HUNTER-HURLER SYNDROME - GEL FILTRATION AND DIALYSIS OF URINARY ACID MUCOPOLYSACCHARIDES

PATIENTS with Hunter-Hurler syndrome, which is an inborn error of metabolism, have excessive deposition of acid mucopolysaccharides (AMPS) in various organs1,2, and large amounts of chondroitin sulphate B and/or heparitin sulphate are produced in the urine3. In contrast, normal people excrete a small amount of AMPS, chiefly in the form of chondroitin sulphate C and chondroitin sulphate A. The determination of urinary AMPS is being used extensively in the study and in the diagnosis of this disease and of its several genetic variants. © 1968 Nature Publishing Group.