| Article ID | Journal | Published Year | Pages | File Type |
|---|---|---|---|---|
| 1971457 | Clinical Biochemistry | 2009 | 7 Pages |
ObjectivesPatients with Ehlers–Danlos syndrome were described to contain reduced activities of β4-galactosyltransferase-7 (β4Gal-T7). Therefore, measurement of β4Gal-T7 activity can help to characterize defects in proteoglycan biosynthesis in patients with connective tissue diseases.Design and methodsWe developed a sensitive and specific method to assay β4Gal-T7 which is based on the transfer of galactose from UDP-galactose to the synthetic peptide Bio-BIK-F-Xyl.ResultsCalibration curves exhibited consistent linearity in the range of 10–2000 μg/L Bio-BIK-F-Xyl-Gal, corresponding to a β4Gal-T7 activity of 3.5–659 μU/L. The limit of detection and the lower limit of quantification were 3.70 μg/L (1.22 μU/L) and 4.50 μg/L Bio-BIK-F-Xyl-Gal (1.48 μU/L β4Gal-T7 activity), respectively. Interassay imprecision (CV) was 8.1–13.1% in the range from 15.9 to 659 μU/L, and mean recovery was 85.3% (range 61.7–106.3%).ConclusionsThis sensitive, robust and interference-free LC-MS/MS assay allows an accurate determination of β4Gal-T7 activity in human body fluids.
