Quantification of glycosaminoglycans in urine by isotope-dilution liquid chromatography-electrospray ionization tandem mass spectrometry.
Published
Journal Article
Mucopolysaccharidoses (MPSs) are complex lysosomal storage disorders that result in the accumulation of glycosaminoglycans (GAGs) in urine, blood, and tissues. Lysosomal enzymes responsible for GAG degradation are defective in MPSs. GAGs including chondroitin sulfate (CS), dermatan sulfate (DS), heparan sulfate (HS), and keratan sulfate (KS) are disease-specific biomarkers for MPSs. This unit describes a stable isotope dilution-tandem mass spectrometric method for quantifying CS, DS, and HS in urine samples. The GAGs are methanolyzed to uronic or iduronic acid-N-acetylhexosamine or iduronic acid-N-sulfo-glucosamine dimers and mixed with internal standards derived from deuteriomethanolysis of GAG standards. Specific dimers derived from HS, DS, and CS are separated by ultra-performance liquid chromatography (UPLC) and analyzed by electrospray ionization tandem mass spectrometry (MS/MS) using selected reaction monitoring for each targeted GAG product and its corresponding internal standard. This new GAG assay is useful for identifying patients with MPS types I, II, III, VI, and VII.
Full Text
Duke Authors
Cited Authors
- Zhang, H; Young, SP; Millington, DS
Published Date
- 2013
Published In
Volume / Issue
- Chapter 17 /
Start / End Page
- Unit - 17.12
PubMed ID
- 23315926
Pubmed Central ID
- 23315926
Electronic International Standard Serial Number (EISSN)
- 1934-8258
Digital Object Identifier (DOI)
- 10.1002/0471142905.hg1712s76
Language
- eng
Conference Location
- United States