CBIQ

An activator of the cystic fibrosis transmembrane conductance regulator (CFTR) and the intermediate-conductance calcium-sensitive K⁺ channel (KCNN4). May act as a novel tool for a new ion channel. First activator of F508 CFTR, the mutant form of CFTR chloride channel present in 75% of cystic fibrosis patients. Other benzoquinolines act at the normal CFTR but not at F508 CFTR. CBIQ also activates KCNN4, which hyperpolarizes airway epithelial cells to promote anion flux, a further benefit in cystic fibrosis.