title: Activities of acid hydrolases in fibroblasts from normal and cystinotic children.
abstract: A study of the lysosomal hydrolases bete-galactosidase, beta-glucuronidase, N-acetyl-beta-D-glucosaminidase, and arylsulphatases A and B has been carried out on fibroblasts cultured from seven patients with cystinosis and eight control subjects. beta-Galactosidase activity was found to be consistently lower in cells derived from cystinotics, while the other enzymes studied showed no significant differences between normals and cystinotics.
mesh_terms: D001192:Arylsulfatases / Q000378:metabolism; D002478:Cells, Cultured; D002648:Child; D003553:Cystine / Q000378:metabolism; D003554:Cystinosis / Q000201:enzymology*; D005347:Fibroblasts / Q000201:enzymology; D005696:Galactosidases / Q000378:metabolism; D006026:Glycoside Hydrolases / Q000378:metabolism; D049671:History, 18th Century; D006801:Humans; D006867:Hydrolases / Q000378:metabolism*; D008247:Lysosomes / Q000201:enzymology; D008928:Mitochondria / Q000201:enzymology
chemical_list: D003553:Cystine; D006867:Hydrolases; D001192:Arylsulfatases; D005696:Galactosidases; D006026:Glycoside Hydrolases