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Breakdown of T cell tolerance and autoimmunity in primary immunodeficiency—lessons learned from monogenic disorders in mice and men
- Source :
-
Current Opinion in Immunology . Dec2008, Vol. 20 Issue 6, p646-654. 9p. - Publication Year :
- 2008
-
Abstract
- A key feature of the immune system is the capacity to monitor and control infections from non-self pathogens while maintaining tolerance to self-antigens. Primary immunodeficiencies (PID) are characterized by an increased susceptibility to infections, often associated with aberrant inflammatory responses and a concomitant high prevalence of autoimmunity. Autoimmunity in PID raises a conundrum: How can an immune system fail to respond to non-self pathogens while reacting vigorously to self-antigens? Recent advances from studies of PID patients and related animal models have revealed the crucial role of Aire-induced expression of self-antigens for deletion of autoreactive T cells in the thymus (central tolerance). Moreover, lessons from PID have provided unequivocal evidence for the essential role of regulatory T cells in suppressing autoreactive T cells in the periphery. Finally, findings from PID have broadened our understanding of how homeostatic proliferation and increased load or decreased clearance of apoptotic cells and non-self pathogens can lead to breakdown of peripheral tolerance. [Copyright &y& Elsevier]
- Subjects :
- *T cells
*AUTOIMMUNITY
*IMMUNODEFICIENCY
*MONOGENIC functions
Subjects
Details
- Language :
- English
- ISSN :
- 09527915
- Volume :
- 20
- Issue :
- 6
- Database :
- Academic Search Index
- Journal :
- Current Opinion in Immunology
- Publication Type :
- Academic Journal
- Accession number :
- 35503084
- Full Text :
- https://doi.org/10.1016/j.coi.2008.10.004