Blood chemistries for wild-type (+/+), untreated mutant (−/−), and NCS-382-treated mutant (−/−) mice
| Panel | SSADH +/+ (n = 7) | SSADH −/− (untreated,n = 7) | Mutant (treated, n = 5)1-a |
|---|---|---|---|
| Bilirubin1-b | 0.13 ± 0.03 | 0.14 ± 0.03 | not done |
| Alk Phos1-c | 394.6 ± 23.9 | 419.3 ± 20.6 | 127.5 ± 42*** |
| Protein1-d | 4.37 ± 0.09 | 4.46 ± 0.12 | 4.8 ± 0.25 |
| BUN1-b | 20.4 ± 2.1 | 16.7 ± 0.52 | 21 ± 1.2** |
| Creatinine1-b | 0.06 ± 0.02 | 0.07 ± 0.02 | not done |
| Phosphorus1-b | 11.0 ± 0.77 | 10.1 ± 0.30 | 7.05 ± 0.45*** |
| Calcium1-b | 9.2 ± 0.29 | 9.74 ± 0.24 | 7.58 ± 0.14*** |
| Amylase1-c | 2809 ± 217.7 | 1985 ± 63.8 | 1817 ± 59.8 |
| Ala Trans1-c | 217.3 ± 71.9 | 42.0 ± 6.73 | 81.0 ± 21.9* |
| Glucose1-b | 165.1 ± 20.0 | 157.6 ± 8.30 | 159.8 ± 18.4 |
| Cholesterol1-b | 121.6 ± 7.35 | 156.9 ± 7.76 | 121.5 ± 10.71* |
Control (+/+) mice and untreated (−/−) mice = 17 days old; NCS-382-treated (−/−) mice = 80 to 111 days old. It was not possible to age-match untreated (−/−) mice since these animals uniformly died at age 16 to 22 days in the absence of therapeutic intervention. NCS-382-treated mice were on i.p. therapy for the first 30 to 32 days of life, after which the injection of drug was stopped. All values are means ± S.E.M.