TABLE 3.
Control subjects (n = 7)2 |
Partial-OTCD subjects (n = 7)2 |
P values |
||||||
Amino acid | Control | Phenylbutyrate | Control | Phenylbutyrate | Pooled SEM | TRT | Gen | TRT × Gen |
μmol/L | μmol/L | μmol/L | ||||||
Leucine | 135 | 94 | 130 | 95 | 10.8 | <0.001 | <0.869 | <0.608 |
Isoleucine | 61 | 45 | 67 | 53 | 5.5 | <0.001 | <0.364 | <0.852 |
Valine | 216 | 161 | 225 | 164 | 14.2 | <0.001 | <0.751 | <0.653 |
Methionine | 29 | 28 | 36 | 30 | 1.9 | <0.007 | <0.074 | <0.067 |
Cysteine | 30a | 22b | 22a,b | 25a,b | 3.7 | <0.264 | <0.583 | <0.006 |
Phenylalanine | 80 | 72 | 76 | 79 | 3.9 | <0.422 | <0.771 | <0.063 |
Tyrosine | 66 | 80 | 83 | 93 | 6.1 | <0.022 | <0.063 | <0.617 |
Lysine | 204 | 195 | 210 | 193 | 14.3 | <0.047 | <0.935 | <0.524 |
Arginine | 79 | 81 | 67 | 64 | 6.3 | <0.905 | <0.081 | <0.539 |
Ornithine | 49 | 43 | 56 | 47 | 5.4 | <0.006 | <0.451 | <0.519 |
Citrulline | 27 | 23 | 27 | 19 | 3.7 | <0.001 | <0.683 | <0.099 |
Glutamate | 30 | 40 | 55 | 68 | 8.7 | <0.019 | <0.026 | <0.669 |
Glutamine | 571 | 531 | 720 | 680 | 44.6 | <0.081 | <0.017 | <0.985 |
Aspartate | 4 | 2 | 5 | 6 | 1.2 | <0.519 | <0.085 | <0.227 |
Asparagine | 73 | 84 | 83 | 68 | 8.0 | <0.675 | <0.764 | <0.033 |
Ammonia | 12 | 7 | 33 | 20 | 9.2 | <0.002 | <0.193 | <0.131 |
TRT, treatment (control compared with phenylbutyrate treatments); Gen, genotype (control compared with partial-OTCD subjects); TRT × Gen, interaction of main effects. Data were analyzed by using mixed models (fixed effects for TRT, Gen, and TRT × Gen; random effects for subject). Values with different superscript letters differed (Tukey-Kramer–adjusted P < 0.05).
Values are means.