Table 1.
Separate and combined measurements of chloride and bicarbonate secretory currents in epithelial monolayers (2D HIOs). Peak forskolin-induced and CFTRinh172-inhibited Isc responses in Meyler, chloride only, or bicarbonate only bath fluid. A, non-CF individual; B, Patient 1 (E193K/F508del); C, Patient 2 (R334W/F508del); D, G542X/F508del. Mean ± SD of n technical replicates is shown.
| A | Non-CF | |||||||||
| Meyler | Bicarbonate | Chloride | ||||||||
| n | Mean | SD | n | Mean | SD | n | Mean | SD | ||
| DMSO | ΔIsc Forskolin |
3 | 189.3 | 73.7 | 3 | 36.8 | 28.2 | 3 | 151.6 | 60.6 |
| B | E193K/F508del | |||||||||
| Meyler | Bicarbonate | Chloride | ||||||||
| n | Mean | SD | n | Mean | SD | n | Mean | SD | ||
| DMSO | ΔIsc Forskolin |
6 | 50.3 | 54.7 | 6 | 8.2 | 9.7 | 6 | 36.6 | 35.3 |
| ΔIsc CFTRinh172 |
6 | −35.3 | 39.2 | 6 | −3.0 | 2.2 | 6 | −25.3 | 22.9 | |
| ELX/TEZ/IVA | ΔIsc Forskolin |
6 | 84.7 | 50.6 | 6 | 21.7 | 16.7 | 6 | 85.0 | 62.1 |
| ΔIsc CFTRinh172 |
6 | −71.6 | 43.6 | 6 | −11.1 | 6.4 | 6 | −67.9 | 51.4 | |
| C | R334W/F508del | |||||||||
| Meyler | Bicarbonate | Chloride | ||||||||
| n | Mean | SD | n | Mean | SD | n | Mean | SD | ||
| DMSO | ΔIsc Forskolin |
3 | 6.1 | 2.5 | 4 | 1.3 | 1.4 | 4 | 6.1 | 1.9 |
| ΔIsc CFTRinh172 |
3 | −2.9 | 1.3 | 4 | −0.7 | 0.5 | 4 | −3.9 | 1.5 | |
| ELX/TEZ/IVA | ΔIsc Forskolin |
3 | 16.6 | 10.6 | 3 | 4.6 | 0.3 | 3 | 22.1 | 7.6 |
| ΔIsc CFTRinh172 |
3 | −17.1 | 8.1 | 3 | −3.2 | 2.2 | 3 | −19.4 | 6.4 | |
| D | G542X/F508del | |||||||||
| Meyler | Bicarbonate | Chloride | ||||||||
| n | Mean | SD | n | Mean | SD | n | Mean | SD | ||
| DMSO | ΔIsc Forskolin |
4 | 0.4 | 0.6 | 4 | 0.6 | 0.7 | 4 | 2.2 | 3.2 |
| ΔIsc CFTRinh172 |
4 | 0.1 | 0.4 | 4 | 0.3 | 1.0 | 4 | −1.9 | 3.2 | |
| ELX/TEZ/IVA | ΔIsc Forskolin |
4 | 8.0 | 6.2 | 4 | 3.1 | 2.0 | 4 | 11.4 | 6.9 |
| ΔIsc CFTRinh172 |
4 | −8.0 | 5.6 | 4 | −1.7 | 0.9 | 4 | −10.7 | 5.9 | |