title: The end organ defect in cystic fibrosis; a hypothesis: disinhibited inositol cycle activation?
abstract: 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.
mesh_terms: D001798:Blood Proteins / Q000378:metabolism; D002118:Calcium / Q000378:metabolism; D002463:Cell Membrane Permeability; D002712:Chlorides / Q000378:metabolism; D002925:Ciliary Motility Disorders / Q000378:metabolism; D003550:Cystic Fibrosis / Q000188:drug therapy / Q000378:metabolism*; D006801:Humans; D007294:Inositol / Q000378:metabolism*; D007473:Ion Channels / Q000378:metabolism; D008094:Lithium / Q000627:therapeutic use; D008954:Models, Biological; D010716:Phosphatidylinositols / Q000378:metabolism
chemical_list: D001798:Blood Proteins; D002712:Chlorides; D007473:Ion Channels; D010716:Phosphatidylinositols; C007065:ciliary dyskinesia factor; D007294:Inositol; D008094:Lithium; D002118:Calcium