Table 1.
Residual PAH activity, % | Steady-state level of Phe, mM | t of clearance of a load of Phe, min |
---|---|---|
100 | 0.057 (0.059) | 65 (89, 60–120) |
50 | 0.079 (0.092)* | 144 (159, 112–245)* |
40 | 0.093 | 180 |
20 | 0.129 | 360 |
10 | 0.154 | 705 |
3 | 0.226 | 1,410 |
0 | 0.314† | 3,240 |
Values in parentheses for steady-state levels of phenylalanine are published mean values. For the t of clearance, values in parentheses are mean values followed by the range of values reported for normals and obligate heterozygotes. See text for references. Phe, phenylalanine.
For obligate heterozygotes.
This steady-state concentration of Phe was reached in 295 h, during which time it was assumed that no food (i.e., no Phe) was consumed. Clearly, it is not a value that would be reached in any clinical study.