Figure 4.

Cell and gene expression changes in lung mesenchymal cells in alveolar capillary dysplasia with misalignment of pulmonary veins (ACDMPV). (A) Changes in mesenchymal cell type proportions using snRNA-seq of ACDMPV (n = 5), control (n = 3; 3 yr old), and preterm neonate (n = 3; 1–4 d of age and 29–31 wk of gestational age) lungs. P value represents the significance of the difference in cell proportions using a two-tailed Wilcoxon rank sum test. (B) Gene expression changes between ACDMPV and control lungs in pericytes in snRNA-seq. The criteria were as follows: differentially expressed in ACDMPV (P < 0.05, fold change ⩾ 1.5, and expression percentage ⩾ 20%) and selectively expressed in pericytes in either ACDMPV or control lungs (P < 0.05, fold change ⩾ 1.2, and expression percentage ⩾ 10%). A two-tailed Wilcoxon rank sum test was used. (C) Differential expression of FGF7, HGF, and T-box transcription factors in ACDMPV AF1 cells and pericytes compared with control or preterm neonate lungs. The asterisk denotes gene expression changes satisfying the following criteria: Bonferroni-adjusted P < 0.1, expression percentage ⩾ 20%, and fold change of average expression or expression percentage ⩾ 1.5. Boxplots represent 25%, 50%, and 75% quantiles. AF1 = alveolar fibroblast 1; AF2 = alveolar fibroblast 2; ASMC = airway smooth muscle cell; DEG = differentially expressed gene; FGF7 = fibroblast growth factor 7; HGF = hepatocyte growth factor; LAMC3 = laminin subunit gamma 3; MyoFB = myofibroblast; NR3C1 = nuclear receptor subfamily 3 group C member 1; snRNA-seq = single-nucleus RNA sequencing; TBX = T-box transcription factor; TJP1 = tight junction protein ZO-1; VSMC = vascular smooth muscle cell.