TABLE 4.
ATP levels in controls and premutation PBMCs
| Pathway | Control (n = 11) | FXTAS-free (n = 21) | FXTAS-affected (n = 9) | P |
|---|---|---|---|---|
| Oxphos | 15 ± 4 (67%) | 5.2 ± 0.6 (38%) | 2.9 ± 0.8 (27%) | 0.019 |
| Glycolysis | 7.3 ± 0.9 (33%) | 8.4 ± 0.4 (62%) | 8 ± 2 (73%) | 0.390 |
| Total ATP | 22.3 ± 1.2 | 13.6 ± 0.2 | 10.9 ± 0.2 | 0.0001 |
ATP levels from oxphos were calculated [as nanomoles ATP × (glucose × 106 cells)−1] (means ± sem) (%)] by the oligomycin-sensitive oxygen uptake of glucose-supplemented intact cells and normalized by glucose consumed per million cells. ATP from glycolysis was calculated from the lactate-to-glucose ratio obtained by metabolomics analysis and normalized as above. In parentheses, the percentage of total ATP produced by each pathway. P values were computed by Student’s t test for comparisons between FXTAS-free and -affected individuals.