Table 2.
Concentrations of serum amino acid/thiol compounds, urinary thiol compounds, and liver glutathione in adult wild-type, Mpst (1st–3rd)-KO, Cth-KO, and Mpst (1st)/Cth-DKO mice.
| Mpst | Mpst (1st)/Cth | |||||
|---|---|---|---|---|---|---|
| Mice | WT (a) | 1st KO (b) | 2nd KO | 3rd KO | Cth-KO (c) | -DKO (d) |
| Serum (µM) | (n = 10) | (n = 7) | (n = 7) | (n = 7) | (n = 5) | (n = 10) |
| Ala | 479 ± 128 | 377 ± 56 | 439 ± 79 | 430 ± 88 | 574 ± 239 | 407 ± 88 |
| Arg | 120 ± 19 c | 104 ± 23 c | 120 ± 14 | 113 ± 24 | 168 ± 37 abd | 146 ± 26 c |
| Asn/Asp | 6.28 ± 2.66 c | 9.33 ± 6.68 c | 17.4 ± 9.4 | 6.01 ± 4.41 | 28.1 ± 17.8 abd | 14.3 ± 6.4 c |
| Gln | 807 ± 135 | 752 ± 133 | 762 ± 74 | 801 ± 35 | 935 ± 172 d | 654 ± 147 c |
| Glu | 27.4 ± 21.4 c | 26.1 ± 17.9 c | 36.7 ± 15.7 | 19.9 ± 8.1 | 54.5 ± 17.9 ab | 39.2 ± 10.7 |
| Gly | 328 ± 75 | 291 ± 38 | 352 ± 28 | 306 ± 64 | 368 ± 109 | 345 ± 49 |
| His | 42.0 ± 24.5 cd | 44.2 ± 23.3 cd | 85.1 ± 19.1 | 39.3 ± 20 | 131 ± 58 ab | 90.4 ± 10.4 ab |
| Ile | 109 ± 18 | 106 ± 18 | 113 ± 21 | 105 ± 14 | 123 ± 20 | 106 ± 20 |
| Leu | 159 ± 21 | 155 ± 22 | 170 ± 26 | 169 ± 15 | 196 ± 11 d | 156 ± 32 c |
| Lys | 217 ± 37 | 226 ± 42 | 211 ± 48 | 255 ± 33 | 298 ± 24 | 184 ± 44 |
| Met | 75.3 ± 19.4 | 69.9 ± 16.7 c | 86.9 ± 8.0 | 67.6 ± 11.0 | 104 ± 25 b | 96.6 ± 22 |
| Phe | 95.6 ± 26.9 | 79.5 ± 13.9 | 86.0 ± 7.0 | 89.8 ± 6.2 | 106 ± 23 | 83.3 ± 13.4 |
| Pro | 131 ± 68 | 91.2± 26.7 | 120 ± 30 | 94.5 ± 28.4 | 177 ± 89 | 135 ± 36 |
| Ser | 145 ± 58 | 120 ± 18 | 143 ± 18 | 125 ± 29 | 175 ± 62 | 141 ± 30 |
| Thr | 175 ± 42 | 151 ± 35 | 180 ± 21 | 163 ± 45 | 199 ± 35 | 194 ± 43 |
| Trp | 71.2 ± 17.1 | 73.9 ± 11.1 | 61.5 ± 16.5 | 62.9 ± 12.0 | 69.9 ± 9.6 | 82.9 ± 22.3 |
| Tyr | 94.8 ± 32.0 | 87.6 ± 25.0 | 110 ± 18 | 101 ± 26 | 99.2 ± 58.9 | 80.3 ± 30.4 |
| Val | 242 ± 58 | 225 ± 40 c | 253 ± 29 | 226 ± 27 | 304 ± 43 b | 251 ± 41 |
| Cystathionine | 44.1 ± 19.7 cd | 39.5 ± 12.8 cd | 56.2 ± 12.6 | 37.6 ± 8.1 | 124 ± 15 ab | 133 ± 32 ab |
| Citrulline | 79.2 ± 19.3 cd | 63.0 ± 15.2 cd | 68.6 ± 14.1 | 67.2 ± 12.7 | 169 ± 24 ab | 135 ± 34 ab |
