Data for Targeted Metabolomic Analysis in Patients with Wilson Disease Reveals Dysregulated Choline, Methionine and Aromatic Amino Acid Metabolism: Implications for Hepatic and Neurological Phenotypes (Study ST001259)

(Analysis AN002089)

Values for each metabolite have been scaled by dividing by the mean across all factors

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MetaboliteF1F2F3F4
1_15N2-L-Arginine iSTD 0.8259 0.7798 1.0415 1.0515
1-(1Z-Hexadecenyl)-sn-glycero-3-phosphocholine 1.3710 1.5030 0.9376 0.8596
1-(1Z-Octadecenyl)-2-(4Z,7Z,10Z,13Z,16Z,19Z-docosahexaenoyl)-sn-glycero-3-phosphocholine 0.8786 0.7970 0.9929 1.0787
1-(1Z-Octadecenyl)-2-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-sn-glycero-3-phosphocholine 0.7976 0.8577 0.9848 1.1029
1-(1Z-Octadecenyl)-2-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-sn-glycero-3-phosphoethanolamine 1.8351 0.7301 0.8980 0.8728
1-(1Z-Octadecenyl)-sn-glycero-3-phosphocholine 0.9497 0.9531 1.0110 1.0132
1,2-Di-(9Z,12Z,15Z-octadecatrienoyl)-sn-glycero-3-phosphocholine 1.5122 2.0410 0.8722 0.7906
1,2-Di-(9Z-octadecenoyl)-sn-glycero-3-phosphocholine 1.0530 0.9854 0.9865 0.9983
1,2-Diarachidonoyl-sn-glycero-3-phosphocholine 1.5304 2.0959 0.7902 0.8552
1,2-Dieicosenoyl-sn-glycero-3-phosphocholine 1.1494 0.7188 1.0794 0.9204
1,2-Dipalmitoleoyl-sn-glycero-3-phosphocholine 1.3333 1.6563 0.9702 0.8155
1,2-Distearoyl-sn-glycero-3-phosphocholine 1.0089 0.9073 1.0230 0.9898
1,2-Ditetradecanoyl-sn-glycero-3-phosphocholine 1.0854 1.4898 0.9737 0.9189
1,3-Dicyclohexylurea 1.0279 1.3059 0.8918 1.0464
1-(9Z-Octadecenoyl)-2-tetradecanoyl-sn-glycero-3-phosphocholine 0.6340 0.9040 1.1695 0.9696
1-(9Z-Octadecenoyl)-sn-glycero-3-phosphocholine 1.3293 1.4555 0.9342 0.8840
1-(9Z-Octadecenoyl)-sn-glycero-3-phosphoethanolamine 1.1481 1.4849 0.9646 0.9083
1_CUDA iSTD 0.9476 0.8005 1.0273 1.0227
1_D3-1-Methylnicotinamide iSTD 1.2008 1.1200 0.8696 1.0420
1_D3-Creatine iSTD 0.7634 0.8067 1.0269 1.0814
1_D3-Creatinine iSTD 0.8634 0.6672 1.0679 1.0320
1_D3-DL-Alanine iSTD 0.9162 0.6340 1.0295 1.0576
1_D3-DL-Aspartic acid iSTD 0.6665 0.4814 1.1043 1.0903
1_D3-DL-Glutamic acid iSTD 0.8096 0.6699 1.0149 1.1003
1_D3-Histamine, N-methyl- iSTD 0.9373 0.9457 1.0073 1.0219
1_D3-L-Carnitine iSTD 0.8138 0.8176 0.9572 1.1309
1_D5-L-Glutamine iSTD 0.7791 0.6431 1.0569 1.0738
1_D9-Betaine iSTD 0.9579 0.8686 1.0584 0.9783
1_D9-Butyrobetaine iSTD 0.7005 0.7828 1.2128 0.9257
1_D9-Caffeine iSTD 0.7639 0.7393 1.0549 1.0651
1_D9-Choline iSTD 0.6677 0.5853 1.0428 1.1327
1_D9-Crotonobetaine iSTD 0.7952 0.6689 1.0214 1.0987
1_D9-TMAO iSTD 0.7924 0.7792 1.0421 1.0618
1-Hexadecanoyl-2-(9Z-octadecenoyl)-sn-glycero-3-phosphocholine 1.1345 1.0469 0.9706 0.9775
1-Hexadecanoyl-2-(9Z-octadecenoyl)-sn-glycero-3-phosphoethanolamine 0.9291 0.7022 1.1099 0.9645
