IPEX as a result of mutations in FOXP3

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Abstract

Immunodysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome is a rare disorder caused by mutations in the FOXP3 gene that result in the defective development of CD4+ CD25+ regulatory T cells which constitute an important T cell subset involved in immune homeostasis and protection against autoimmunity. Their deficiency is the hallmark of IPEX and leads to severe autoimmune phenomena including autoimmune enteropathy, dermatitis, thyroiditis, and type 1 diabetes, frequently resulting in death within the first 2 years of life. Apart from its clinical implications, IPEX illustrates the importance of immunoregulatory cells such as CD4+ CD25+ regulatory T cells. Copyright © 2007 H. J. J. van der Vliet and E. E. Nieuwenhuis.

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Van Der Vliet, H. J. J., & Nieuwenhuis, E. E. (2007). IPEX as a result of mutations in FOXP3. Clinical and Developmental Immunology. https://doi.org/10.1155/2007/89017

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