Table 3. Plasma Amino Acids (μmol/L) in patients with propionic acidemia.
Values are averages ± SD of at least 12 observations.
| Amino Acid | Normal | Baseline | Citrate | Glutamine | OKG |
|---|---|---|---|---|---|
| Alanine | 240–600 | 349±85 | 331±91 | 328±79 | 362±65 |
| Arginine | 40–160 | 42.8±11.7 | 36.5±10.2 | 45.3±21.4 | 41.4±10.9 |
| Asparagine | 15–40 | 31.2±13.1 | 27.6±9.9 | 26±9.5 | 29.2±5.0 |
| Aspartate | 0–20 | 11.1±4.6 | 9.9±2.8 | 9.7±2.5 | 11.7±2.8 |
| Citrulline | 10–60 | 19.6±4.2 | 17.5±4.9 | 21.4±7.9 | 21.9±4.8 |
| Cysteine | 7–70 | 34±7.5 | 29.7±6.9 | 33.5±7.1 | 32.1±7 |
| Glutamine | 410–700 | 365±55 | 375±49 | 367±55 | 391±74 |
| Glutamate | 10–120 | 51.2±14.2 | 50.7±15.6 | 49.7±12.6 | 52±7.3 |
| Glycine | 140–490 | 1268±177 | 1173±244 | 1249±229 | 1376±158 |
| Histidine | 50–130 | 76.9±20.3 | 67.7±22.1 | 60.8±20.2 | 71.9±20.4 |
| Isoleucine | 30–130 | 20.7±10.6 | 20.6±9.5 | 20.7±11 | 23.3±9.5 |
| Leucine | 60–230 | 69.5±35.7 | 60.8±21.7* | 64.3±22.6 | 50.1±15.8 |
| Lysine | 80–250 | 227±76 | 226±68 | 244±103 | 224±82 |
| Methionine | 17–53 | 12.6±3 | 13.5±3.6 | 13.9±3.9 | 12.7±2.9 |
| Ornithine | 20–135 | 40.7±16.9 | 32.6±16 | 37.3±12.7 | 94.3±39.2* |
| Phenylalanine | 30–80 | 42.6±9.5 | 41±7 | 39.4±7.9 | 34.3±5.2* |
| Proline | 110–500 | 252±92 | 308±79 | 264±64 | 284±70 |
| Serine | 60–200 | 142±22 | 132±29 | 132±33 | 147± 7 |
| Taurine | 25–80 | 67.8±15.6 | 60.8±8.8 | 61.2±7.1 | 66.5±10.2 |
| Threonine | 60–220 | 65.3±15.2 | 74±26 | 55.4± 4.2 | 54.1±8.7* |
| Tyrosine | 30–120 | 79.8±20.8 | 67.4±20 | 80.1±21 | 66.1±17.6 |
| Valine | 140–350 | 60.7±25.6 | 67.8±28.2 | 64±26.6 | 55.5± 4.8 |
*
p < 0.05 (or better) versus baseline. Values outside the normal range are indicated in bold.