Dinucleotides useful for the treatment of cystic fibrosis and for hydrating mucus secretions

摘要

A compound of Formula (I), or a pharmaceutically acceptable salt thereof, is disclosed: (I) wherein: n is from 1 to 6; X is -OH or -SH; A and B are each independently selected from the group consisting of: wherein R is H or Br. The compounds are useful in the treatment of airway diseases such as cystic fibrosis. Pharmaceutical formulations comprising a compound of Formula (I), and methods of hydrating mucous secretions in the lungs of a subject in need of such treatment, comprising administering to the lungs of the subject a compound of Formula I as given above, are also disclosed.