{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,24]],"date-time":"2026-10-24T12:09:20Z","timestamp":1792843760104,"version":"4.5.0"},"reference-count":150,"publisher":"MDPI AG","issue":"9","license":[{"start":{"date-parts":[[2025,4,30]],"date-time":"2025-04-30T00:00:00Z","timestamp":1745971200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"National Natural Science Foundation of China","award":["82460923"],"award-info":[{"award-number":["82460923"]}]},{"name":"National Natural Science Foundation of China","award":["202401AZ070001-022"],"award-info":[{"award-number":["202401AZ070001-022"]}]},{"name":"Basic Research Program of Yunnan Provincial Science and Technology Department","award":["82460923"],"award-info":[{"award-number":["82460923"]}]},{"name":"Basic Research Program of Yunnan Provincial Science and Technology Department","award":["202401AZ070001-022"],"award-info":[{"award-number":["202401AZ070001-022"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Pulmonary hypertension (PH) is a serious cardiovascular disease caused by a variety of pathogenic factors, which is characterized by increased pulmonary vascular resistance (PVR) and progressive elevation of mean pulmonary artery pressure (mPAP). This disease can lead to right ventricular hypertrophy and, in severe cases, right heart failure and even death. Vascular remodeling\u2014a pathological modification involving aberrant vasoconstriction, cell proliferation, apoptosis resistance, and inflammation in the pulmonary vascular system\u2014is a significant pathological hallmark of PH and a critical process in its progression. Recent studies have found that vascular remodeling involves the participation of a diversity of cellular pathological alterations, such as the dysfunction of pulmonary artery endothelial cells (PAECs), the proliferation and migration of pulmonary artery smooth muscle cells (PASMCs), the phenotypic differentiation of pulmonary artery fibroblasts, the inflammatory response of immune cells, and pericyte proliferation. This review focuses on the mechanisms and the intercellular crosstalk of these cells in the PH process, emphasizing recent advances in knowledge regarding cellular signaling pathways, inflammatory responses, apoptosis, and proliferation. To develop better treatments, a list of possible therapeutic approaches meant to slow down certain biological functions is provided, with the aim of providing new insights into the treatment of PH by simplifying the intricacies of these complex connections. In this review, comprehensive academic databases such as PubMed, Embase, Web of Science, and Google Scholar were systematically searched to discuss studies relevant to human and animal PH, with a focus on vascular remodeling in PH.<\/jats:p>","DOI":"10.3390\/ijms26094265","type":"journal-article","created":{"date-parts":[[2025,4,30]],"date-time":"2025-04-30T05:50:17Z","timestamp":1745992217000},"page":"4265","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":50,"title":["Vascular Remodeling: The Multicellular Mechanisms of Pulmonary Hypertension"],"prefix":"10.3390","volume":"26","author":[{"ORCID":"https:\/\/orcid.org\/0009-0000-7519-3706","authenticated-orcid":false,"given":"Jinjin","family":"Dai","sequence":"first","affiliation":[{"name":"School of Basic Medical Sciences, Yunnan University of Chinese Medicine, Kunming 650500, China"},{"name":"Yunnan Key Laboratory of Integrated Traditional Chinese and Western Medicine for Chronic Disease in Prevention and Treatment, Kunming 650500, China"},{"name":"Key Laboratory of Microcosmic Syndrome Differentiation, Education Department of Yunnan, Kunming 650500, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5452-8116","authenticated-orcid":false,"given":"Hongyang","family":"Chen","sequence":"additional","affiliation":[{"name":"School of Basic Medical Sciences, Yunnan University of Chinese Medicine, Kunming 650500, China"},{"name":"Yunnan Key Laboratory of Integrated Traditional Chinese and Western Medicine for Chronic Disease in Prevention and Treatment, Kunming 650500, China"},{"name":"Key Laboratory of Microcosmic Syndrome Differentiation, Education Department of Yunnan, Kunming 650500, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0007-7221-9887","authenticated-orcid":false,"given":"Jindong","family":"Fang","sequence":"additional","affiliation":[{"name":"School of Basic Medical Sciences, Yunnan University of Chinese Medicine, Kunming 650500, China"},{"name":"Yunnan Key Laboratory of Integrated Traditional Chinese and Western Medicine for Chronic Disease in Prevention and Treatment, Kunming 650500, China"},{"name":"Key Laboratory of Microcosmic Syndrome Differentiation, Education Department of Yunnan, Kunming 650500, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0006-1985-2010","authenticated-orcid":false,"given":"Shiguo","family":"Wu","sequence":"additional","affiliation":[{"name":"School of Basic Medical Sciences, Yunnan University of Chinese Medicine, Kunming 650500, China"},{"name":"Yunnan Key Laboratory of Integrated Traditional Chinese and Western Medicine for Chronic Disease in Prevention and Treatment, Kunming 650500, China"},{"name":"Key Laboratory of Microcosmic Syndrome Differentiation, Education Department of Yunnan, Kunming 650500, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9376-6595","authenticated-orcid":false,"given":"Zhuangzhuang","family":"Jia","sequence":"additional","affiliation":[{"name":"School