Table 1.
List of Acyl-carnitines, Amino Acids, Biogenic Amines, and Sugar Included in This Study
| Analyte Description | Control | Knockout | P Value | ||||
|---|---|---|---|---|---|---|---|
| Metabolite Class | Short Name | Biochemical Name | Mean | SD | Mean | SD | t Test |
| Calculated total carnitine (C0 to C18) | 143.83 | 26.740 | 160.99 | 25.590 | |||
| Calclated total acyl-carnitines (C2 to C18) | 40.06 | 7.631 | 31.81 | 9.145 | 0.01 | ||
| Acyl/free ratio | 0.392 | 0.073 | 0.254 | 0.082 | <0.001 | ||
| Summed short-chain acyl-carnitines (C3 to C5) | 2.568 | 0.6889 | 3.312 | 0.8628 | 0.01 | ||
| Summed medium-chain acyl-carnitines (C6 to C12) | 6.000 | 0.200 | 0.680 | 0.148 | |||
| Summed long-chain acyl-carnitines (C14 to C18) | 0.6502 | 0.3473 | 0.5409 | 0.2713 | |||
| Acyl-carnitines | C0 | dl-Carnitine (free carnitine) | 104 | 21.5 | 129‡ | 26.5 | 0.009 |
| C10 | Decanoyl-l-carnitine | 0.068* | 0.029 | 0.071* | 0.017 | ||
| C10.1 | Decenoyl-l-carnitine | 0.067* | 0.032 | 0.076* | 0.026 | ||
| C10.2 | Decadienyl-l-carnitine | 0.025* | 0.013 | 0.031* | 0.010 | ||
| C12 | Dodecanoyl-l-carnitine | 0.042* | 0.019 | 0.050* | 0.019 | ||
| C12.DC | Dodecanedioyl-l-carnitine | 0.11* | 0.047 | 0.12* | 0.028 | ||
| C12.1 | Dodecenoyl-l-carnitine | 0.065* | 0.031 | 0.008* | 0.026 | ||
| C14 | Tetradecanoyl-l-carnitine | 0.025* | 0.0075 | 0.023* | 0.0080 | ||
| C14.1 | Tetradecenoyl-l-carnitine | 0.016 | 0.0062 | 0.021 | 0.011 | ||
| C14.1.OH | Hydroxytetradecenoyl-l-carnitine | 0.015 | 0.0050 | 0.012* | 0.0024 | ||
| C14.2 | Tetradecadienyl-l-carnitine | 0.006* | 0.002 | 0.006* | 0.001 | ||
| C14.2.OH | Hydroxytetradecadienyl-l-carnitine | 0.01* | 0.002 | 0.01* | 0.001 | ||
| C16 | Hexadecanoyl-l-carnitine | 0.066† | 0.040 | 0.043† | 0.028 | ||
| C16.OH | Hydroxyhexadecanoyl-l-carnitine | 0.020 | 0.0078 | 0.021 | 0.0097 | ||
| C16.1 | Hexadecenoyl-l-carnitine | 0.052* | 0.016 | 0.056* | 0.021 | ||
| C16.1.OH | Hydroxyhexadecenoyl-l-carnitine | 0.021 | 0.011 | 0.022 | 0.014 | ||
| C16.2 | Hexadecadienyl-l-carnitine | 0.01 | 0.004 | 0.01 | 0.004 | ||
| C16.2.OH | Hydroxyhexadecadienyl-l-carnitine | 0.01* | 0.004 | 0.01* | 0.003 | ||
| C18 | Octadecanoyl-l-carnitine | 0.03† | 0.0080 | 0.022† | 0.012 | ||
| C18.1 | Octadecenoyl-l-carnitine | 0.20 | 0.14 | 0.11 | 0.095 | ||
| C18.1.OH | Hydroxyoctadecenoyl-l-carnitine | 0.042 | 0.022 | 0.046 | 0.027 | ||
| C18.2 | Octadecadienyl-l-carnitine | 0.13 | 0.10 | 0.12 | 0.085 | ||
| C2 | Acetyl-l-carnitine | 36.2‡ | 7.27 | 27.3 | 9.28 | 0.007 | |
| C3 | Propionyl-l-carnitine | 0.428 | 0.117 | 0.475 | 0.226 | ||
| C3.DC, C4.OH | Malonyl-l-carnitine/hydroxybutyryl-l-carnitine | 0.992 | 0.369 | 1.45 | 0.538 | 0.01 | |
| C3.OH | Hydroxypropionyl-l-carnitine | 0.033* | 0.0048 | 0.036* | 0.097 | ||
| C3.1 | Propenoyl-l-carnitine | 0.009* | 0.003 | 0.01* | 0.002 | ||
| C4 | Butyryl-l-carnitine | 0.188 | 0.0510 | 0.381 | 0.126 | <0.001 | |
| C4.1 | Butenyl-l-carnitine | 0.030* | 0.0078 | 0.047 | 0.016 | 0.001 | |
| C5 | Valeryl-l-carnitine | 0.14 | 0.040 | 0.14 | 0.030 | ||
| C5.DC. C6.OH | Glutaryl-l-carnitine/hydroxyhexanoyl-l-carnitine | 0.10 | 0.032 | 0.12 | 0.028 | ||
| C5.M.DC | Methylglutaryl-l-carnitine | 0.015* | 0.0029 | 0.015 | 0.0021 | ||
| C5.OH, C3.DC.M | Methylmalonyl-l-carnitine/hydroxyvaleryl-l-carnitine | 0.559 | 0.101 | 0.557 | 0.108 | ||
