G551D Mutation Impairs Protein Kinase A (PKA)-dependent Activation of CFTR Channel that can be Restored by Novel Gain-Of-Function (GOF) Mutations.

G551D is a major disease-associated gating mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) protein, an ATP- and phosphorylation-dependent chloride channel. G551D causes s...