title: alpha-L-Fucosidase in cystic fibrosis: analysis of skin fibroblasts and liver.
abstract: The lysosomal enzyme alpha-L-fucosidase has been examined by thin layer gel and column isoelectric focusing in skin fibroblasts and liver from patients with cystic fibrosis and controls. All three common phenotypes of the enzyme were observed in both control and CF fibroblasts. When individuals of the same alpha-L-fucosidase phenotype were compared, no major differences between the isozyme profiles of cystic fibrosis patients and controls were detected in either fibroblasts or liver tissue.
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000201:enzymology*; D005347:Fibroblasts / Q000201:enzymology; D006801:Humans; D007525:Isoelectric Focusing; D007527:Isoenzymes / Q000235:genetics; D008099:Liver / Q000201:enzymology*; D008247:Lysosomes / Q000201:enzymology; D010641:Phenotype; D012867:Skin / Q000201:enzymology*; D005644:alpha-L-Fucosidase / Q000235:genetics*
chemical_list: D007527:Isoenzymes; D005644:alpha-L-Fucosidase