Studies on the basic defect in cystic fibrosis: the kinin hypothesis.

It is our hypothesis that in cystic fibrosis there is a genetically 
determined specific deficiency in the activity of either the glandular kallikrein 
or in its activating enzyme, and that this defect is the 
primary defect in the disease. The immediate consequence of either of 
these defects is the absence of kallidin and the resulting failure 
of reabsorption. The fact that there may be mutations affecting distinct 
enzymes could account for the genetic heterogeneity of cystic fibrosis. A 
consequence of a defective kallikrein system would be accumulation of a 
precursor related to the low molecular weight kininogen and we propose 
that this compound is the ciliary-dyskinesia factor. 