A Novel LC–MS/MS Assay to Quantify Dermatan Sulfate in Cerebrospinal Fluid as a Biomarker for Mucopolysaccharidosis II

• DOI: 10.4155/bio-2018-0025
• Publication Date: 2018-06-04T16:40:22Z
• Authors: Peng Pan; Mu Chen; Zhiling Zhang; Amauri Dalla Corte; Carolina Souza; Roberto Giugliani; Luying Pan; Yongchang Qiu; Lakshmi Amaravadi; Jiang Wu
• Keywords: 0303 health sciences; Swine; Dermatan Sulfate; Reproducibility of Results; Clinical Chemistry Tests; 3. Good health; 03 medical and health sciences; Tandem Mass Spectrometry; Calibration; Animals; Biomarkers; Chromatography, Liquid; Mucopolysaccharidosis II

The study aimed to develop an LC-MS/MS assay to measure dermatan sulfate (DS) in human cerebrospinal fluid (CSF).DS was quantified by ion pairing LC-MS/MS analysis of the major disaccharides derived from chondroitinase B digestion. Artificial CSF was utilized as a surrogate for calibration curve preparation. The assay was fully validated, with a linear range of 20.0-4000 ng/ml, accuracy within ±20%, and precision of ≤20%. CSF samples from mucopolysaccharidoses (MPS) II patients showed an average of 11-fold increase in DS levels compared with controls.The described assay is capable of differentiating DS levels in the CSF of MPS II patients from controls and can be used to monitor disease progression and therapeutic responses.