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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.


Journal article


Clin Chim Acta

Publication Date





171 - 178


Adolescent, Adult, Child, Child, Preschool, Cystic Fibrosis, Fibroblasts, Humans, Isoelectric Focusing, Isoenzymes, Liver, Lysosomes, Phenotype, Skin, alpha-L-Fucosidase