| Ornithine | 42.1 ± 10.9 c | 35.1 ± 10.2 c | 35.7 ± 6.6 | 43.0 ± 16.2 | 66.4 ± 19.1 ab | 49.5 ± 13.8 |
| Taurine | 675 ± 349 | 491 ± 136 | 642 ± 181 | 514 ± 88 | 601 ± 240 | 664 ± 235 |
| Total Cys | 319 ± 40 d | 322 ± 52 d | 341 ± 78 | 315 ± 32 | 274 ± 11 | 261 ± 51 ab |
| Total Hcy | 5.78 ± 1.22 cd | 5.90 ± 2.05 cd | 5.58 ± 0.77 | 6.23 ± 1.11 | 83.6 ± 18.6 abd | 121 ± 15 abc |
| Total GSH | 252 ± 71 cd | 230 ± 141 d | 221 ± 42 | 224 ± 37 | 124 ± 59 a | 107 ± 42 ab |
| Total Cys-Gly | 3.23 ± 0.39 b | 4.43 ± 0.59 ac | 4.79 ± 0.55 | 3.93 ± 0.39 | 3.15 ± 0.93 b | 3.87 ± 0.86 |
| Total γ Glu-Cys | 10.4 ± 2.8 | 13.2 ± 4.0 cd | 12.1 ± 2.4 | 8.37 ± 1.94 | 8.17 ± 0.45 b | 8.32 ± 2.16 b |
| Urine (mmol/mol creatinine) | ||||||
| (n = 10) | (n = 9) | (n = 7) | (n = 7) | (n = 6) | (n = 9) | |
| 3-ML | 10.4 ± 2.0 bd | 61.3 ± 9.9 acd | 57.4 ± 29.4 | 75.9 ± 20.7 | 16.9 ± 3.2 bd | 91.2 ± 21.0 abc |
| Total Cys | 156 ± 28 cd | 187 ± 24 cd | 222 ± 50 | 211 ± 84 | 624 ± 272 ab | 601 ± 115 ab |
| Total Hcy | 7.32 ± 2.66 cd | 6.24 ± 2.81 cd | 6.15 ± 1.11 | 5.44 ± 1.24 | 169 ± 51 ab | 195 ± 69 ab |
| Total GSH | 2.00 ± 1.28 | 1.40 ± 0.81 | 2.31 ± 1.09 | 1.74 ± 0.44 | 2.67 ± 1.84 | 2.15 ± 1.09 |
| Total Cys-Gly | 26.6 ± 10.0 cd | 25.4 ± 7.1 cd | 41.3 ± 9.6 | 32.4 ± 12.0 | 85.8 ± 37.7 ab | 82.4 ± 33.9 ab |
| Total γ Glu-Cys | 10.2 ± 4.9 cd | 9.45 ± 3.70 cd | 21.9 ± 7.0 | 14.3 ± 3.5 | 24.3 ± 13.0 ab | 26.8 ± 7.7 ab |
| Liver | ||||||
| Total GSH (nmol/mg protein; n = 5 each) | ||||||
| 10.6 ± 1.5 cd | 10.9 ± 1.7 cd | N.T. | N.T. | 6.51 ± 0.75 ab | 6.25 ± 1.25 ab | |
| GSSG/tGSH (%; n = 5 each) | ||||||
| 4.04 ± 0.48 cd | 3.82 ± 0.48 cd | N.T. | N.T. | 5.42 ± 0.62 ab | 5.69 ± 0.89 ab | |
Means ± SD are presented (n: sample numbers). N.T., not tested. All parameters were similar in Mpst-KO mice lines; therefore, statistical analyses were done between WT, Mpst (1st)-KO, Cth-KO, and Mpst (1st)/Cth-DKO mice by one-way ANOVA with Tukey’s multiple comparison test. Significant differences (p < 0.05) versus WT (a), Mpst (1st)-KO (b), Cth-KO (c), and Mpst (1st)/Cth-DKO mice (d).