期刊
Current Opinion in Allergy and Clinical Immunology
卷 15, 期 6, 页码 525-532出版社
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/ACI.0000000000000214
关键词
forkhead domain; forkhead box protein 3; immunodysregulation; polyendocrinopathy; enteropathy and X-linked syndrome; Treg cells
资金
- CIHR [MOP67211, MOP84041]
- Canada Research Chair program
Purpose of review This article presents a comprehensive review of the immunodysregulation, polyendocrinopathy, enteropathy and X- linked ( IPEX) syndrome, covering both the clinical and molecular aspects of the disease. Recent findings The IPEX syndrome is a rare immunological disorder in humans caused by inheritable mutations in the FOXP3 gene, the master transcriptional regulator for the development and function of CD4_ regulatory T ( Treg) cells. Forkhead box protein 3 ( FOXP3+) Treg cells represent a unique T- cell lineage with inhibitory functions, and are responsible for immune homeostasis and tolerance to self and nonself antigens. Evidence shows that a Treg developmental deficiency or dysfunction underlies the severe, multiorgan, autoimmune disease of IPEX. Summary An in- depth structural and functional analysis of the molecular domains of FOXP3 is essential for our understanding of the observed clinical heterogeneity and prognosis in IPEX.
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