2014
DOI: 10.4172/2155-9872.s2-006
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Assay for Glycosaminoglycans by Tandem Mass Spectrometry and its Applications

Abstract: Glycosaminoglycans (GAGs) are distributed in the whole body and play a variety of important physiological roles associated with inflammation, growth, coagulation, fibrinolysis, lipolysis, and cell-matrix biology. Accumulation of undegraded GAGs in lysosomes gives rise to a distinct clinical syndrome, mucopolysaccharidoses. Measurement of each specific GAG in a variety of specimens is urgently required to understand GAG interaction with other molecules, physiological status of patients, and prognosis and pathog… Show more

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Cited by 27 publications
(20 citation statements)
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“…The age of control samples ranged between 0 and 80 years (mean 5.2 years). In previous LC-MS/MS experiments, we confirmed that specificity and sensitivity of each GAG value were comparable using plasma or serum [7, 1417, 19]. Diagnosis for MPS patients was confirmed by enzyme assay at each institute providing samples.…”
Section: Methodssupporting
confidence: 73%
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“…The age of control samples ranged between 0 and 80 years (mean 5.2 years). In previous LC-MS/MS experiments, we confirmed that specificity and sensitivity of each GAG value were comparable using plasma or serum [7, 1417, 19]. Diagnosis for MPS patients was confirmed by enzyme assay at each institute providing samples.…”
Section: Methodssupporting
confidence: 73%
“…We have demonstrated 1) that the RapidFire platform is capable of detecting disaccharides of ΔDiHS-0S and ΔDiHS-NS in blood with the same reproducibility, sensitivity, and specificity as described for the LC-MS/MS method [7, 1417, 1922], suggesting that chromatographic separation of disaccharides is not essential for detection and identification of ΔDiHS-0S and ΔDiHS-NS, 2) that HS assays by HT-MS/MS can distinguish untreated patients with MPS II or III from healthy controls, showing the potential use of this technology as a diagnostic platform, 3) that patient groups treated by ERT or HSCT had reduced HS levels, suggesting that HS measured by HT-MS/MS can be used as a biomarker for monitoring therapeutic effect, 4) that the run time of six to eight seconds per sample provides 10–100 fold higher throughput when compared to traditional LC-MS/MS, indicating that HT-MS/MS may be useful for mass screening applications for MPS, and 5) that levels of HS in newborn DBS were elevated in all MPS I, II and III patients, suggesting that HT-MS/MS could be used in a NBS to identify these patients.…”
Section: Discussionmentioning
confidence: 99%
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