Primary amniotic fluid cell, skin fibroblast and liver alpha-L-fucosidase and its relation to cystic fibrosis.

Cultured skin fibroblast and primary amniotic fluid cell alpha-L- fucosidase had a 
double optimum of pH 5.0 and 6.0. Alpha-L-fucosidase was largely bound 
as a single peak to DEAE-cellulose at pH 6.6. Sucrose density 
isoelectric focusing revealed up to seven components with pI values of 
4.9, 5.2, 5.4, 5.8, 6.1, 6.5 and 7.1 with their apparent 
KM values (77--500 mumol/l) being higher than that (57 mumol/l) of 
the unfocused enzyme. Liver, skin fibroblast and amniotic fluid cell alpha-L-fucosidase 
was separated into two peaks by gel filtration. Peak one was 
more active and stable at low pH and more thermostable at 
50 degrees C than peak two, while both peaks had an 
apparent KM of 52 mumol/l. Apart from the different proportions of 
the peaks separated by gel filtration, the results for the three 
tissues were similar. The properties of alpha-L-fucosidase studied were similar for 
control and cystic fibrosis liver or skin fibroblasts. 