Isolation and characterization of peptidoglycans in urine from patients with mucopolysaccharidoses

Abstract

1. 1. Urinary dermatan sulfate (DS) and heparan sulfate (HS) were purified from mucopolysaccharidosis patients. 2. 2. DS shows average mol. wt 8600–12,000 (approx. one half of tissue DS), iduronic acid content 99.1–99.6% (81.8% in tissue DS), core peptide mostly di- or tri-peptide (—Ser—Gly— or —Ser—Gly—Glu—). 3. 3. Molecular weight of HS ranged from 2500 to 20,000, averaging about 5000. 4. 4. Highly sulfated HS was found in the low molecular weight fraction, and no bound core peptide. 5. 5. By contrast, HS in the high molecular weight fraction bound one sulfate per repeating unit, which include core peptide.