Cystic fibrosis serum alpha-l-fucosidase: confirmation of normal activity levels and normal kinetic and isoelectric focusing properties.
Comparable alpha-L-fucosidase activity was found for normal and cystic fibrosis sera, 7.8 +/- 3.3 and 9.8 +/- 4.7 nmol/min/ml, respectively. Isoelectric focusing of normal and cystic fibrosis sera alpha-L-fucosidase revealed similar isoelectric profiles with most activity between isoelectric points of 4.7--5.4. Kinetic analysis of normal and cystic fibrosis sera alpha-L-fucosidase revealed identical apparent Michaelis constants for the 4-methylumbelliferyl substrate (51 +/- 6 mumol/l and 50 +/- 8 mumol/l, respectively), similar pH optima curves (both have broad optima between pH 4.8 and 6.5) and identical thermostability curves at three different preincubation temperatures (37 degrees, 45 degrees and 55 degrees C).
D003550:Cystic Fibrosis / Q000097:blood*; D006801:Humans; D007525:Isoelectric Focusing; D007700:Kinetics; D005644:alpha-L-Fucosidase / Q000097:blood* / Q000378:metabolism
D005644:alpha-L-Fucosidase