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Mechanism of immune dysregulation of recently described primary immune deficiencies.
| Mechanism of immune dysregulation | Omenn | STIM1 | WAS | STAT3 | CD25 | xIAP | STAT5b | IL10R | APECED | IPEX |
|---|---|---|---|---|---|---|---|---|---|---|
| Expansion of oligoclonal T cells | + | |||||||||
| Apoptosis/loss of TR cells | + | + | + | + | + | +/− | + | |||
| Defective IL-2 expression/responsiveness | + | + | + | + | ||||||
| Defective responsiveness to TR suppression | + | + | ||||||||
| Defective suppression by TR cells | + | + | + | |||||||
| Defective AIRE expression in thymus | + | + | ||||||||
|
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