| C5.1 | Tiglyl-l-carnitine | 0.059 | 0.012 | 0.076 | 0.011 | 0.001 | |
| C5.1.DC | Glutaconyl-l-carnitine | 0.013* | 0.0021 | 0.015* | 0.0030 | ||
| C6, C4.1.DC | Fumaryl-l-carnitine/hexanoyl-l-carnitine | 0.036* | 0.01 | 0.037* | 0.0069 | ||
| C6.1 | Hexenoyl-l-carnitine | 0.021* | 0.0046 | 0.030* | 0.0077 | 0.001 | |
| C7.DC | Pimelyl-l-carnitine | 0.044 | 0.018 | 0.058 | 0.016 | 0.04 | |
| C8 | Octanoyl-l-carnitine | 0.0916* | 0.0101 | 0.0933* | 0.00969 | ||
| C8.1 | Octenoyl-l-carnitine | n.d. | n.d. | n.d. | n.d. | ||
| C9 | Nonayl-l-carnitine | 0.033* | 0.016 | 0.037* | 0.014 | ||
| Ala | Alanine | 769 | 125 | 793 | 118 | ||
| Arg | Arginine | 101 | 12.1 | 115 | 20.7 | 0.03 | |
| Asn | Asparagine | 56.8 | 25.1 | 77.8 | 40.2 | ||
| Amino acids | Asp | Aspartate | n.d. | n.d. | n.d. | n.d. | |
| Cit | Citrulline | 40.9 | 12.7 | 29.0 | 7.42 | 0.005 | |
| Gln | Glutamine | 1783‡ | 436.0 | 1360 | 298.6 | 0.005 | |
| Glu | Glutamate | n.d. | n.d. | n.d. | n.d. | n.d. | |
| Gly | Glycine | 226 | 36.9 | 499 | 78.5 | <0.001 | |
| His | Histidine | 76.6 | 13.8 | 58.2 | 8.81 | <0.001 | |
| Ile | Isoleucine | 59.1 | 7.79 | 73.8 | 13.1 | 0.001 | |
| Leu | Leucine | 118 | 15.4 | 138 | 25.7 | 0.01 | |
| Lys | Lysine | 162 | 38.9 | 207 | 59.0 | 0.02 | |
| Met | Methionine | 39.4 | 6.29 | 47.9 | 10.4 | 0.01 | |
| Orn | Ornithine | 5.20 | 3.65 | 9.84 | 4.97 | 0.008 | |
| Phe | Phenylalanine | 60.7 | 11.8 | 68.7 | 12.2 | ||
| Pro | Proline | 76.3 | 12.0 | 100. | 15.6 | <0.001 | |
| Ser | Serine | 170. | 38.1 | 247 | 51.4 | <0.001 | |
| Thr | Threonine | 108 | 12.9 | 153 | 34.8 | <0.001 | |
| Trp | Tryptophan | 16.9 | 2.77 | 20.0 | 4.37 | 0.03 | |
| Tyr | Tyrosine | 56.1 | 12.9 | 65.7 | 13.4 | ||
| Val | Valine | 93.5 | 13.0 | 127 | 23.6 | <0.001 | |
| Sugar | H1 | Hexose | 667 | 189 | 424 | 202 | 0.002 |
| Biogene amines | Creatinine | Creatinine | 25.5 | 4.38 | 23.4 | 3.55 | |
| ADMA | Asymetric dimethylarginine | 0.854 | 0.268 | 0.958 | 0.212 | ||
| Ac-Orn | Acetylornithine | 2.79 | 0.97 | 3.53 | 1.03 | ||
| Carnosine | Carnosine | 2.95 | 1.20 | 2.40 | 1.42 | ||
| DOPA | 0.940 | 0.597 | 0.725 | 0.352 | |||
| Histamine | Histamine | 0.557 | 0.182 | 1.42 | 0.842 | 0.001 | |
| Kynurenine | Kynurenine | 0.229 | 0.129 | 0.303 | 0.151 | ||
| PEA | Phenylethylamine | n.d. | n.d. | n.d. | n.d. | ||
| Putrescine | Putrescine | 1.67 | 0.536 | 1.882 | 0.408 | ||
| Serotonin | Serotonin | 0.654 | 0.157 | 0.323 | 0.081 | <0.001 | |
| Spermidine | Spermidine | 19.8 | 3.76 | 22.44‡ | 5.19 | ||
| Spermine | Spermine | n.d. | n.d. | n.d. | n.d. | ||
| α-AAA | α-Aminoadipic acid | n.d. | n.d. | n.d. | n.d. | ||
| Total DMA | Total dimethylarginine | 0.434† | 0.152 | 0.502† | 0.102 | ||
The quantification range was determined by BIOCRATES and adopted from the manual: “AbsoluteIDQ p150 kit—Analytical Specifications” (BIOCRATES Life Sciences AG, Innsbruck, Austria). Total carnitine was defined as the concentration of all acyl-carnitines measured plus free carnitine. Total acyl-carnitine was defined as the concentration of all measured acyl-carnitines. The acyl/free ratio was defined as the sum of the concentration of all measured acyl-carnitines divided by the concentration of free carniti. n.d. indicates not detected.
*
Quantification range: *Mean below LOD (limit of detection)
†
Mean below LLOQ (lower limit of quantification)
‡
Mean below ULOQ (upper limit of quantification).