We found that, together with other steps of OXPHOS, ANT-dependent ADP/ATP exchange was impaired in CF cells and, importantly, treatment of CF cells with the small molecules VX-809 and 4,6,4′-trimethylangelicin, which act as “correctors” for F508del CFTR by rescuing the F508del CFTR-dependent chloride secretion, significantly improved the mitochondrial parameters towards values found in the airway cells expressing wt-CFTR. The gene discussed is CFTR; the disease is cystic fibrosis.