Biochemistry of cystic fibrosis: an overview. pp. 157-64.

Cystic fibrosis has been a difficult problem to solve. One amino acid 
is not found in elevated concentrations in the urine, one abnormal 
sugar or polysaccharide is not found in secretions of the bronchial 
tree which causes pulmonary damage, and a unique material which inhibits 
sodium transport has not yet been identified in sweat. Instead of 
viewing the current state of CF research as chaotic, perhaps we 
should say in a positive way that the diversity of ideas 
stimulates thinking, and in the process of sorting out the primary 
defect, a wide spectrum of pathophysiologic changes will be recognized. Recently 
a positive-charged polypeptide associated with immunoglobulin G has been identified which 
may be responsible for the ciliary dyskinesis effect of CF serum. 
This material should demonstrate the same properties when bound to immunoglobulin 
G from control sera. It would be interesting if it were 
identical with the basic protein recently found only in the serum 
and IgG fraction of CF patients and carriers. Further work may 
identify how the polypeptide affects cell membrane surfaces, and whether a 
defective peptidase, perhaps an arginine esterase, fails to break down the 
polypeptide. A unifying hypothesis could then emerge and tie biochemical and 
physiological findings together. 