Abnormal distribution of α- l-fucosidase in cystic fibrosis: Decreased activity in serum

Abstract

The activity of α- 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 α- l-fucosidase in cystic fibrosis.