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

摘要

Summary: 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 P 2-macroglobulin-protease complexes, the resulting deficient proteolytic activity could generate the “factors” previously identified in the disease. These “factors” may interact with UDP-galactose:glycoprotein galactosyl transferase in a manner similar to that observed with the model polypeptides employed in this study, enhancing its activity.