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Projection of an immunological self shadow within the thymus by the aire protein.

Humans expressing a defective form of the transcription factor AIRE (autoimmune regulator) develop multiorgan autoimmune disease. We used aire- deficient mice to test the hypothesis that this transcription factor regulates autoimmunity by promoting the ectopic expression of peripheral tissue- restricted antigens in medullary epithelial cells of the thymus. This hypothesis proved correct. The mutant animals exhibited a defined profile of autoimmune diseases that depended on the absence of aire in stromal cells of the thymus. Aire-deficient thymic medullary epithelial cells showed a specific reduction in ectopic transcription of genes encoding peripheral antigens. These findings highlight the importance of thymically imposed "central" tolerance in controlling autoimmunity.

Pubmed ID: 12376594


  • Anderson MS
  • Venanzi ES
  • Klein L
  • Chen Z
  • Berzins SP
  • Turley SJ
  • von Boehmer H
  • Bronson R
  • Dierich A
  • Benoist C
  • Mathis D


Science (New York, N.Y.)

Publication Data

November 15, 2002

Associated Grants

  • Agency: NIDDK NIH HHS, Id: 2 P30 DK36836-16
  • Agency: NIDDK NIH HHS, Id: 2T32 DK07260-26
  • Agency: NIDDK NIH HHS, Id: KO8-DK59958-01A1
  • Agency: NIDDK NIH HHS, Id: R01 DK60027-01
  • Agency: NCI NIH HHS, Id: T32CA70083-05

Mesh Terms

  • Aging
  • Animals
  • Autoantibodies
  • Autoantigens
  • Autoimmune Diseases
  • Autoimmunity
  • Epithelial Cells
  • Female
  • Gene Expression Profiling
  • Gene Expression Regulation
  • Gene Targeting
  • Humans
  • Lymphocytes
  • Male
  • Mice
  • Mice, Inbred C57BL
  • Mice, Knockout
  • Polyendocrinopathies, Autoimmune
  • Radiation Chimera
  • Reverse Transcriptase Polymerase Chain Reaction
  • Self Tolerance
  • Stromal Cells
  • T-Lymphocytes
  • Thymus Gland
  • Transcription Factors