Expression of Angioproliferative Molecules in Plexiform like Lesions in Lungs of Domestic Fowl Selected for Susceptibility to Pulmonary Arterial Hypertension

Abstract

Domestic fowl (broiler chickens) bred for meat production spontaneously develop pulmonary arterial hypertension (PAH) accompanied by the formation of plexiform‐like complex vascular lesions (CVLs). CVLs formed distal to branch points in inter‐parabronchial pulmonary arterioles that typically exhibit medial hypertrophy and intimal proliferation. The CVLs in the lungs of broilers closely resemble the histology of plexiform lesions in the lungs of human PAH patients. Paraformaldehyde fixed lung tissues from 8‐ to 24‐week old broilers were stained immunohistochemically for detection of various angioproliferative molecules expressed in the CVLs. These molecules include: smooth muscle actin; von Willebrand factor; vascular endothelial growth factor (VEGF); VEGF receptor II; hypoxia inducible factor 1 alpha; survivin; collagen; fibronectin; and tenascin. The CVLs in broiler lungs exhibited positive staining for these molecules. Expression of these molecules within the CVL of PAH‐susceptible broilers combined with the close histological resemblance of CVLs to plexiform lesions of human PAH patients further supports the use of broilers from our PAH susceptible line as an excellent animal model for PAH and plexogenic arteriopathy.“Supported by NIH grant 1R15HL092517”