Fig. 7. AAV6.2FF human proSFTPB cDNA (AAV-hSPB) prevents lung damage.

a Schematic of the human proSFTPB cDNA transgene in the rAAV2 vector genome. b Study design to determine whether 5 × 10¹⁰ vg per mouse of AAV-hSPB improves lung structure and function in SP-B deficient mice. c Percentage change in body weight following AAV injection while still on dox (duration of 28 days). (n = 11 biologically independent animals per group). d Percentage change in body weight after dox removal (duration of 4 days). All body weight measurements are presented as the mean with SD (n = 11, except 1 × PBS plus BLES post-dox where n = 4; *P = 0.0221). e Representative gross lung images at harvest 4 days following dox removal. Yellow arrows indicate regions of lung injury. The lung image from the mouse found dead was taken within 24 h of death. f Representative H&E and WGJ staining of paraffin embedded whole right and left lungs 4 days after dox removal (H&E scale bar, 200 μm; WGJ scale bar, 100 μm). g Representative epifluorescence images of SP-B (red) and DAPI (blue) of frozen lung sections from 1× PBS plus BLES treated mice off dox (n = 2), and 5 × 10¹⁰ vg AAV-hSPB plus BLES treated mice off dox (n = 2) for 4 days (Scale bar, 50 μm). h Pressure-volume curve 4 days following dox removal corrected for body weight (in mL per g) for mice On Dox (black circles), mice treated with 1× PBS plus BLES off dox (blue squares), and mice treated with AAV-hSPB plus BLES off dox (pink triangles). The source data for h has been provided as a Source Data file. i The %V₁₀ corrected for body weight. All lung function data are presented as the mean with SD (For h and in = 11 except 1× PBS n = 4; *P = 0.0169, **P = 0.0021). (All P values in Fig. 7 = 2-tailed ordinary one-way ANOVA with Tukey’s multiple comparisons post hoc test, ns = not significant) (BLES = Bovine Lipid Extract Surfactant).