1-Hexadecanoyl-2-octadecadienoyl-sn-glycero-3-phosphocholine 1.1563 1.0507 0.9594 0.9807
1-Hexadecanoyl-2-octadecanoyl-sn-glycero-3-phosphocholine 0.6768 0.5923 1.2308 0.9466
1-Hexadecanoyl-sn-glycero-3-phosphocholine 1.5945 2.0741 0.7850 0.8430
1-hexadecyl-2-acetyl-glycero-3-phosphocholine 1.1664 1.4303 0.9585 0.9171
1-Hexadecyl-sn-glycero-3-phosphocholine 1.1049 0.6556 0.9855 1.0358
1-Lignoceroyl-2-hydroxy-sn-glycero-3-phosphocholine 1.4388 1.3879 0.9118 0.8812
1-Methylnicotinamide 1.1694 1.1584 1.0729 0.8492
1-Myristoyl-2-stearoyl-sn-glycero-3-phosphocholine 0.6769 0.5716 1.0931 1.0832
1-Myristoyl-sn-glycero-3-phosphocholine 1.3934 2.3115 0.8565 0.8005
1-O-(cis-9-Octadecenyl)-2-O-acetyl-sn-glycero-3-phosphocholine 1.2796 1.3007 0.9636 0.8965
1-Octadecanoyl-2-(4Z,7Z,10Z,13Z,16Z,19Z-docosahexaenoyl)-sn-glycero-3-phosphocholine 0.8288 0.9941 1.0028 1.0534
1-Octadecanoyl-2-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-sn-glycero-3-phosphocholine 0.0018 0.0021 2.4332 0.1021
1-Octadecanoyl-2-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-sn-glycero-3-phosphoserine 2.0795 2.1711 0.7902 0.6660
1-Octadecanoyl-2-docosahexaenoyl-sn-glycero-3-phosphoethanolamine 1.0055 0.9257 1.0895 0.9236
1-Octadecanoyl-2-eicosatetraenoyl-sn-glycero-3-phospho-(1^-myo-inositol) 1.2359 1.2332 0.9462 0.9383
1-Octadecanoyl-2-octadecenoyl-sn-glycero-3-phosphocholine 1.1769 0.9930 1.0906 0.8564
1-Octadecanoyl-sn-glycero-3-phosphoethanolamine 0.9992 1.1161 1.1060 0.8790
1-O-Hexadecyl-2-O-arachidonoyl-sn-glycero-3-phosphorylcholine 1.0224 0.9109 0.9688 1.0373
1-O-Hexadecyl-2-O-dihomo-linolenoylsn- glycero-3-phosphorylcholine 1.1741 0.9183 0.9870 0.9696
1-Palmitoyl-2-docosahexaenoyl-sn-glycero-3-phosphocholine 1.2187 1.1513 0.9578 0.9459
1-Palmitoyl-2-myristoyl-sn-glycero-3-phosphocholine 0.7961 0.9953 1.0977 0.9720
1-Palmitoyllysophosphatidylcholine 1.0890 1.2070 0.9829 0.9544
1-Pentadecanoyl-sn-glycero-3-phosphocholine 1.4900 4.4410 0.8804 0.4027
1-Stearoyl-2-hydroxy-sn-glycero-3-phosphocholine 1.2293 1.2356 1.0126 0.8758
1_Val-Tyr-Val iSTD 0.8397 0.7782 1.0273 1.0610
2-Amino-1-phenylethanol 0.5627 0.5724 1.0373 1.1740
2-Hydroxycarbamazepine 0.0005 0.0009 0.0005 2.5376
2-Linoleoyl-1-palmitoyl-sn-glycero-3-phosphoethanolamine 0.9070 0.8664 1.1059 0.9490
3-Methylhistidine 0.8947 0.5594 1.0904 1.0176
3-O-Hexadecyl-2-O-acetyl-sn-glycero-1-phosphorylcholine 1.1903 1.4001 0.9382 0.9339
4-Aminomethylcyclohexanecarboxylic acid 1.4021 1.2265 0.9378 0.8940
5-Aminovaleric acid 0.2534 1.3054 1.0369 1.1559
9-Octadecenamide 1.1266 1.2459 0.9899 0.9292
Acetylcarnitine 0.9241 0.9774 1.0974 0.9339
Acetylcholine 0.8734 1.0024 0.9856 1.0544
Alanine 0.9727 0.6881 1.0740 0.9875
beta-Cryptoxanthin 2.2547 1.6283 0.8745 0.6154
Betaine 0.9235 0.9426 0.9834 1.0500
Caffeine 2.0084 1.1565 0.7100 0.9301
Carbamazepine 0.0001 0.0001 0.0003 2.7688
Carnitine 0.9428 0.9557 0.9765 1.0484
