Enhanced UDP-galactose:glycoprotein galactosyl transferase activity in cultivated skin fibroblasts from patients with cystic fibrosis and its possible relationship to the pathogenesis of the disease.

Homogenates of cultivated skin fibroblasts derived from patients with cystic fibrosis had 
a higher level of UDP-galactose:ovalbumin galactosyl transferase activity compared to fibroblasts 
derived from control subjects. The activity in control subjects was 1.82 
+/- 0.43 nmol galactose transferred/hr/mg protein, whereas the activity in fibroblasts 
of patients was 2.95 +/- 0.77. The difference was significant at 
P less than 0.01. Activity in the fibroblasts of obligate heterozygotes 
was 2.15 +/- 0.60. The difference between the activities in fibroblasts 
of heterozygotes and patients was significant at P less than 0.05. 
The activity in control fibroblasts could be enhanced by basic polypeptides 
like polylysine, polyarginine, histone, and protamine but not by neutral or 
acidic polypeptides. Fibroblasts from patients released significantly higher amounts of a 
soluble form of the enzyme activity into the culture medium than 
control fibroblasts. 