-99 -Glycosaminoglycans (GAG) in normal human plasma and urine were compared after fractionation by exactly same procedures, employing perchloric acid (PCA) -fractionation, phosphotungustic acid (PTA)-fractionation, and Dowex 1 (chloride form) column chromatography, in succession.The resulting fractions were then examined by electrophoresis on cellulose acetate membrane and by digestion with chondroitinases AC and ABC. Following differences were found between the fractions from plasma and urine: 1) plasma contained macromolecular GAG (proteochondroitin sulfate A and hyaluronie acid), which were absent in urine; 2) most of plasma glycoproteins (or glycopeptides) (GP) and GAG were associated with much larger peptides than the corresponding ones from urine; 3) proportion of GP to GAG in plasma was much larger than that in urine; 4) yield (mg/liter) of each subfraction obtained by Dowex 1 column chromatography of GP and GAG from plasma was higher than that of the corresponding one from urine; 5) degree of sulfation of oversulfated chondroitin sulfate(s) in plasma was higher than that in urine. On the other hand, following similarities were observed: 1) plasma and urine contained substantially identical GAG, partially degraded chondroitin sulfate(s), as the major GAG, which were eluted with 1.25 M NaCl from Dowex 1 column; 2) corresponding subfractions from 1.25 M Fr to 2.0 M Fr obtained by Dowex 1 column chromatography from plasma and urine contained GAG giving similar or identical bands on electrophoretograms on Separax (cellulose acetate membrane), and these GAG were susceptible to chondroitinases.The present study indicates that GAG in plasma and urine are very heterogeneous.Moreover, it is suggested that two types of GAG are present in plasma, one is macrcmolecular GAG similar to tissue GAG, and another is partially depolymerized and desulfated GAG similar to or identical with urinary GAG. The latter may be excreted directly into urine.glycosaminoglycans; plasma glycosaminoglycans; urinary glycosaminoglycans Received for publication,

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A classic case of progeria (Hutchinson-Gilford syndrome) in a 9-year-old Japanese boy is presented. The characteristic clinical features in this patient were similar to those reported in the literature. The total amount of acid glycosaminoglycans excreted in the urine was within the normal range, but there was an increase in hyaluronic acid excretion. The hyaluronuria was a novel finding in progeria, providing a common linkage with the hyaluronuria found in Werner's syndrome.

Crude glycosaminoglycan (GAG) fraction was directly precipitated with cetylpyridinium chloride without prior dialysis of urine of orthopedic patients. The crude GAG fraction was then fractionated with trichloroacetic acid (TCA). The TCA-insoluble peptide-bound GAG fraction thus obtained was treated with alkali to eliminate the peptide moiety for enzymatic analysis. The GAG compositions of this fraction and the TCA-soluble fraction were determined by digestion with mucopolysaccharidases (chondroitinase AC, chondroitinase B, chondroitinase C, heparitinase and Streptomyces hyaluronidase). When the amount of the crude GAG fraction was small, no significant amount of the TCA-insoluble peptide-bound GAG fraction was obtained. The GAG composition of this case was also determined by the same procedures after direct alkali-treatment of the crude GAG fraction. The data indicated that the proportion of the TCA-insoluble peptide-bound GAG fraction was very small. The alkali-treated TCA-insoluble peptide-bound GAG fraction contained a larger proportion of heparan sulfate than the TCA-soluble GAG fraction. It was clearly demonstrated that the patients with Werner's syndrome and mucopolysaccharidosis I-S (Scheie) excreted large amounts of hyaluronic acid and dermatan sulfate respectively, into urines. It was indicated in most cases that major urinary GAG were chondroitin 4-sulfate, chondroitin 6-sulfate plus chondroitin and heparan sulfate, while minor ones were dermatan sulfate and hyaluronic acid. In addition, the data suggested a wide range of the degree of desulfation of urinary GAG, and the presence of significant amounts of keratan sulfate plus acidic glycopeptides in the urinary GAG fractions. The present data provided more precise information on urinary GAG from orthopedic patients than those reported previously. glycosaminoglycan; urinary glycosaminoglycans; mucopolysaccharidases; orthopedic patientSince the presence of mucosubstances (glycosaminoglycans and glycoproteins) in human urine was first recognized by Morner (1895), numerous investigations have