Gas chromatography was used for the determination of monosaccharides obt,ained b)-depolymerisation of acid mucopolysaccharides (AMPS). Purified AMPS and AMPS precipitated from the urine of patients with mucopolysaccharidoses can be identified in this manner. The utility of the technique was illustrated by demonstrating the difference in the hexosehexosamine pattern obtained by depolymerization of urinary AMPS from patients with Hurler's and Sanfilippo's disease. The diagnostic value of the technique in delineating more subtle differences in relative amounts of AMPS awaits further experimentat.ion.
The approach of determining the composition of the acid mucopolysaccharides (AMPS) mixture excreted in the urine has proved valuable in the diagnosis of mucopolysaccharidoses (1). The composition of the known AMPS and the occurrence of these compounds in the urine of patients with various types of mucopolysaccharidoses is given in Table 1. The method most often employed to resolve AMPS is column chromatography using anion exchange resins such as Dowex 1 X 2 (2) or DEAE-Sephadex A-25 (3). The various AMPS are separated by elution from the column with aqueous solutions of NaCI of increasing concentrations depending on their polyanionic character. The efficiencies of various anion exchange resins used in this technique have been recently compared (4). The AMPS in the effluent can he quantitated by color reactions such as the one used for uranic acids (5).
The column chromatography technique has two major disadvantages. In the firs? place, milligram amounts of AMPS are needed for an accnrate analysis, and secondly: the time recluired for the workup of the sample 465