Loss of family with sequence similarity 13, member A exacerbates pulmonary hypertension through accelerating endothelial-to-mesenchymal transition
Fig 3
Genetic loss of FAM13A exacerbates pulmonary hypertension.
(A) Pulse pressure diagram for right ventricles in WT and Fam13a-/- mice exposed to chronic hypoxia (10% O2 for 3 weeks). (B) Right ventricular systolic pressure (RVSP) was measured in WT and Fam13a-/- mice exposed to either normoxic or hypoxic conditions (n = 5 each for normoxia group, n = 10 each for hypoxia group). (C) Ratio of right ventricle compared to left ventricle + septum (Fulton’s Index) was calculated (n = 5 each for normoxia group, n = 10 each for hypoxia group). (D) HE staining of the lung sections in WT and Fam13a-/- mice exposed to chronic hypoxia. Prominent muscularization in small pulmonary artery was detected in the lungs of Fam13a-/- mice. Bars: 50 μm. Wall thickness was quantitatively analyzed (n = 8–9 each). (E) Immunohistochemistry for α-smooth muscle actin (α-SMA) and vWF in the lung sections of WT and Fam13a-/- mice exposed to chronic hypoxia. Muscularization of small pulmonary artery was deteriorated in the lungs of Fam13a-/- mice. Fully muscularized small pulmonary artery was quantified (n = 8–9 each). (F) Elastica van Gieson staining of the lung sections in WT and Fam13a-/- mice exposed to chronic hypoxia. Significant reduction in the number of small pulmonary artery (<50μm diameter) was observed in the lungs of Fam13a-/- mice. Average ratio of small pulmonary artery per 100 alveoli was calculated (n = 8–9 each). Data are presented as the mean ± SEM. *P < 0.05 and **P < 0.01.