Table 3.
Guanidino compound levels (µM) in serum of 4-month-old untreated Arg1−/−, AAV-mArg1-injected Arg1−/−, and littermate control mice. Values are given as mean ± SD. α-K-δ-GVA = α-keto-δ-guanidinovaleric acid, GSA = guanidinosuccinic acid, CT = creatine, GAA = guanidinoacetic acid, α-N-AA = α-N-acetylarginine, ArgA = argininic acid, CTN = creatinine, γ-GBA = γ-guanidinobutyric acid, Arg = Arginine, HARG = homoarginine, G = guanidine, and MG = methylguanidine.
| αKδ-GVA | GSA | CT | GAA | α-N-AA | ArgA | |
|---|---|---|---|---|---|---|
| Control Littermates | 0.43 ± 0.06 | 0.18 ± 0.05 | 151.67 ± 15.04 | 2.49 ± 0.57 | 1.79 ± 0.40 | 0.10 ± 0.02 |
| Treated Arg1−/− | 1.24 ± 0.92 | 0.06 ± 0.07 | 138.14 ± 74.05 | 1.96 ± 0.88 | 1.65 ± 0.71 | 0.37 ± 0.30 |
| Untreated Arg1−/− | 23.00 ± 4.68 | 0.03 ± 0.03 | 234.60 ± 65.64 | 14.82 ± 6.19 | 13.85 ± 3.94 | 20.46 ± 9.23 |
| p-value Treated* | 0.00000006 | 0.0007 | 0.20 | 0.58 | 0.40 | 0.00001 |
| p-value Untreated* | 0.000002 | 0.0005 | 0.02 | 0.002 | 0.0001 | 0.0009 |
| CTN | γ-GBA | Arg | HArg | G | MG | |
|---|---|---|---|---|---|---|
| Control Littermates | 13.10 ± 2.56 | 0.35 ± 0.08 | 223.0 ± 54.47 | 0.70 ± 0.32 | 3.43 ± 4.42 | 0.08 ± 0.02 |
| Treated Arg1−/− | 10.91 ± 5.33 | 0.44 ± 0.24 | 175.29 ± 69.15 | 1.11 ± 0.76 | 3.21 ± 1.18 | 0.04 ± 0.02 |
| Untreated Arg1−/− | 19.53 ± 10.03 | 0.99 ± 0.48 | 1948.60 ± 489.37 | 4.04 ± 2.20 | 0.36 ± 0.08 | 0.01 ± 0.00 |
| p-value Treated* | 0.95 | 0.003 | 0.50 | 0.04 | 0.80 | 0.02 |
| p-value Untreated* | 0.21 | 0.02 | 0.00003 | 0.009 | 0.21 | 0.0002 |
*
Compared to Heterozygote
Values are expressed as µM.