Structural determination of three glycoasparagines isolated from the urine of a patient with aspartylglycosaminuria.
Three different glycoasparagines have been isolated from the urine of a patient with aspartylglycosaminuria and their structures determined using sugar, amino acid and methylation analysis, enzymic degradation and measurements of the optical rotations. The structures were 2-acetamido-1-N-(4'-L-aspartyl)-2-deoxy-beta-D-glucopyranosylamine (yield 135 mg/l) beta-D-galactopyranosyl-(1 leads to 4)-2-acetamido-1-N-(4'-L-aspartyl)-2-deoxy-beta-D-glucopyranosylamine (yield 35 mg/l), and alpha-D-mannopyranosyl-(1 leads to 6)-beta-D-mannopyranosyl-(1 leads to 4)-2-acetamido-2-deoxy-beta-D-glucopyranosyl-(1 leads to 4)-2-acetamido-1-N-(4'-L-aspartyl)-2-deoxy-beta-D-glucopyranosylamine (yield 30 mg/l). The first two compounds have previously been described, whereas the third compound is different from any of the glycoasparagines isolated before.
D000117:Acetylglucosamine / Q000032:analysis; D000328:Adult; D001216:Asparagine / Q000652:urine*; D005260:Female; D005690:Galactose / Q000032:analysis; D006801:Humans; D008358:Mannose / Q000032:analysis; D008661:Metabolism, Inborn Errors / Q000652:urine*; D008968:Molecular Conformation; D009844:Oligosaccharides / Q000652:urine*; D009904:Optical Rotation
D009844:Oligosaccharides; D001216:Asparagine; D008358:Mannose; D000117:Acetylglucosamine; D005690:Galactose