The end organ defect in cystic fibrosis; a hypothesis: disinhibited inositol cycle activation?

Activation of the inositol cycle by a factor capable of by-passing the normal controls on exocrine secretion by an interaction with a coupling protein could produce effects similar to a calcium ionophore or the ciliary dyskinesia factor. The chloride permeability defect may represent a secondary adaptive change, able to limit the consequences of this via an acid shift in intracellular pH. The model predicts that lithium treatment would limit the effects of the disease.