Table 3.
Values of amino acids or biogenic amines and their derivatives, and of selected sums and ratios.
| Metabolite | HMBD ID | Patients | Controls | p-value | q-value | mean FC |
|---|---|---|---|---|---|---|
| Ac-Orn | HMDB0003357 | NA | NA | NA | NA | NA |
| ADMA | HMDB01539 | 0.51 ± 0.12 | 0.41 ± 0.05 | 0.0004 | 0.002↑ | 1.26 |
| Alanine | HMDB00161 HMDB01310 | 401.4 ± 84.2 | 410.5 ± 123.0 | 0.99 | 0.99 | −1.02 |
| alpha-AAA | HMDB00510 | 1.12 ± 0.61 | 0.77 ± 0.44 | 0.03 | 0.06 | 1.44 |
| Arginine | HMDB00517 HMDB03416 | 91.7 ± 26.8 | 108.2 ± 21.8 | 0.03 | 0.06 | −1.18 |
| Asparagine | HMDB00168 HMDB003378 | 43.7 ± 7.7 | 50.7 ± 8.0 | 0.007 | 0.016↓ | −1.16 |
| Aspartic acid | HMDB00191 HMDB06483 | 18.0 ± 10.4 | 17.2 ± 6.9 | 0.93 | 0.96 | 1.05 |
| c4-OH-Pro | HMDB0240251 | NA | NA | NA | NA | NA |
| Carnosine | HMDB00033 | NA | NA | NA | NA | NA |
| Citrulline | HMDB00904 | 29.4 ± 6.3 | 29.2 ± 6.6 | 0.91 | 0.95 | 1.01 |
| DOPA | HMDB00181 HMDB00609 | NA | NA | NA | NA | NA |
| Dopamine | HMDB00073 | NA | NA | NA | NA | NA |
| Glutamine | HMDB00641 HMDB03423 | 717.4 ± 134.1 | 749.0 ± 111.7 | 0.38 | 0.47 | −1.04 |
| Glutamic acid | HMDB00148 HMDB03339 | 85.9 ± 63.4 | 47.0 ± 21.8 | 0.0006 | 0.002↑ | 1.83 |
| Glycine | HMDB00123 | 321.6 ± 74.1 | 345.3 ± 91.7 | 0.41 | 0.5 | −1.07 |
| Histidine | HMDB00177 | 93.1 ± 15 | 110.4 ± 24.4 | 0.008 | 0.02↓ | −1.19 |
| Histamine | HMDB00870 | NA | NA | NA | NA | NA |
| Isoleucine | HMDB00172 HMDB0000557 | 106.6 ± 31.6 | 100.9 ± 39.5 | 0.44 | 0.53 | 1.06 |
| Kynurenine | HMDB00684 | NA | NA | NA | NA | NA |
| Leucine | HMDB00687 HMDB0013773 | 222.9 ± 78.6 | 232.8 ± 106.6 | 0.99 | 0.99 | −1.04 |
| Lysine | HMDB00182 HMDB03405 | 168.1 ± 23.5 | 165.8 ± 32.4 | 0.69 | 0.79 | 1.01 |
| Methionine | HMDB00696 | 27.9 ± 8.3 | 29.4 ± 7.8 | 0.47 | 0.55 | −1.06 |
| Met-SO | HMDB02005 | 0.94 ± 0.32 | 0.58 ± 0.23 | 0.00008 | 0.0005↑ | 1.64 |
| Nitro-Tyr | HMDB01904 | NA | NA | NA | NA | NA |
| Ornithine | HMDB00214 HMDB03374 | 139.4 ± 82.3 | 111.6 ± 40.9 | 0.22 | 0.29 | 1.25 |
| PEA | HMDB0012275 | NA | NA | NA | NA | NA |
| Phenylalanine | HMDB00159 | 77.7 ± 16.9 | 77.0 ± 12.9 | 0.99 | 0.99 | 1.01 |
| Proline | HMDB00162 HMDB03411 | 259.4 ± 63.7 | 265.7 ± 61.6 | 0.73 | 0.81 | −1.02 |
| Putrescine | HMDB01414 | NA | NA | NA | NA | NA |
| Sarcosine | HMDB00271 | 1.57 ± 0.94 | 1.62 ± 0.79 | 0.73 | 0.81 | −1.03 |
| SDMA | HMDB03334 | 0.46 ± 0.06 | 0.42 ± 0.08 | 0.1 | 0.15 | 1.08 |
| Serine | HMDB00187 HMDB03406 | 158.4 ± 33.3 | 146.5 ± 23.7 | 0.21 | 0.29 | 1.08 |
| Serotonin | HMDB00259 | 0.56 ± 0.33 | 0.7 ± 0.21 | 0.03 | 0.06 | −1.26 |
| Spermidine | HMDB01257 | NA | NA | NA | NA | NA |
| Spermine | HMDB01256 | NA | NA | NA | NA | NA |
| t4-OH-Pro | HMDB00725 HMDB0006055 | 15.8 ± 5.06 | 11.7 ± 5.2 | 0.007 | 0.02↑ | 1.35 |
| Taurine | HMDB00251 | 100.4 ± 57.1 | 132.6 ± 56.54 | 0.02 | 0.049↓ | −1.32 |
| Threonine | HMDB04041 HMDB00167 | 105.6 ± 21.0 | 127.3 ± 28.4 | 0.008 | 0.02↓ | −1.21 |
| Tryptophan | HMDB00929 HMDB0013609 | 90.5 ± 20.2 | 89.5 ± 23.3 | 0.84 | 0.89 | 1.01 |
| Tyrosine | HMDB00158 | 87.0 ± 23.0 | 83.6 ± 19.4 | 0.69 | 0.79 | 1.04 |
| Valine | HMDB00883 | 298.9 ± 72.5 | 290.7 ± 76.9 | 0.7 | 0.79 | 1.03 |
| BCAA | 628 ± 174 | 624 ± 217 | 0.8 | 0.87 | 1.01 | |
| AAA | 255 ± 53 | 250 ± 47 | 0.8 | 0.87 | 1.02 | |
| Total AA | 3544 ± 466 | 3588 ± 682 | 0.93 | 0.96 | −1.01 | |
| Essential AA | 1098 ± 223 | 1113 ± 293 | 0.97 | 0.99 | −1.01 | |
| Fischer ratio | 2.47 ± 0.52 | 2.45 ± 0.5 | 0.9 | 0.95 | 1.01 | |
| Met-SO/Met | 0.04 ± 0.016 | 0.02 ± 0.007 | <0.00001 | 0.0001↑ | 1.78 |
Metabolite concentrations for all analytes with selected sums and ratios, grouped by Fontan patients vs. controls. Values are given as mean ± standard deviation, unit of values of data [µmol/l]. FC, fold change (Fontan patients vs. controls); HMBD ID, Human Metabolome Database identification; AA, amino acids; AAA, aromatic amino acids; Ac-Orn, acetylornithine; ADMA, asymmetric dimethylarginine; alpha-AAA, alpha aminoadipic acid; BCAA, branched-chain amino acids; c4-OH-Pro, cis-4-hydroxyproline; DOPA, dihydroxyphenylalanine; Met-SO, methionine sulfoxide; Nitro-Tyr, nitrotyrosine; PEA, phenylethylamine; SDMA, symmetric dimethylarginine; t4-OH-Pro, trans-4-hydroxyproline; ↑, statistically significant higher serum concentration in Fontan patients than in controls; ↓, statistically significant lower serum concentration in Fontan patients than in controls. ‘Fischer ratio’ is the ratio of BCAA/AAA.