Identification of 4-N-2-acetamido-2-deoxy-beta-D-glucopyranosyl-L-asparagine in biological materials by gas chromatography-mass spectrometry.
A specific and sensitive method for the identification of 4-N-2-acetamido-2-deoxy-beta-D-glucopyranosyl-L-asparagine (GlcNAc-Asn) in urine in aspartylglycosaminuria and in hydrolysates of glycoproteins is described. The method involves permethylation of GlcNAc-Asn followed by gas chromatographic-mass spectrometric analysis of the methylated derivative. It can be used to confirm the diagnosis of aspartylglycosaminuria and to assess the excretion of GlcNAc-Asn in urine during various phases of the disease. The presence of an N-acetylglucosaminyl-asparagine type of carbohydrate-peptide linkage in a glycoprotein can be determined by applying the method to the partial acid hydrolysate of a proteolytically digested glycoprotein.
D000117:Acetylglucosamine / Q000031:analogs & derivatives* / Q000652:urine; D000328:Adult; D001216:Asparagine / Q000031:analogs & derivatives* / Q000652:urine; D002849:Chromatography, Gas; D005260:Female; D005944:Glucosamine / Q000031:analogs & derivatives*; D006020:Glycopeptides / Q000032:analysis; D006801:Humans; D013058:Mass Spectrometry; D009081:Mucolipidoses / Q000652:urine*; D010047:Ovalbumin
D006020:Glycopeptides; C000009:N-acetylglucosaminylasparagine; D001216:Asparagine; D010047:Ovalbumin; D005944:Glucosamine; D000117:Acetylglucosamine