Table 4.
Porphyrin precursor and porphyrins in R167Q+/+ and T1/T2 mice
| Body Fluid | Porphyrin Precursor or Porphyrin |
Wild type
c
(Sv129/C57Bl6) |
R167Q
+/+
(Sv129/C57Bl6) |
Fold-change over Wild type |
Wild type
d
(C57Bl6) |
T1/T2
(C57Bl6) |
Fold change over Wild type |
|---|---|---|---|---|---|---|---|
| Plasmaa | ALA | 0.14 ± 0.05 | 0.98 ± 0.32 | 7 | 0.22 ± 0.03 | 0.53 ± 0.19 | 2.4 |
| PBG | UDL | 6.69 ± 2.76 | >134 | 0.08 ± 0.01 | 0.48 ± 0.21 | 6 | |
| Urineb | ALA | 11.53 ± 6.38 | 52.18 ± 28.21 | 4.5 | 14.8 ± 2.87 | 23.84 ± 6.57 | 1.6 |
| PBG | 2.10 ± 1.25 | 127 ± 21.47 | 60 | 0.91 ± 0.22 | 6.10 ± 1.52 | 6.7 | |
| URO I | 0.82 ± 0.83 | 12.15 ± 7.31 | 15 | 0.70 ± 0.32 | 10.99 ± 4.24 | 16 | |
| URO III | 0.32 ± 0.41 | 15.76 ± 11.47 | 49 | 0.18 ± 0.03 | 4.04 ± 1.97 | 22 | |
| 7-COOH I + III | 0.37 ± 0.21 | 1.09 ± 0.61 | 2.9 | 0.22 ± 0.06 | 0.93 ± 0.46 | 4.2 | |
| 6-COOH I + III | 1.18 ± 0.82 | 1.75 ± 0.68 | 1.5 | 0.92 ± 0.29 | 0.83 ± 1.21 | 0.9 | |
| 5-COOH I + III | 1.28 ± 0.90 | 1.91 ± 0.74 | 1.5 | 1.00 ± 0.31 | 2.01 ± 0.66 | 2.0 | |
| COPRO I | 0.86 ± 0.69 | 1.20 ± 0.32 | 1.4 | 0.59 ± 0.18 | 2.40 ± 1.05 | 4.1 | |
| COPRO III | 5.47 ± 3.92 | 6.07 ± 4.21 | 1.1 | 5.66 ± 1.83 | 8.97 ± 4.09 | 1.6 |
UDL, under detection limit of 0.05 μmol/L; URO, uroporphyrin; 7-COOH, monodecarboxylated URO; 6-COOH, didecarboxylated URO; 5-COOH, tridecarboxylated URO; COPRO, coproporphyrin.
aPlasma ALA and PBG are expressed in μmol/L.
bUrinary ALA and PBG are expressed in mmol/mol creatinine, while porphyrins are expressed in μmol/mol creatinine.
c,dR167Q+/+ and T1/T2 controls are Sv129/C57Bl6 and C57Bl6 wild-type mice, respectively.
Data shown are means ± SDs (n ≥ 4).