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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

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

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)

  1. 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.