Can you explain the cell signaling pathway involved in cystic fibrosis, what goes wrong, and the right way for it to work?

CFTR: The cftr protein is on the surface of cells lining the lungs, intestine, pancreas, other organs. When activated by camp, a signaling molecule, it opens to release chloride from the inside of the cell. Water follows the chloride out of the cell. In cystic fibrosis, cftr is non-functional, so the mucus at the surface of the epithelial cell gets dehydrated and thick.