Table 5.
ALA and PBG concentrations in various non-CNS and CNS tissues
| Tissue |
Wild type
c
(Sv129/C57Bl6) ALA, PBG |
R167Q
+/+
(Sv129/C57Bl6) ALA, PBG |
Fold change over wild type |
Wild type
d
(C57Bl6) ALA, PBG |
T1/T2
(C57Bl6) ALA, PBG |
Fold change over wild type |
|---|---|---|---|---|---|---|
| Non-CNS tissues and cells | ||||||
| Liver | 10.1 ± 2.6, 2.4 ± 0.9 |
19.0 ± 2.9, 934 ± 225 |
1.9 390 |
6 ± 2.5, UDL |
7.1 ± 0.6, 2.6 ± 0.6 |
1.2 N/A |
| Kidney | 22.6 ± 12.5, 8.0 ± 4.5 |
38.3 ± 4.6, 582 ± 245 |
1.7 73 |
ND, ND |
ND, ND |
N/A N/A |
| Heart | 34.6 ± 10.0, UDL |
87.6 ± 13.4, 1,006 ± 20.8 |
2.5 N/A |
ND, ND |
ND, ND |
N/A N/A |
| Spleen | 36.2 ± 12.3, 3.4 ± 0.5 |
53.8 ± 5.3, 376 ± 176 |
1.5 110 |
ND, ND |
ND, ND |
N/A N/A |
| Erythrocytes | 1.8 ± 0.6, 1.2 ± 0.2 |
2.7 ± 0.8, 45.3 ± 11.9 |
1.5 38 |
ND, ND |
ND, ND |
N/A N/A |
| CNS tissues and fluids | ||||||
| Brain (whole) | 6.2 ± 5.6 UDL |
33.7 ± 4.4, 886 ± 118 |
5.4 N/A |
5.6 ± 1.8, UDL |
6.4 ± 1.7, 23.6 ± 5.1 |
1.1 N/A |
| Cerebrum | 6.5 ± 2.5 UDL |
29.1 ± 10.7, 856 ± 403 |
4.5 N/A |
ND, ND |
ND, ND |
N/A N/A |
| Cerebellum | 7.9 ± 4.0 UDL |
30.3 ± 6.8, 687 ± 96.6 |
3.8 N/A |
ND, ND |
ND ND |
N/A N/A |
| Upper brain stema | 10.9 ± 5.7 UDL |
43.2 ± 19.2, 1,162 ± 211 |
4 N/A |
ND, ND |
ND, ND |
N/A N/A |
| Lower brain stemb | 16.5 ± 10.5 UDL |
35.7 ± 7.9, 1,124 ± 32 |
2.2 N/A |
ND, ND |
ND, ND |
N/A N/A |
| Spinal cord | 7.3 ± 0.3, UDL |
68 ± 5.4, 1,759 ± 391 |
9.3 N/A |
9.8 ± 0.3, UDL |
25.6 ± 4.8, 306 ± 57 |
2.6 N/A |
| CSF | 0.07 ± 0.01, 0.12 ± 0.07 |
0.15 ± 0.04, 8.9 ± 5.1 |
2.1 74 |
0.07 ± 0.01 0.12 ± 0.07 |
0.09 ± 0.01, 1.73 ± 0.25 |
1.3 14 |
UDL, under detection limit of 0.05 μmol/L; ND, not determined; N/A, not applicable
a,bUpper and lower brain stems include midbrain—pons and the medulla, respectively.
c,dR167Q+/+ and T1/T2 controls are Sv129/C57Bl6 and C57Bl6 wild-type mice, respectively.
Data presented are means ± SDs (n ≥ 4) expressed as pmol/mg protein in tissues and μmol/L in CSF.