Table 2.
5-Aminolevulinic acid (ALA) and porphobilinogen (PBG) concentrations in tissues, plasma, and urine from PBGD-deficient and control mice with or without phenobarbital administration for 4 d
| Sample | PBGD-deficient mice (n = 6) | PBGD-deficient mice + phenobarbital (n = 9) | Control mice (n = 3) | Control mice + phenobarbital (n = 2) |
|---|---|---|---|---|
| ALA concentration (mean ± SD) | ||||
| Liver | UD | 3.8 ± 1.9 | UD | UD |
| Kidney | UD | 5.6 ± 3.5 | UD | UD |
| Spleen | UD | UD | UD | UD |
| Lung | UD | UD | UD | UD |
| Heart | UD | UD | UD | UD |
| Brain | UD | UD | UD | UD |
| Plasma | 0.54 ± 0.45 (n = 64) | 7.0 ± 3.3 (n = 6) | UD | 0.72 ± 0.05 |
| Urine | 19.5 ± 3.9 | 287 ± 24.2 (n = 3) | 15.7 ± 7.4 | 18.4 ± 1.2 (n = 3) |
| PBG concentration (mean ± SD) | ||||
| Liver | UD | 120 ± 40 | UD | UD |
| Kidney | UD | 27 ± 19 | UD | UD |
| Spleen | UD | UD | UD | UD |
| Lung | UD | UD | UD | UD |
| Heart | UD | UD | UD | UD |
| Brain | UD | UD | UD | UD |
| Plasma | 0.32 ± 0.54 (n = 64) | 12.5 ± 3.1 (n = 6) | 0.23 ± 0.08 | 0.63 ± 0.30 |
| Urine | 3.6 ± 1.6 | 168 ± 20.6 (n = 3) | 1.9 ± 1.9 | 2.6 ± 0.9 (n = 3) |
ALA and PBG in tissues are expressed as pmol/mg tissue, in plasma as μmol/L, and in urine as mmol/mol creatinine (UD = under detection limit, i.e. in tissues ALA < 1.0 and PBG < 3.5 pmol/mg tissue, respectively, and in plasma ALA < 0.1 μmol/L). The porphyrin precursors in tissues and plasma were measured 6 h after the 4th injection of phenobarbital. The urine was collected during 24 h after the 4th injection.