alexa Autoimmune polyendocrinopathy candidiasis ectodermal dystrophy: known and novel aspects of the syndrome.
Immunology

Immunology

Journal of Clinical & Cellular Immunology

Author(s): Kisand K, Peterson P

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Abstract Autoimmune polyendocrinopathy candidiasis ectodermal dystrophy (APECED) is a monogenic autosomal recessive disease caused by mutations in the autoimmune regulator (AIRE) gene and, as a syndrome, is characterized by chronic mucocutaneous candidiasis and the presentation of various autoimmune diseases. During the last decade, research on APECED and AIRE has provided immunologists with several invaluable lessons regarding tolerance and autoimmunity. This review describes the clinical and immunological features of APECED and discusses emerging alternative models to explain the pathogenesis of the disease. © 2011 New York Academy of Sciences. This article was published in Ann N Y Acad Sci and referenced in Journal of Clinical & Cellular Immunology

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