alpha-L-Iduronidase activity in established lymphoblastoid cells from patients with Hurler and Scheie syndromes transformed by Epstein-Barr virus.
alpha-L-Iduronidase activity was determined in established lymphoblastoid cells, which were transformed in vitro by Epstein-Barr virus, of lymphocytes-rich cell populations isolated from peripheral blood of patients with Hurler and Scheie syndromes. alpha-L-Iduronidase activities in established lymphoblastoid cells from patients were undetectable, while activities of control subjects were clearly detected. These results suggest that established lymphoblastoid cells are useful for the enzymatic study of genetic mucopolysaccharidoses.
D000293:Adolescent; D002472:Cell Transformation, Viral; D005260:Female; D006026:Glycoside Hydrolases / Q000032:analysis*; D004854:Herpesvirus 4, Human; D006801:Humans; D007068:Iduronidase / Q000032:analysis*; D007223:Infant; D008214:Lymphocytes / Q000201:enzymology*; D008297:Male; D009083:Mucopolysaccharidoses / Q000201:enzymology*; D008059:Mucopolysaccharidosis I / Q000201:enzymology*
D006026:Glycoside Hydrolases; D007068:Iduronidase