Table 1.
Genetic diseases | Tregs | CXCR5+ Tregs (%Tregs) | Total memory | CD4+CXCR5− memory cells | |||
---|---|---|---|---|---|---|---|
Th1 (CXCR3+ CCR6−) | Th2 (CXCR3−CCR6−) | Th17 (CXCR3−CCR6+) | Th1/17 (CXCR3+ CCR6+) | ||||
Controls (n=47–71) |
6.1 ± 0.3 | 8.2 ± 0.9 | 52.2 ± 1.8 | 15.5 ± 1.0 | 47.3 ± 1.8 | 23.0 ± 1.1 | 14.1 ± 1.1 |
STAT3LOF (n=12–22) |
7.4 ± 0.7 | 8.7 ± 1.7 | 29.6 ± 3.7*** | 34.4 ± 4.3**** | 46.3 ± 5.1 | 5.3 ± 0.7**** | 10.0 ± 1.5 |
STA T1GOF (n=16–23) |
5.6 ± 0.7 | 12.0 ± 1.6 | 41.0 ± 3.7 | 27.4 ± 3.4* | 56.9 ± 3.7 | 6.9 ± 1.05**** | 9.5 ± 1.6 |
STA T1LOF =5–6) |
7.5 ± 1.1 | 15.9 ± 5.5 | 38.0 ± 6.1 | 22.4 ± 6.2 | 37.9 ± 7.6 | 20.5 ± 4.4 | 19.2 ± 5.0 |
IL-21/R (n=5) |
5.8 ± 1.1 | 6.6 ± 2.4 | 32.2 ± 7.1 | 25.1 ± 11.2 | 57.6 ± 10.5 | 12.0 ± 3.3 | 5.3 ± 1.1 |
IL10R (n=4) |
6.4 ± 1.2 | 9.8 ± 5.3 | 31.5 ± 9.4 | 20.1 ± 17.0 | 52.8 ± 12.3 | 23.0 ± 10.9 | 4.1 ± 1.8 |
CD40LG (n=8) |
3.6 ± 0.8 | 6.0 ± 1.4 | 25.9 ± 3.8** | 6.9 ± 2.0 | 70.7 ± 6.0* | 17.9 ± 2.8 | 4.6 ± 2.0 |
NEMO (n=8–12) |
9.0 ± 1.7 | 8.3 ± 1.7 | 40.2 ± 7.6 | 2.6 ± 1.6 | 85.2 ± 4.0**** | 10.7 ± 2.3** | 1.4 ± 0.7** |
BTK (n=4–11) |
4.9 ± 0.7 | 5.5 ± 1.1 | 30.0 ± 5.2** | 12.3 ± 3.6 | 71.4 ± 6.5 | 11.2 ± 1.8* | 5.2 ± 1.6 |
ICOS (n=6) |
8.8 ± 0.9 | 10.1 ± 2.8 | 53.1 ± 7.1 | 30.5 ± 6.3 | 50.1 ± 4.5 | 10.4 ± 1.4* | 8.9 ± 1.7 |
IL12RB1 (n=3) |
4.1 ± 1.8 | 10.8 ± 2.2 | 16.4 ± 3.0* | 12.8 ± 2.1 | 67.8 ± 7.6 | 16.3 ± 8.4 | 3.0 ± 1.2 |
IFNGR1/2 (n=4–7) |
15.8 ±5.0**** | 10.5 ± 1.5 | 49.1 ± 7.0 | 15.7 ± 3.6 | 55.9 ± 5.8 | 16.8 ± 2.6 | 11.5 ± 2.9 |
TYK2 (n=4) |
5.4 ± 0.6 | 3.3 ± 1.2 | 56.2 ±10.5 | 18.3 ± 5.6 | 43.4 ± 5.3 | 18.8 ± 4.6 | 19.5 ± 5.1 |
PBMCs from healthy controls or patients with specific gene mutations were labeled with mAb against CD4, CD127, CD25, CD45RA, CXCR5, CXCR3 and CCR6. The proportions of Tregs amongst all CD4+ T cells, the proportions Tregs expressing CXCR5 (putative Tfr cells), proportions of memory cells within the non-Treg cell population, and the proportions of CD4+CD45RA−CXCR5− memory cells with a Th1 (CXCR3+CCR6−), Th2 (CXCR3−CCR6−), Th17 (CXCR3−CCR6+) or Th1/17 (CXCR3+CCR6+) phenotype were then determined by flow cytometric analysis. The values represent mean ± SEM for the indicated number of healthy controls or patients. Significant differences (one-way ANOVA) between healthy controls and patients are indicated.