Abnormal distribution of alpha-L-fucosidase in cystic fibrosis: decreased activity in serum.

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. 