Table 1.
Performance of the MS/MS assays of GALNS, ARSB, and I2S.
| Assay | MS/MS product response (peak area) | Analytical rangea | Mean (%CV) activity for 5 replicates of the QC DBS, μmol · h−1 · L−1a |
|---|---|---|---|
| GALNS FIA-MS/MS with β-NGA | Filter paper, 17 340 PE QC H, 220 050 |
23.4 (PE QC H) | No data |
| GALNS LC-MS/MS without β-NGA | Filter paper, 525 CDC QC H, 138 460 PE QC H, 230 905 |
120 (CDC QC H) 198 (PE QC H) |
Filter paper, 0.02 (9.1%) CDC QC B, 0.08 (1.8%) CDC QC L, 0.22 (8.7%) CDC QC M, 1.36 (12.8%) CDC QC H, 2.46 (12.3%) PE QC L, 0.29 (19.0%) PE QC M, 2.70 (12.0%) PE QC H, 4.07 (7.2%) |
| ARSB FIA-MS/MS with β-NGA | Filter paper, 20 520 PE QC H, 360 020 |
22.4 (PE QC H) | No data |
| ARSB LC-MS/MS without β-NGA | Filter paper, 2750 CDC QC H, 1 209 484 PE QC H, 1 406 271 |
170 (CDC QC H) 226 (PE QC H) |
Filter paper, 0.12 (6.1%) CDC QC B, 0.22 (3.6%) CDC QC L, 1.02 (10.2%) CDC QC M, 11.76 (6.4%) CDC QC H, 20.51 (11.2%) PE QC L, 1.96 (9.5%) PE QC M, 18.11 (5.7%) PE QC H, 27.39 (3.7%) |
| I2S FIA-MS/MS with IDUA | Filter paper, 9902 CDC QC H, 1 379 295 PE QC H, 3 297 559 |
257 (CDC QC H) 387 (PE QC H) |
Filter paper, 0.05 (63.9%) CDC QC B, 1.05 (6.2%) CDC QC L, 1.72 (4.8%) CDC QC M, 8.50 (7.2%) CDC QC H, 11.64 (6.1%) PE QC L, 1.17 (3.8%) PE QC M, 9.90 (3.5%) PE QC H, 17.50 (2.8%) |
| I2S LC-MS/MS with IDUA | Filter paper, 4146 CDC QC H, 2 207 739 PE QC H, 3 104 262 |
424 (CDC QC H) 721 (PE QC H) |
Filter paper, 0.03 (60%) CDC QC B, 1.31 (6.3%) CDC QC L, 2.19 (4.3%) CDC QC M, 10.35 (2.7%) CDC QC H, 12.93 (6.4%) PE QC L, 1.34 (7.0%) PE QC M, 11.42 (5.8%) PE QC H, 19.40 (3.3%) |
| I2S LC-MS/MS without IDUA | Filter paper, 1756 CDC QC H, 1 578 629 PE QC H, 1 905 935 |
521 (CDC QC H) 681 (PE QC H) |
Filter paper, 0.04 (145.7%) CDC QC B, 1.10 (6.4%) CDC QC L, 2.20 (3.6%) CDC QC M, 12.60 (4.7%) CDC QC H, 18.66 (5.6%) PE QC L, 1.53 (2.2%) PE QC M, 14.29 (3.1%) PE QC H, 24.41 (6.4%) |
a
Values calculated as described in the online Data Supplement. QC samples from the CDC are base pool (B, 0% whole blood), low (L, 5% whole blood), medium (M, 50% whole blood), and high (H, 100% whole blood). Same for the PerkinElmer (PE) samples except there is no base pool.