The autoimmune targets in IPEX are dominated by gut epithelial proteins
Regulatory T (Treg) cells play a key role in immune system homeostasis by suppressing detrimental immune responses against self-proteins, in particular in the intestine.1 Patients with mutations in the gene FOXP3 display a severe defect of Treg-cell function that results in IPEX syndrome (OMIM 304790) characterized by impaired immune tolerance and multiorgan autoimmunity. Dermatitis, type 1 diabetes, and in particular autoimmune enteropathy are hallmarks of the syndrome. Intestinal Treg cells normally suppress immune responses against harmless dietary antigens and commensal microorganisms, but the intestinal self-proteins that are guarded by Treg cells have not yet been fully identified.
Source: Journal of Allergy and Clinical Immunology - Category: Allergy & Immunology Authors: Daniel Eriksson, Rosa Bacchetta, H örður Ingi Gunnarsson, Alice Chan, Federica Barzaghi, Stephan Ehl, Åsa Hallgren, Frederic van Gool, Fabian Sardh, Christina Lundqvist, Saila M. Laakso, Anders Rönnblom, Olov Ekwall, Outi Mäkitie, Sophie Bensing, Eys Tags: Letter to the Editor Source Type: research
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