Table 2. Surface marker deregulated on resting or activated CD4+ T cells from Alagille Syndrome patients.
Jagged1 | Notch1 | CD46 | CD127 | CD132 | ||||||
---|---|---|---|---|---|---|---|---|---|---|
NA | CD3 CD46 |
NA | CD3 CD46 |
NA | CD3 CD46 |
NA | CD3 CD46 |
NA | CD3C D46 |
|
HD1 | + | ↑ | + | ↑ | + | ↓ | + | ↓ | + | ↑ |
HD2 | + | ↑ | + | ↑ | + | ↓ | + | ↓ | + | ↑ |
AP1 | + (↑) | ↑(↑) | + | ↑ | + | ↓ | + | ↑ | + (↑↑) | ↑↑ |
AP2 | + (↑) | ↑(↑) | + | ↑ | + | ↓ (50%) | + | ↓ | + (↑) | ↔ |
AP3 | + (↑) | ↑(↑) | + (↓) | ↑ | + | ↔ | + | ↓ | + (↑) | ↔ |
AP4 | + (↑) | ↔ | + (↓) | ↑ | + | ↓ | +(↑↑) | ↔ | + (↑↑) | ↔ a |
+, present on resting T cells; ↑, increased expression upon activation; ↓, decreased expression upon activation; (↑); increased baseline expression compared to HD1 and 2 (≤ 250%); (↑↑), increased baseline expression compared to HD1 and 2 (< 250%); (↓), decreased baseline expression compared to HD1 and 2; ↔, no change in expression compared to non-activated sample;
expression remains increased compared to HDs (Supplementary Fig. 6)
AP, Alagille Syndrome patient; HD, healthy donor; NA, non-activated