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Figure 4 | Molecular Medicine

Figure 4

From: Biochemical Characterization of Porphobilinogen Deaminase-Deficient Mice During Phenobarbital Induction of Heme Synthesis and the Effect of Enzyme Replacement

Figure 4

Plasma PBG clearance following administration of recombinant human PBGD (rhPBGD) to PBGD-deficient mice. Eight male PBGD-deficient mice for each dose were 1st administered daily intraperitoneally with increasing doses of phenobarbital (75 to 90 mg/kg) for 4 d to induce plasma PBG levels. At time zero, the animals were administered with rhPBGD. In (A) the mice were intravenously administered with 3 doses of rhPBGD (0.25, 0.5, or 1.0 mg/kg), and in (B) the animals were subcutaneously administered with 1, 2, or 5 mg/kg rhPBGD. Control animals were injected with phenobarbital and saline. Blood was collected after the 4th phenobarbital administration, and subsequently at different time-points (0.5, 2, and 6 h) after rhPBGD administration. The concentration of PBG was determined using an LC-MS method and expressed in terms of relative levels of plasma PBG ± SD, where 100% was defined as the plasma PBG level after 4 d of phenobarbital administration (8.7 ± 4.4 µmol/L, n = 32). To be able to illustrate the standard deviation for each time-point, the values for 0.25 to 1.0 and 1.0 to 5.0 mg/kg rhPBGD have been shifted +0.05 to 0.15 min.

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