Table 1.
Blood serum concentrations of the metabolites whose levels were increased/decreased in Pah-R261Q mice in respect to the control WT group. Concentrations are expressed as arithmetic mean ± SD; n = 19 WT and 19 Pah-R261Q mice.
| Metabolite (name) | WT (µM) | Pah-R261Q (µM) | Difference | p Value (MW)a |
|---|---|---|---|---|
| Phenylalanine | 71.9 (10.3) | 113 (22) | 41.1 | 0.0000004 |
| β-Hydroxybutyrate | 150 (99) | 282 (133) | 132 | 0.029 |
| Trimethyllysine | 0.803 (0.165) | 0.976 (0.213) | 0.173 | 0.050 |
| Leucine | 143 (23) | 164 (42) | 21 | 0.075 |
| Isoleucine | 87.5 (12.8) | 101 (24) | 13.5 | 0.091 |
| α-Ketoglutaric acid | 38.6 (15.2) | 26.3 (10.8) | −12.3 | 0.003 |
| Glutamic acid | 39.5 (20.7) | 29.4 (10.2) | −10.1 | 0.008 |
| Alanine | 444 (71) | 362 (80) | −82 | 0.010 |
| Tryptophan | 103 (24) | 82.9 (30.9) | −20.1 | 0.013 |
| Quinolinic acid | 0.178 (0.090) | 0.130 (0.041) | −0.48 | 0.023 |
| Creatine | 154 (40) | 127 (30) | −27 | 0.026 |
| Aspartic acid | 27.4 (14.4) | 22.7 (11.0) | −4.7 | 0.043 |
| Glutamine | 687 (76) | 622 (112) | −65 | 0.043 |
| Tyrosine | 81.3 (25.3) | 77.9 (14.0) | −3.4 | 0.050 |
| Methylmalonic acid | 0.701 (0.095) | 0.585 (0.118) | −0.116 | 0.060 |
| Kynurenine | 0.740 (0.234) | 0.573 (0.264) | −0.167 | 0.080 |
| Proline | 90.2 (25.4) | 73.0 (17.2) | −17.2 | 0.085 |
aTwo-tailed p values for differences between serum concentration in WT and Pah-R261Q mice were obtained from Mann Whitney (MW) U test.
See also Supplementary Table 2.