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Zero tolerance! A perspective on monogenic disorders with defective regulatory T cells and IBD ‐like disease
Author(s) -
AbdelMotal Ussama M.,
AlShaibi Ahmad,
Elawad Mamoun,
Lo Bernice
Publication year - 2019
Publication title -
immunological reviews
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 6.839
H-Index - 223
eISSN - 1600-065X
pISSN - 0105-2896
DOI - 10.1111/imr.12717
Subject(s) - biology , immune system , immunology , disease , autoimmune disease , regulatory t cell , microbiome , function (biology) , immune tolerance , inflammatory bowel disease , genetics , t cell , medicine , il 2 receptor , antibody , pathology
Summary Recently, several studies have investigated a number of rare monogenic autoimmune disorders, in which the causative genetic defects were identified and found to affect the development or function of regulatory T cells (Tregs). The studies of these disorders have facilitated a deeper understanding of the mechanisms involved in immune regulation and tolerance. Furthermore, these studies have highlighted the importance of Tregs in maintaining homeostasis at the mucosal interface between the host and microbiome. Here, we offer our perspective on these monogenic autoimmune disorders, highlighting their overlapping clinical features with inflammatory bowel disease.