KM 11060

KM 11060 is a small molecule that corrects the processing of cystic fibrosis transmembrane conductance regulator (CFTR) proteins bearing the F508 deletion (F508del) mutation carried by 90% of cystic fibrosis patients. It increases cell surface expression and restores chloride transport function of F508del-CFTRs in BHK cells at a concentration of 10 µM. KM 11060 restores F508del-CFTR function, as measured by cAMP-stimulated iodide efflux, in CFBE41o- human airway epithelial cells at a concentration of 10 nM. It also restores the secretory response to approximately 51% of wild-type levels ex vivo in ileum isolated from F508del-CFTR mice at a concentration of 20 µM.Formal Name: 7-chloro-4-[4-[(4-chlorophenyl)sulfonyl]-1-piperazinyl]-quinoline. CAS Number: 774549-97-2. Molecular Formula: C19H17Cl2N3O2S. Formula Weight: 422.3. Purity: >98%. Formulation: (Request formulation change), A crystalline solid. Solubility: DMF: 5 mg/ml, DMSO: 2 mg/ml, Ethanol: 0.5 mg/ml. lambdamax: 201, 227, 321 nm. SMILES: ClC1=CC=C(S(N2CCN(C3=CC=NC4=C3C=CC(Cl)=C4)CC2)(=O)=O)C=C1. InChi Code: InChI=1S/C19H17Cl2N3O2S/c20-14-1-4-16(5-2-14)27(25,26)24-11-9-23(10-12-24)19-7-8-22-18-13-15(21)3-6-17(18)19/h1-8,13H,9-12H2. InChi Key: GIEHIZKCIZLXLF-UHFFFAOYSA-N.