ABSTRACT - 592693 Disclosed herein is a compound of formula (II) and a pharmaceutically acceptable salt thereof, wherein the variables are defined in the specification. These compounds are modulators of ATP-Binding Cassette ("ABC") transporters or fragments thereof, including Cystic Fibrosis Transmembrane Conductance Regulator ("CFTR"), and are useful for treating ABC transporter mediated condition, disease, or disorder selected from cystic fibrosis, hereditary emphysema, hereditary hemochromatosis, coagulation-fibrinolysis deficiency selected from protein C deficiency and Type 1 hereditary angioedema, lipid processing deficiencies (selected from familial hypercholesterolemia, Type 1 chylomicronemia and abetalipoproteinemia), lysosomal storage diseases, neurodegenerative diseases, polyglutamine neurological disorders, spongiform encephalopathies, COPD, dry-eye disease, and Sjögren's disease.
