Bibliographic Details
| Title: |
APECED and the place of AIRE in the puzzle of the immune network associated with autoimmunity. |
| Authors: |
Aytekin, Elif Soyak1 (AUTHOR), Cagdas, Deniz2 (AUTHOR) deniz.ayvaz@hacettepe.edu.tr |
| Source: |
Scandinavian Journal of Immunology. Aug2023, Vol. 98 Issue 2, p1-15. 15p. |
| Subject Terms: |
*AUTOIMMUNE diseases, *HEMATOPOIETIC stem cell transplantation, *IMMUNOLOGICAL tolerance, *AUTOIMMUNITY |
| Abstract: |
In the last 20 years, discoveries about the autoimmune regulator (AIRE) protein and its critical role in immune tolerance have provided fundamental insights into understanding the molecular basis of autoimmunity. This review provides a comprehensive overview of the effect of AIRE on immunological tolerance and the characteristics of autoimmune diseases in Autoimmune Polyendocrinopathy‐Candidiasis‐Ectodermal Dystrophy (APECED), which is caused by biallelic AIRE mutations. A better understanding of the immunological mechanisms of AIRE deficiency may enlighten immune tolerance mechanisms and new diagnostic and treatment strategies for autoimmune diseases. Considering that not all clinical features of APECED are present in a given follow‐up period, the diagnosis is not easy in a patient at the first visit. Longer follow‐up and a multidisciplinary approach are essential for diagnosis. It is challenging to prevent endocrine and other organ damage compared with other diseases associated with multiple autoimmunities, such as FOXP3, LRBA, and CTLA4 deficiencies. Unfortunately, no curative therapy like haematopoietic stem cell transplantation or specific immunomodulation is present that is successful in the treatment. [ABSTRACT FROM AUTHOR] |
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Copyright of Scandinavian Journal of Immunology is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.) |
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