Effect of normal and cystic fibrotic serum on ion transport and mucus glycoprotein secretion from the isolated rabbit trachea.

It has been argued that individuals with cystic fibrosis (CF) might be expected to lead reasonably normal lives if the secondary changes associated with the disease could be eliminated or overcome. The purpose of this study was to characterize the electrical and transport properties of the isolated rabbit trachea in the presence and absence of control or CF serum. In addition, we sought to determine whether the presence of CF serum resulted in an alteration in either the amount or type of mucus glycoprotein secreted by the trachea. It was found that both control and CF sera inhibited the short-circuit current of the rabbit trachea due primarily to an inhibition of active sodium transport. The effect was irreversible and could be abolished by heating the serum prior to the experiment and was only observed when the serum was on the luminal surface. There was no differential effect between control or CF serum. Investigation also indicated that exposure of the isolated trachea to CF sera had no effect on the amount of mucus glycoproteins as indicated by the degree of sulfation in secretion samples eluted from DEAE cellulose following exposure to CF serum. It is possible that alterations in the type of mucus secreted subsequent to exposure to CF serum might relate to the turbidity of the mucus seen in such patients.