Figure 3

Effect of CFTR mutation heterozygosity on smoke-induced CFTR dysfunction in murine trachea. C57BL/6 J mice expressing wild type CFTR (CFTR +/+) or heterozygous CFTR knockout (CFTR +/-) were exposed to whole cigarette smoke (4 cigarettes, twice daily) or room air control in whole body chambers for 2 weeks prior to CFTR functional estimation in trachea ex-vivo by Ussing chamber electrophysiology. CFTR activity was estimated as cAMP-dependent change in tracheal short circuit current (Isc) by stimulating with forskolin (20 μM) plus IBMX (100 μM). N = 8/condition, ϕ = 0.25 and, γ = 0.08.