Cystic fibrosis: demonstration of an abnormality in mucopolysaccharides in cultured lymphoid lines.

Cultured lymphoid cells of both homozygotes and heterozygotes for cystic fibrosis could 
be distinguished from those of normals by (1) growth pattern, gross 
clumping, and (2) a relative increase in dermatan sulfate, with a 
normal total mucopolysaccharide content. Lines derived from the genetic mucopolysaccharidoses also 
had these characteristics, but their total mucopolysaccharide content was markedly increased. 
These observations support the hypothesis that the cellular disturbance in cystic 
fibrosis resides in those cellular regions whose functions would be altered 
by mucopolysaccharide composition. 