Scientists have shed new light on protein interactions in cystic fibrosis, which could have possible implications for future drugs to treat the disease.
Published in PNAS, the study analysed interactions of the cystic fibrosis transmembrane conductance regulator (CFTR) protein. In healthy individuals, the CFTR protein and the naturally occurring 14-3-3, interact to ensure the trafficking of the CFTR protein to the cell wall is successful. In around 70% of Caucasians diagnosed with cystic fibrosis, the cause is known to be anomalies in the CFTR protein and a failure of the protein reaching the cell wall.
By isolating this protein-protein interaction in the laboratory, scientists at Eindhoven University of Technology and McGill University discovered that the naturally occurring fusicoccin-A can stabilise the interaction between 14-3-3 and CFTR, by selectively interacting with the mutant CFTR, which in turn stimulates the trafficking of CFTR to the cell wall.
This new finding could lead to drug development in the future as the mechanism described in the paper could act as a model for the development of new trafficking corrector molecules to treat cystic fibrosis.