Further studies on hepatic acidic glycosaminoglycans in the Hurler syndrome.
Acid GAG were isolated from hepatic tissue from 3 patients with Hurler syndrome and 3 normal controls. Gross elevations in the uronic acid and hexosamine contents were found in Hurler livers compared with the normal ones. The total GAG concentration was significantly increased (about 25fold) in Hurler patients.
D002675:Child, Preschool; D006025:Glycosaminoglycans / Q000032:analysis / Q000378:metabolism*; D006595:Hexosamines / Q000032:analysis; D006801:Humans; D008099:Liver / Q000032:analysis / Q000378:metabolism*; D008297:Male; D008059:Mucopolysaccharidosis I / Q000378:metabolism*; D014574:Uronic Acids / Q000032:analysis
D006025:Glycosaminoglycans; D006595:Hexosamines; D014574:Uronic Acids