Production and basal secretion of VWF from BOECs
| Subject . | Amino acid change . | Total VWF production (mU) . | VWF secreted in medium (mU) . |
|---|---|---|---|
| Healthy donor 1 | — | 175.5 ± 0.8 | 61.7 ± 0.7 |
| Healthy donor 2 | — | 98.2 ± 10.2 | 66.2 ± 5.9 |
| Healthy donor 3 | — | 41.5 ± 1.0 | 20.5 ± 1.3 |
| Healthy donor 4 | — | 128.1 ± 6.9 | 42.8 ± 1.6 |
| Healthy donor 5 | — | 181.8 ± 4.6 | 48.8 ± 3.5 |
| P6F1III1 | p.Arg854Gln | 82.2 ± 1.2 | 18.7 ± 0.4 |
| P6F3II1 | p.Ser1285Pro | 16.4 ± 1.1 | 5.4 ± 0.2 |
| P6F13II1 | p.Leu1307Pro | 5.7 ± 0.5 | 3.0 ± 0.3 |
| P6F12II1 | p.Tyr1584Cys | 155.5 ± 35.1 | 45.4 ± 1.2 |
| P6F16II1 | p.Cys2693Tyr | 121.5 ± 0.0 | 16.0 ± 0.1 |
| Subject . | Amino acid change . | Total VWF production (mU) . | VWF secreted in medium (mU) . |
|---|---|---|---|
| Healthy donor 1 | — | 175.5 ± 0.8 | 61.7 ± 0.7 |
| Healthy donor 2 | — | 98.2 ± 10.2 | 66.2 ± 5.9 |
| Healthy donor 3 | — | 41.5 ± 1.0 | 20.5 ± 1.3 |
| Healthy donor 4 | — | 128.1 ± 6.9 | 42.8 ± 1.6 |
| Healthy donor 5 | — | 181.8 ± 4.6 | 48.8 ± 3.5 |
| P6F1III1 | p.Arg854Gln | 82.2 ± 1.2 | 18.7 ± 0.4 |
| P6F3II1 | p.Ser1285Pro | 16.4 ± 1.1 | 5.4 ± 0.2 |
| P6F13II1 | p.Leu1307Pro | 5.7 ± 0.5 | 3.0 ± 0.3 |
| P6F12II1 | p.Tyr1584Cys | 155.5 ± 35.1 | 45.4 ± 1.2 |
| P6F16II1 | p.Cys2693Tyr | 121.5 ± 0.0 | 16.0 ± 0.1 |
VWF:Ag was determined for all BOECs at passage 6. Cells were grown until confluent in 12-well plates (approximately 1 × 105 cells per well) and incubated for 5 hours with EGM-2 medium (containing 2% serum). Total VWF production = VWF:Ag in conditioned medium (basal secretion) + VWF:Ag in the lysate. Each value represents the mean ± standard error of the mean of duplicate or triplicate measurements.