The development of mouse APECED models provides new insight into the role of AIRE in immune regulation

Autoimmune polyendocrinopathy candidiasis ectodermal dystrophy is a rare recessive autoimmune disorder caused by a defect in a single gene called AIRE (autoimmune regulator). Characteristics of this disease include a variable combination of autoimmune endocrine tissue destruction, mucocutaneous cand...

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Bibliographic Details
Published in:Clinical & developmental immunology 2005-09, Vol.12 (3), p.211-216
Main Authors: Pereira, Lara E, Bostik, Pavel, Ansari, Aftab A
Format: Article
Language:English
Subjects:
AIRE Protein
Animals
Disease Models, Animal
Humans
Mice
Mice, Knockout
Mice, Transgenic
Mutation
Polyendocrinopathies, Autoimmune - etiology
Polyendocrinopathies, Autoimmune - genetics
Polyendocrinopathies, Autoimmune - immunology
Transcription Factors - chemistry
Transcription Factors - deficiency
Transcription Factors - genetics
Transcription Factors - immunology
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Summary:Autoimmune polyendocrinopathy candidiasis ectodermal dystrophy is a rare recessive autoimmune disorder caused by a defect in a single gene called AIRE (autoimmune regulator). Characteristics of this disease include a variable combination of autoimmune endocrine tissue destruction, mucocutaneous candidiasis and ectodermal dystrophies. The development of Aire-knockout mice has provided an invaluable model for the study of this disease. The aim of this review is to briefly highlight the strides made in APECED research using these transgenic murine models, with a focus on known roles of Aire in autoimmunity. The findings thus far are compelling and prompt additional areas of study which are discussed.
ISSN:1740-2522
2314-8861
2314-7156
1365-2567
1740-2530
DOI:10.1080/17402520500212589