance mechanisms are defective as a result of AIRE mutation. By tracing the fate of autoreactive CD4+ T cells with high affinity for a pancreatic antigen in transgenic mice with an Aire mutation, we show here that Aire deficiency causes almost complete failure to delete the organ-specific cells in the thymus.These results indicate that autoimmune polyendocrinopathy syndrome I is caused by failure of a specialized mechanism for deleting forbidden T cell clones, establishing a central role for this tolerance mechanism.
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Keywords: autoimmune regulator protein; CD4 antigen; cell antigen; transcription factor; unclassified drug; animal cell; animal experiment; animal model; antibody specificity; article; autoimmune disease; autoimmune polyendocrinopathy candidiasis ectodermal dystrop
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Source
Nature Immunology
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Journal article
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Restricted until
2037-12-31
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