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Article

Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome: A systematic review.

Autoimmunity reviews - 1 Jun 2020

Park Jae Hyon, Lee Keum Hwa, Jeon Bokyoung, Ochs Hans D, Lee Joon Suk, Gee Heon Yung, Seo Seeun, Geum Dongil, Piccirillo Ciriaco A, Eisenhut Michael, van der Vliet Hans J, Lee Jiwon M, Kronbichler Andreas, Ko Younhee, Shin Jae Il

Abstract excerpt

BACKGROUND: Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome is a monogenic disorder characterized by early onset fatal multi-system autoimmunity due to loss-of-function mutations in the gene encoding the forkhead box P3 (FOXP3) transcription factor which is crucial for the development, maturation, and maintenance of CD4+ regulatory T (T-reg) cells. Various autoimmune phenomena such...

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PMID: 32234571
DOI: 10.1016/j.autrev.2020.102526

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