Lung function in pallid mice after intrahepatic electroporation-mediated hAAT gene transfer. Respiratory parameters were performed on a flexiVent system (SCIREQ): static compliance, total lung capacity, and hysteresis were obtained in tracheotomize mice. B6, C57BL/6 mice; Pa, pallid mice; EV, pallid mice treated with an empty vector; AAT, pallid mice treated with AAT plasmid (n = 5 per group). Lung function measurements: (a) static compliance, (b) total lung capacity, and (c) hysteresis (area under the (pressure volume) PV loop). Static compliance was calculated from the deflating part of the PV loop between 3–7 cm H2O, total lung capacity was measured using a deep inflation manoeuvre, and hysteresis represents the area between the inflating and deflating parts of the PV loop. Each symbol represents the average of three correct manoeuvres per mouse. Values are shown as mean ± SD; * p < 0.05, *** p < 0.001 compared to untreated control group. (d) Pressure-volume (PV) loops, (e) analysis of the PV-loops, and (f) parameter K representing the curvature of the PV loops curves: Two-way ANOVA applying Bonferroni’s multiple comparisons test compared the mean inspiratory volume of four groups (used at each pressure step up to 30 cm H2O). Statistical significance * p < 0.05. t-tests were used in B and C. Values are expressed as mean and ± SD * p < 0.05, *** p < 0.001 compared to the untreated control group.