Urinary alpha-L-fucosidase in cystic fibrosis

The binding of the urinary lysosomal enzyme alpha-L-fucosidase to free- and Sepharose 4B-bound concanavalin A has been compared in cystic fibrosis (CF) patients and normal controls. The concentration of methyl-alpha-D-mannoside necessary to prevent 50% of total alpha-L-fucosidase activity to bind to free and bound concanavalin A (Ki, 50%) was similar for CF (0.68 +/- 0.20 and 1.3 +/- 0.3 mmol/l, respectively) and normal controls (0.53 +/- 0.18 and 1.9 +/- 0.5 mmol/l, respectively). The CF and normal urinary alpha-L-fucosidase also showed similar pH optima (4.8), Km, app (0.071 and 0.074 mmol/l, respectively) and thermodenaturation curves at 44 degrees C (t1/2 = 108 min). We report that the kinetic and the concanavalin A-binding affinity of alpha-L-fucosidase are similar from urine of cystic fibrosis patients and controls.