Safety and Pharmacokinetic Study of N6022 in Subjects With Cystic Fibrosis Homozygous for the F508del-CFTR Mutation

Inclusion Criteria: * Homozygous for F508del-CFTR gene * Sweat chloride ≥ 60 mEq/L by quantitative pilocarpine iontophoresis * Body weight ≥ 40 kg * FEV1 ≥ 40% predicted * Oxygen saturation ≥ 90% breathing ambient air * Hematology and clinical chemistry of blood and urine results with no clinically significant abnormalities that would interfere with the study assessments * Negative pregnancy test for women of child bearing potential * Sexually active subjects of child bearing potential willing to follow contraception requirements Exclusion Criteria: * Previous enrollment in another cohort for this study. * Any acute infection, including acute upper or lower respiratory infections and pulmonary exacerbations that require treatment within 4 weeks of Study Day 1. * Any change in chronic therapies for CF lung disease within 4 weeks of Study Day 1. * Blood hemoglobin \<10 g/dL at screening. * Serum albumin \<2.5 g/dL at screening. * Abnormal liver function defined as ≥ 3 x upper limit of normal (ULN) in three or more of the following: AST, ALT, GGT, ALP, total bilirubin at screening. * History of abnormal renal function (creatinine clearance \< 50 mL/min using Cockcroft-Gault equation) within a year at screening. * History, including the screening assessment, of ventricular tachycardia or other ventricular arrhythmias. * History, including the screening assessment, of prolonged QT and/or QTcF interval (\> 450 msec). * History of solid organ or hematological transplantation. * Intranasal medication changes within 14 days prior to Study Day 1 * Required Use of continuous (24 hr/d) or nocturnal supplemental oxygen. * Concomitant use of any inhibitors or inducers of CYP3A4.