Compounds as modulators of a mutant cftr protein and their use for treating diseases associated with cftr protein malfunction

摘要

Disclosed herein is the use of 9H-fluorene compounds of formula III where the substituents are as defined herein in the manufacture of medicaments for the treatment of cystic fibrosis. Disclosed is the use of compounds of formula III, where the substituents are as defined herein, in the manufacture of a medicament for treating cystic fibrosis. Also disclosed are compositions, pharmaceutical preparations and methods of correcting the cellular alteration of a mutant CFTR protein wherein the CFTR mutation is a mutation AF508-CFTR, or another mutation of class II.