Abstract

Autoimmune diabetes can occur in rare monogenic disorders such as APECED (autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy) and IPEX (immune dysregulation, polyendocrinopathy, enteropathy, X-linked), but it is more frequently inherited as a common multifactorial trait. The molecular bases of the two rare Mendelian forms of autoimmune diabetes, both characterized by a severe autoimmune pathology of several organs and tissues, have recently been clarified, providing key insights into some mechanisms leading to autoimmunity in humans and in mice.

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