Supplementary material for Lee et al., Proc. Natl. Acad. Sci. USA, 10.1073/pnas.140082197
Table 7.
Nitrogen kinetics in patients with urea cycle disordersSubject | Diagnosis | Urea flux, µmol/kg per h | Glutamine flux, µmol/kg per h | 15 N-urea/15N-glutamine |
Null activity patients | ||||
2001 | ASSD | 45 | 526 | 0 |
2002 | ASSD | 87 | 282 | 0.004 |
2003 | ASSD | 93 | 353 | 0.02 |
2004 | ASSD | 82 | 581 | 0 |
1101 | OTCM | 92 | 479 | 0 |
3001 | ASLD | 84 | 493 | 0 |
Mean |
| 82 | 456 | 0.003 |
SD |
| 17 | 103 | 0.007 |
Partial OTC Males | ||||
1105 | OTCM | 49 | 437 | 0.151 |
1103 | OTCM | 151 | 464 | 0.125 |
1104 | OTCM | 115 | 398 | 0.229 |
1102 | OTCM | 74 | 520 | 0.146 |
Mean |
| 97 | 454 | 0.16 |
SD |
| 45 | 51 | 0.04 |
ASSD, citrullinemia; OTCM, OTC-deficient male; ASLD, argininosuccinic aciduria.