Pathogenic and protective autoantibodies in autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED)

Sakeen W. Kashem, Bryce A. Binstadt

Research output: Contribution to journalReview articlepeer-review

1 Scopus citations

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.

Original languageEnglish (US)
Article number1
JournalAntibodies
Volume6
Issue number1
DOIs
StatePublished - Mar 1 2017

Bibliographical note

Funding Information:
S.K. was supported by National Institutes of Health (NIH) grant T32GM008244.

Keywords

  • AIRE
  • APECED
  • Autoantibodies
  • T cells

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