Synthesis of a deuterated disaccharide internal standard for LC-MS/MS quantitation of heparan sulfate in biological samples

This is the authors' abstract. We don't add key points for this paper.

High levels of heparan sulfate (HS) are a marker for several mucopolysaccharidosis (MPS) disorders which are lysosomal storage diseases caused by genetic defects in HS-degrading enzymes. Quantitation of HS in biological samples is therefore critical for diagnosis and evaluating the efficacy of new therapies. Herein we present the efficient synthesis of a butylated GlcN-GlcA disaccharide and its deuterated derivative for use as an internal standard in a quantitative mass spectrometry-based assay for analysis of HS following butanolysis. The synthesis features the stereoselective 1,2- cis glycosylation of a GlcA acceptor with a 6- O -benzoyl-2-deoxy-2-azido thioglucoside donor. • Heparan sulfate (HS) is a marker for several mucopolysaccharidosis (MPS) disorders. • HS quantitation by LC-MS/MS requires a deuterated internal standard. • Efficient synthesis of butylated GlcN-GlcA-d 9 as an internal standard.