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Clearing the AIRE: on the pathophysiological basis of the autoimmune polyendocrinopathy syndrome type-1.

Abstract:

Autoimmune polyendocrine syndrome type-1 clinically manifests as the triad of hypoparathyroidism, primary adrenocortical insufficiency, and chronic mucocutaneous candidiasis. Mutations in the gene that encodes the autoimmune regulator protein, AIRE, have been identified as the cause of the autoimmune polyendocrine syndrome type-1. The loss of immunologic tolerance to tissue-restricted antigens consequent to an absence of AIRE expression in the thymus results in the thymic export of autoreacti...

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Publication status:
Published

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Publisher copy:
10.1016/j.ecl.2009.01.011

Authors


Journal:
Endocrinology and metabolism clinics of North America
Volume:
38
Issue:
2
Pages:
273-vii
Publication date:
2009-06-05
DOI:
EISSN:
1558-4410
ISSN:
0889-8529
URN:
uuid:81ecc268-6d5d-4d47-a78e-9be5051be863
Source identifiers:
309042
Local pid:
pubs:309042

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