Heparan sulfate and dermatan sulfate from the liver of a patient with Hurler syndrome: high performance liquid chromatography of their degradation products after incubation with alpha-L-iduronidase-deficient fibroblasts.
Using a high performance liquid chromatography method, degradation products of heparan sulfate (HS) and dermatan sulfate (DS) were investigated after incubation of control and alpha-L-iduronidase-deficient fibroblasts with HS or DS. Characteristic elution profiles of the degradation products were obtained from the respective alpha-L-iduronidase-deficient fibroblasts. Moreover, alpha-L-iduronidase in control fibroblasts was resolved into two distinct components, forms A and B, on DEAE-cellulose column chromatography. Form A alpha-L-iduronidase could degrade HS, but not DS. Conversely, form B alpha-L-iduronidase could not degrade HS, but could degrade DS.
D002807:Chondroitin / Q000031:analogs & derivatives*; D002851:Chromatography, High Pressure Liquid; D003871:Dermatan Sulfate / Q000378:metabolism*; D005347:Fibroblasts / Q000201:enzymology*; D006025:Glycosaminoglycans / Q000378:metabolism*; D006026:Glycoside Hydrolases / Q000172:deficiency*; D006497:Heparan Sulfate / Q000378:metabolism*; D006801:Humans; D007068:Iduronidase / Q000172:deficiency*; D008099:Liver / Q000378:metabolism*; D009083:Mucopolysaccharidoses / Q000378:metabolism*; D012867:Skin / Q000201:enzymology
D002807:Chondroitin; D003871:Dermatan Sulfate; D006025:Glycosaminoglycans; D006026:Glycoside Hydrolases; D006497:Heparan Sulfate; D007068:Iduronidase