This is the extension of the FFC#11/2019 pilot project; it aims at discovering what are the modifications of the CFTR protein during the maturation process which takes place after its synthesis is carried out. In fact, the F508del mutant protein’s functional recovery induced by correctors is associated with a number of modifications, called “post-translational” modifications, which are a characteristic of the normal protein. The main objective is to clarify the real contribution of the post-translational modifications in the functional recovery of F508del. For this purpose, 5 enzymes, called “demethylases”, have been identified, which are involved in the F508del’s maturation induced by correctors by acting on protein’s methylation (addition or subtraction of the methyl group CH3). The study intends to understand whether it is possible to promote the recovery of the mutated channel by controlling those changes, with particular reference to protein methylation.
WHO ADOPTED THE PROJECT
€ 40.000
€ 21.500
Delegazione FFC di Vigevano
€ 47.000