Fig 1: Adult Vangl2Lp/+ mice have altered lung function and disrupted elastin organization. (A) Scatter plot showing lung function measurements in 10-week-old mice. Vangl2Lp/+ mice show reduced elastance (16.66±0.856 cmH2O/ml) compared with WT littermates (19.67±0.651 cmH20/ml) (mean±s.e.m., each point shows an individual mouse with data combined from two independent experiments; Student's t-test, *P<0.05). Miller's elastin staining of (B,C) WT and (D,E) Vangl2Lp/+ 10-week-old lung sections highlights elastin (dark blue colour). Elastin deposition is prominent at the tips of alveolar crests (red arrowheads). Insets in C and E show a diagrammatic representation of the patterns of elastin deposition at alveolar crests. (F) The area of elastin at the tips of alveolar crests is increased in Vangl2Lp/+ lungs (15.89±1.30 µm2) compared with WT littermates (8.89±0.45 µm2) (WT, n=4 and Vangl2Lp/+, n=5; Student's t-test *P<0.05). (G) Western blot of total elastin (60 kDa) in 10-week-old WT and Vangl2Lp/+ littermates, and Gapdh (37 kDa) loading control. (H) Quantification revealed no significant difference in elastin protein (n=4 per genotype, with samples run on a single gel; Student's t-test, P=0.28). (I) Sircol assay for quantification of total collagen levels, in lungs of Vangl2Lp/+ and WT littermates shows no significant difference (n=3 per genotype; Student's t-test, P=0.78). (J) No significant differences are observed in relative fold change of remodelling genes, Eln, Col1a1, Col3a1 and Fn1, between adult Vangl2Lp/+ and WT controls (WT, n=8 and Vangl2Lp/+, n=6; Student's t-test, P>0.05). Scale bars: 12.5 µm (B,D), 5 µm (C,E).