Ivacaftor-Lumacaftor Combination for CBAVD-Associated CFTR Dysfunction

Lumacaftor acts as a CFTR corrector facilitating proper protein folding and trafficking to cell surface, while ivacaftor functions as a potentiator increasing chloride channel gating probability. This dual mechanism addresses both the processing defects and reduced function of mutant CFTR proteins commonly associated with CBAVD, potentially restoring ductal fluid secretion necessary for vas deferens patency.