Cholest-4-en-26-oic acid, 7.alpha.-hydroxy-3-oxo 0.7312 1.0329 1.0208 1.0612
Cholesterol 1.1053 1.1047 1.0163 0.9334
Choline 0.3658 0.2885 1.2365 1.0904
Cholylglycine 0.5456 0.2208 1.1895 1.0889
Conjugated linoleic Acid (10E,12Z) 0.9804 1.0563 1.0478 0.9510
Cotinine 0.5106 0.2420 0.5739 1.6925
Creatine 1.3249 0.7167 1.0354 0.9066
Creatinine 1.0163 1.1623 0.9495 1.0174
Decanoyl-L-carnitine 1.3587 2.0691 0.7636 0.9407
Deoxycholic acid 0.9663 0.9181 0.5321 1.4607
Docosahexaenoyl PAF C-16 1.2615 3.1967 0.7685 0.7854
Eicosapentaenoyl PAF C-16 1.1342 2.0094 0.8679 0.9218
Epsilon-Caprolactam 1.2313 0.9462 0.9926 0.9412
Gabapentin 1.2591 0.4116 1.1138 0.8774
gamma-butyrobetaine 0.9225 0.9314 1.0719 0.9665
Glycoursodeoxycholic acid 0.4956 0.2909 0.8343 1.4374
Hippuric acid 2.3227 0.9633 0.8307 0.7431
Histidine 0.7825 0.6157 1.1658 0.9717
Hypoxanthine 1.2285 1.2608 1.0074 0.8771
Isoleucine 0.9648 1.3044 0.9018 1.0573
Kynurenine 0.7623 0.9792 1.0281 1.0528
Lauroyl L-carnitine 1.3489 3.2360 0.7235 0.7944
Leucine 0.9415 1.1077 0.9526 1.0474
Metformin 9.7874 0.0012 0.0015 0.0014
Methionine 0.5353 0.5654 1.0982 1.1250
Metronidazole 0.0330 0.0065 2.5222 0.0165
N-(15Z-Tetracosenoyl)-1-.beta.-galactosyl-sphing-4-enine 0.7496 0.9376 0.9455 1.1436
N.epsilon.-Methyl-L-lysine 0.6762 0.7781 1.2023 0.9445
N-Lauroyl-D-erythro-sphingosylphosphorylcholine 1.4896 1.2907 1.0760 0.7216
N-Methylproline 1.2632 0.8275 0.9661 0.9758
N-Oleoyl-D-erythro-sphingosylphosphorylcholine 1.3469 1.2313 0.9982 0.8525
N-Palmitoylsphingosine 0.8896 1.0962 1.0124 1.0081
N-Stearoyl-4-sphingenyl-1-O-phosphorylcholine 0.3910 0.4850 0.9727 1.3059
N-Tetracosenoyl-4-sphingenyl-1-O-phosphorylcholine 0.6542 0.5397 0.9519 1.2323
Oleoyl L-carnitine 1.3195 1.5404 0.9594 0.8491
Omeprazole 0.0080 0.0103 0.0411 2.4076
Ornithine 0.6454 0.4407 1.1726 1.0376
Oxindole 1.5579 1.1689 0.9444 0.8465
Palmitamide 1.1352 1.1894 1.0083 0.9178
Palmitoleoyl ethanolamide 0.9321 0.9325 1.0195 1.0140
Palmitoylcarnitine 1.4441 2.1370 1.0442 0.6306
Phenylalanine 0.5536 0.5683 1.0347 1.1800
Phe Phe 1.4455 1.7556 0.8502 0.8794
Piperine 1.8717 1.0082 0.7342 0.9748
Proline 0.8048 0.9721 0.9818 1.0851
Ranitidine 0.0025 NA 2.1968 0.0027
Sotalol 0.5610 0.6600 1.1682 1.0336
Sphingosine-1-phosphate 1.5495 1.4802 0.9032 0.8390
Theobromine 2.2462 2.3012 0.6498 0.7270
Theophylline 1.7177 1.7225 0.7904 0.8548
TMAO 1.5290 0.9835 0.9637 0.8672
Trigonelline 1.8478 1.6437 0.5977 1.0120
Tryptophan 0.9959 1.3067 0.9569 0.9936
Tyrosine 0.7397 0.7653 1.1151 1.0104
Urea 1.0978 1.1857 0.9339 1.0025
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Factors:

F1Treatment Group:Healthy | Gender:Female
F2Treatment Group:Healthy | Gender:Male
F3Treatment Group:Wilson disease | Gender:Female
F4Treatment Group:Wilson disease | Gender:Male
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