TABLE 1.
Affinity of mutant αTTP proteins for NBD-tocopherol
| TTP variant | Clinical phenotype | Kda (nm) |
|---|---|---|
| Wild typeb | 8.5 ± 6.3 | |
| Wild type | 9.8 ± 2.3 | |
| R192Hb | AVED (mild) | 26.8 ± 7.7 |
| H101Qb | AVED (mild) | 38.9 ± 12.7 |
| A120Tb | AVED (mild) | 9.2 ± 5.4 |
| R59Wb | AVED (severe) | 70.8 ± 17.6 |
| E141Kb | AVED (severe) | 11.7 ± 2.7 |
| R221Wb | AVED (severe) | 21.7 ± 11.4 |
Averages and S.D. from more than three titrations are shown.
Kd values were determined using GST fusion proteins; n ≥ 3.