The Hurler syndrome: a study of cultured lymphoid cell lines.
Lymphoid suspension lines have been established from three patients with the Hurler syndrome and four normals. The Hurler lines can be distinguished from normals by (a) staining characteristics, (b) increase in total cellular mucopolysaccharide content, and (c) increase in dermatan sulfate. Hyaluronic acid is absent in cultured lymphoid cells from normal persons and patients with the Hurler syndrome. The availability of biochemically marked suspension cultures should prove useful for enzymatic studies as well as for further elucidation of this clinical syndrome.
D002239:Carbohydrate Metabolism, Inborn Errors; D002460:Cell Line / Q000378:metabolism*; D002478:Cells, Cultured; D011232:Chemical Precipitation; D002807:Chondroitin / Q000032:analysis; D004586:Electrophoresis; D005347:Fibroblasts / Q000378:metabolism*; D006025:Glycosaminoglycans / Q000378:metabolism*; D006651:Histocytochemistry; D006801:Humans; D006820:Hyaluronic Acid / Q000032:analysis; D066298:In Vitro Techniques; D008221:Lymphoid Tissue / Q000378:metabolism*; D008059:Mucopolysaccharidosis I / Q000235:genetics / Q000378:metabolism*; D011726:Pyridinium Compounds; D012867:Skin / Q000166:cytology; D013194:Staining and Labeling; D014574:Uronic Acids / Q000032:analysis
D006025:Glycosaminoglycans; D011726:Pyridinium Compounds; D014574:Uronic Acids; D006820:Hyaluronic Acid; D002807:Chondroitin