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.