Peng Pan1, Mu Chen2, Zhiling Zhang2, Amauri Dalla Corte3,4, Carolina Souza3, Roberto Giugliani3,4,5,6, Luying Pan1, Yongchang Qiu1, Lakshmi Amaravadi1, Jiang Wu1. 1. Shire, 300 Shire Way, Lexington, MA 02421, USA. 2. Frontage Laboratories, Inc., 700 Pennsylvania Drive, Exton, PA 19341, USA. 3. Medical Genetics Service, HCPA, Porto Alegre, RS 90035-903, Brazil. 4. Post-Graduate Program in Medical Sciences, UFRGS, RS 90035-903, Brazil. 5. Post-Graduate Program in Genetics & Molecular Biology, UFRGS, Porto Alegre, RS 91501-970, Brazil. 6. Department of Genetics, UFRGS, Porto Alegre, RS 91501-970, Brazil.
Abstract
AIM: The study aimed to develop an LC-MS/MS assay to measure dermatan sulfate (DS) in human cerebrospinal fluid (CSF). METHODS & RESULTS: 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. CONCLUSION: 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.
AIM: The study aimed to develop an LC-MS/MS assay to measure dermatan sulfate (DS) in human cerebrospinal fluid (CSF). METHODS & RESULTS: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. CONCLUSION: The described assay is capable of differentiating DS levels in the CSF of MPS IIpatients from controls and can be used to monitor disease progression and therapeutic responses.
Authors: Jean-Philippe F Gourdine; Matthew H Brush; Nicole A Vasilevsky; Kent Shefchek; Sebastian Köhler; Nicolas Matentzoglu; Monica C Munoz-Torres; Julie A McMurry; Xingmin Aaron Zhang; Peter N Robinson; Melissa A Haendel Journal: Database (Oxford) Date: 2019-01-01 Impact factor: 3.451
Authors: Junhua Wang; Akhil Bhalla; Julie C Ullman; Meng Fang; Ritesh Ravi; Annie Arguello; Elliot Thomsen; Buyankhishig Tsogtbaatar; Jing L Guo; Lukas L Skuja; Jason C Dugas; Sonnet S Davis; Suresh B Poda; Kannan Gunasekaran; Simona Costanzo; Zachary K Sweeney; Anastasia G Henry; Jeffrey M Harris; Kirk R Henne; Giuseppe Astarita Journal: Int J Mol Sci Date: 2020-07-30 Impact factor: 5.923