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Aire regulates negative selection of organ-specific T cells

Liston, Adrian; Lesage, Sylvie; Wilson, Judith; Peltonen, L; Goodnow, Christopher

Description

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...[Show more]

dc.contributor.authorListon, Adrian
dc.contributor.authorLesage, Sylvie
dc.contributor.authorWilson, Judith
dc.contributor.authorPeltonen, L
dc.contributor.authorGoodnow, Christopher
dc.date.accessioned2015-12-13T23:17:29Z
dc.identifier.issn1529-2908
dc.identifier.urihttp://hdl.handle.net/1885/89727
dc.description.abstractAutoimmune 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.
dc.publisherNature Publishing Group
dc.sourceNature Immunology
dc.subjectKeywords: 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
dc.titleAire regulates negative selection of organ-specific T cells
dc.typeJournal article
local.description.notesImported from ARIES
local.description.refereedYes
local.identifier.citationvolume4
dc.date.issued2003
local.identifier.absfor110703 - Autoimmunity
local.identifier.ariespublicationMigratedxPub19837
local.type.statusPublished Version
local.contributor.affiliationListon, Adrian, College of Medicine, Biology and Environment, ANU
local.contributor.affiliationLesage, Sylvie, College of Medicine, Biology and Environment, ANU
local.contributor.affiliationWilson, Judith, College of Medicine, Biology and Environment, ANU
local.contributor.affiliationPeltonen, L, University of Helsinki
local.contributor.affiliationGoodnow, Christopher, College of Medicine, Biology and Environment, ANU
local.description.embargo2037-12-31
local.bibliographicCitation.issue4
local.bibliographicCitation.startpage350
local.bibliographicCitation.lastpage354
local.identifier.doi10.1038/ni906
dc.date.updated2015-12-12T08:53:07Z
local.identifier.scopusID2-s2.0-0037383758
CollectionsANU Research Publications

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