Pullamsetti, Soni SavaiSoni SavaiPullamsettiSitapara, RavikumarRavikumarSitaparaOsterhout, RobinRobinOsterhoutWeiss, AstridAstridWeissCarter, Laura L.Laura L.CarterZisman, Lawrence S.Lawrence S.ZismanSchermuly, Ralph TheoRalph TheoSchermuly2023-09-212023-09-212023https://jlupub.ub.uni-giessen.de/handle/jlupub/18487http://dx.doi.org/10.22029/jlupub-17851Pulmonary arterial hypertension (PAH) is a complex disorder characterized by vascular remodeling and a consequent increase in pulmonary vascular resistance. The histologic hallmarks of PAH include plexiform and neointimal lesions of the pulmonary arterioles, which are composed of dysregulated, apoptosis-resistant endothelial cells and myofibroblasts. Platelet-derived growth factor receptors (PDGFR) α and β, colony stimulating factor 1 receptor (CSF1R), and mast/stem cell growth factor receptor kit (c-KIT) are closely related kinases that have been implicated in PAH progression. In addition, emerging data indicate significant crosstalk between PDGF signaling and the bone morphogenetic protein receptor type 2 (BMPR2)/transforming growth factor β (TGFβ) receptor axis. This review will discuss the importance of the PDGFR-CSF1R-c-KIT signaling network in PAH pathogenesis, present evidence that the inhibition of all three nodes in this kinase network is a potential therapeutic approach for PAH, and highlight the therapeutic potential of seralutinib, currently in development for PAH, which targets these pathways.enNamensnennung 4.0 InternationalPDGFRc-KITCSF1RABLimatinibdasatinibinhalationddc:610