Abstract
Summary 1. Acid mucopolysaccharides (AMPS) were isolated from the urine of 23 patients with mucopolysaccharidoses, 8 with Type I disease (Hurler syndrome), 2 with Type II (Hunter syndrome) and 13 with Type III (Sanfilippo syndrome). 2. The AMPS-uronic acid excreted by all of the patients with mucopolysaccharidoses was significantly higher than that of normals and higher in Type I than in Type III patients. 3. The AMPS excreted were chromatographed on an anion exchange resin column. A distinctive pattern of AMPS distribution was seen with each Type of mucopolysaccharidoses permitting the differentiation of the various Types from each other as well as normals. 4. The fractions of AMPS isolated by anion exchange chromatography were characterized chemically and the size of AMPS was assessed by gel filtration chromatography. The dermatan sulfate and heparitin sulfate which appear in excess in the urine varied both in molecular size and degree of sulfation and they probably represent a series of degradation products of the native mucopolysaccharide polymers.
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