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Antibodies 2017, 6(1), 1; doi:10.3390/antib6010001

Pathogenic and Protective Autoantibodies in Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy (APECED)

Center for Immunology, Department of Pediatrics, Medical School, University of Minnesota, Minneapolis, MN 55455, USA
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Author to whom correspondence should be addressed.
Academic Editor: Christiane Hampe
Received: 27 October 2016 / Revised: 22 November 2016 / Accepted: 13 January 2017 / Published: 17 January 2017
(This article belongs to the Special Issue Auto-Antibody and Autoimmune Disease)
View Full-Text   |   Download PDF [200 KB, uploaded 17 January 2017]

Abstract

Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) is a rare disorder caused by mutations in the autoimmune regulator (AIRE) gene, leading to defects in T cell selection. The disease manifestations include both autoimmune tissue destruction and immunodeficiency, with specific susceptibility to chronic mucocutaneous candidiasis. Studies have demonstrated a wide repertoire of high affinity tissue- and cytokine-specific antibodies in patients with APECED. Here, we review the antigenic targets and function of these disease-causing and disease-ameliorating antibodies. View Full-Text
Keywords: APECED; autoantibodies; T cells; AIRE APECED; autoantibodies; T cells; AIRE
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. (CC BY 4.0).

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MDPI and ACS Style

Kashem, S.W.; Binstadt, B.A. Pathogenic and Protective Autoantibodies in Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy (APECED). Antibodies 2017, 6, 1.

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