A comprehensive map of proteoglycan expression and deposition in the pulmonary arterial wall in health and pulmonary hypertension
Ayse Ceren Mutgan, Nemanja Radic, Francesco Valzano, Slaven Crnković, Natalia El-Merhie, Matthias Evermann, Konrad Höetzenecker, Vasile Foris, Luka Brčić, Leigh M. Marsh, Karin Tran‐Lundmark, Katharina Jandl, Grażyna Kwapiszewska
Abstract
Idiopathic pulmonary arterial hypertension (IPAH) pulmonary arteries (PAs) displayed the greatest proteoglycan (PG) changes, with PH associated with pulmonary fibrosis (PH-PF) and PH associated with chronic obstructive pulmonary disease (PH-COPD) following. Agrin, an endothelial cell-specific PG, was solely upregulated in IPAH. Among all cells, neo-intima-forming smooth muscle cells (SMCs) displayed the most significant PG increase. Increased levels of circulating decorin, lumican, and versican, mainly derived from SMCs, and adventitial fibroblasts, may serve as systemic indicators of pulmonary remodeling, reflecting perivascular fibrosis and neointima formation.