CELL SIGNALLING FOR CYSTIC FIBROSIS: MOLECULAR MECHANISM AND THERAPEUTIC INTERVENTIONS
Transport proteins located in the plasma membrane play critical roles in speeding up and controlling the movements of molecules and ions into and out of the cells. If any of these proteins is defective, the transport of a particular ion or molecule is likely to be impaired and disease may result. Cystic fibrosis (CF), a clinically heterogeneous disease, is the first genetic disease for which adult population screening has been initiated in the United States. Since the discovery of the Cystic fibrosis transmembrane regulator (CFTR) gene in 1989, much has been learned about the pathophysiology and molecular genetics of this disorder. This review includes an overview of the genetics of CF, throws light on defective cellular signalling pathway, discusses pathophysiology, and clinical and anatomic pathology, molecular diagnostics and concludes with a review of therapeutic possibilities for this dreadful disease. Armed with information about the gene and the protein associated with CF, researchers are now trying to develop new promising treatments or perhaps even a cure for the disease