Cystic fibrosis: Demonstration of an abnormality in mucopolysaccharides in cultured lymphoid lines

Abnormal levels of 3':5'-cyclic AMP in isoproterenol-stimulated fibroblasts from patients with cystic fibrosis.

“…Several laboratories (3)(4)(5)(6) have shown that the spent medium from such cultures contains a factor, the ciliary dyskinesia factor, which disrupts normal ciliary motion in several tissues (7,8). Also, there have been reports that CF fibroblast strains and lymphoid cell lines may differ from normal ones with respect to the activity of lysosomal enzymes (9,10), glycogen content (11), mucopolysaccharide metabolism (12), and some ultrastructural features (13). However, these results have not provided a clear-cut indication of the biochemical basis of the defect in CF.…”

The search for relevant cell culture research in cystic fibrosis: one researcher's opinion.

“…The search for relevant research would be enhanced by identification of a culture model which demonstrates these cellular abnormalities so well documented in vivo. The long-term lymphoid lines established from peripheral blood from homozygotes and heterozygotes for cystic fibrosis may be just such a cell model (Danes et al, 1974). Cystic fibrosis lymphoid lines can be distinguished from normal lymphoid lines by a relative increase in dermatan sulphate with a normal total mucopolysaccharide content.…”