title: Abnormal distribution of alpha-L-fucosidase in cystic fibrosis: decreased activity in serum.
abstract: The activity of alpha-L-fucosidase is decreased in the serum of cystic fibrosis patients when compared to age-matched controls. This result, combined with the elevated activity in skin fibroblasts, supports the concept of an abnormal intracellular and extracellular distribution of alpha-L-fucosidase in cystic fibrosis.
mesh_terms: D000118:Acetylglucosaminidase / Q000097:blood; D000135:Acid Phosphatase / Q000097:blood; D000328:Adult; D002648:Child; D003550:Cystic Fibrosis / Q000097:blood* / Q000201:enzymology; D005347:Fibroblasts / Q000201:enzymology; D006801:Humans; D007231:Infant, Newborn; D012867:Skin / Q000201:enzymology; D005644:alpha-L-Fucosidase / Q000032:analysis / Q000097:blood*
chemical_list: D000135:Acid Phosphatase; D005644:alpha-L-Fucosidase; D000118:Acetylglucosaminidase