Cultured cells in cystic fibrosis research: A review.

Cystic fibrosis (CF) is a common inherited disorder which is characterized by 
the production of exocrine secretions with elevated ion content and abnormally 
viscous mucus. Over the last few years cells obtained from the 
peripheral blood or cultured from tissues of cystic fibrosis patients have 
been used increasingly in the study of the disease. Investigations of 
the following properties of cystic fibrosis cells are reviewed: morphology, ultrastructure, 
growth kinetics, cellular metachromasia, the production of ciliary inhibitors, cellular composition, 
plasma membrane composition, the transport of inorganic ions and small organic 
molecules, lysosomal enzyme content, and RNA methylation. Studies of the effects 
on cultured cells and erythrocyte membranes of factors in CF cell 
culture medium and biological fluids from CF patients are discussed. 