levels of wild type cftr analysis of band c of u03b4f508 cftr cho cells showed that very little band c was present in u03b4f508 cells compared to wild type cells exposure of these cells to test compounds at 100 u03bcm for 24 hours at 37 u00b0 c did not affect the level of band c cftr in either wild type or u03b4f508 cftr expressing cells in contrast analysis of band b cftr in u03b4f508 cells showed that test compounds at 100 u03bcm resulted in a significant increase about 6 7 fold in the level of band b compared to u03b4f508 cells not exposed to the test compounds test compounds of the invention were administered to three groups of 10 mice at 100 300 and 1 000 mg kg an additional group received vehicle 20 hydroxypropyl u03b2 cyclodextrin aqueous solution at 10 ml kg mortality morbidity checks were made twice daily clinical observations were recorded predose and or postdose on day 1 and daily thereafter through day 8 body weights were recorded on the day of dosing day 1 and on day 8 mice were euthanized by co 2 asphyxiation and necropsied on day 8 or upon death one test compound was tested so far.