of Basic Medical Sciences, Yunnan University of Chinese Medicine, Kunming 650500, China"},{"name":"Yunnan Key Laboratory of Integrated Traditional Chinese and Western Medicine for Chronic Disease in Prevention and Treatment, Kunming 650500, China"},{"name":"Key Laboratory of Microcosmic Syndrome Differentiation, Education Department of Yunnan, Kunming 650500, China"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,4,30]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"678","DOI":"10.1161\/CIRCULATIONAHA.116.022362","article-title":"Systemic Consequences of Pulmonary Hypertension and Right-Sided Heart Failure","volume":"141","author":"Rosenkranz","year":"2020","journal-title":"Circulation"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"373","DOI":"10.1186\/s12931-024-02994-w","article-title":"Survival, morbidity, and quality of life in pulmonary arterial hypertension patients: A systematic review of outcomes reported by population-based observational studies","volume":"25","author":"Reinders","year":"2024","journal-title":"Respir. Res."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"3618","DOI":"10.1093\/eurheartj\/ehac237","article-title":"2022 ESC\/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension","volume":"43","author":"Humbert","year":"2022","journal-title":"Eur. Heart J."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"1801913","DOI":"10.1183\/13993003.01913-2018","article-title":"Haemodynamic definitions and updated clinical classification of pulmonary hypertension","volume":"53","author":"Simonneau","year":"2019","journal-title":"Eur. Respir. J."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"544","DOI":"10.1016\/j.healun.2022.12.012","article-title":"Vascular pathobiology of pulmonary hypertension","volume":"42","author":"Ntokou","year":"2023","journal-title":"J. Heart Lung Transplant. Off. Publ. Int. Soc. Heart Transplant."},{"key":"ref_6","doi-asserted-by":"crossref","unstructured":"Jia, Z., Wang, S., Yan, H., Cao, Y., Zhang, X., Wang, L., Zhang, Z., Lin, S., Wang, X., and Mao, J. (2023). Pulmonary Vascular Remodeling in Pulmonary Hypertension. J. Pers. Med., 13.","DOI":"10.3390\/jpm13020366"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"2401095","DOI":"10.1183\/13993003.01095-2024","article-title":"Pathology and pathobiology of pulmonary hypertension: Current insights and future directions","volume":"64","author":"Guignabert","year":"2024","journal-title":"Eur. Respir. J."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"H702","DOI":"10.1152\/ajpheart.00021.2022","article-title":"Mechanisms of pulmonary vascular dysfunction in pulmonary hypertension and implications for novel therapies","volume":"322","author":"Christou","year":"2022","journal-title":"Am. J. Physiol. Heart Circ. Physiol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"197","DOI":"10.1038\/s41569-022-00770-1","article-title":"Vascular endothelial cell development and diversity","volume":"20","author":"Trimm","year":"2023","journal-title":"Nat. Rev. Cardiol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"4833","DOI":"10.1016\/j.cell.2024.07.012","article-title":"A systems view of the vascular endothelium in health and disease","volume":"187","author":"Augustin","year":"2024","journal-title":"Cell"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1016\/j.cophys.2018.12.003","article-title":"Revisiting the role of hypoxia-inducible factors in pulmonary hypertension","volume":"7","author":"Shimoda","year":"2019","journal-title":"Curr. Opin. Physiol."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"1533","DOI":"10.1161\/CIRCULATIONAHA.123.067005","article-title":"ErbB3 Governs Endothelial Dysfunction in Hypoxia-Induced Pulmonary Hypertension","volume":"150","author":"Bian","year":"2024","journal-title":"Circulation"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"1584","DOI":"10.1016\/j.ajpath.2020.04.003","article-title":"Understanding the Oxygen-Sensing Pathway and Its Therapeutic Implications in Diseases","volume":"190","author":"Liao","year":"2020","journal-title":"Am. J. Pathol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"230183","DOI":"10.1183\/16000617.0183-2023","article-title":"The nitric oxide-soluble guanylate cyclase-cGMP pathway in pulmonary hypertension: From PDE5 to soluble guanylate cyclase","volume":"33","author":"Benza","year":"2024","journal-title":"Eur. Respir. Rev."},{"key":"ref_15","doi-asserted-by":"crossref","unstructured":"Moccia, F., Negri, S., Shekha, M., Faris, P., and Guerra, G. (2019). Endothelial Ca2+ Signaling, Angiogenesis and Vasculogenesis: Just What It Takes to Make a Blood Vessel. Int. J. Mol. Sci., 20.","DOI":"10.3390\/ijms20163962"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"846","DOI":"10.1016\/j.freeradbiomed.2024.11.009","article-title":"Expression of soluble guanylate cyclase (sGC) and its ability to form a functional heterodimer are crucial for reviving the NO-sGC signaling in PAH","volume":"225","author":"Sumi","year":"2024","journal-title":"Free Radic. Biol. Med."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"e133","DOI":"10.1161\/CIRCRESAHA.124.324924","article-title":"Prostacyclin Synthase Deficiency Leads to Exacerbation or Occurrence of Endothelium-Dependent Contraction and Causes Cardiovascular Disorders Mainly via the Non-TxA2 Prostanoids\/TP Axis","volume":"135","author":"Ge","year":"2024","journal-title":"Circ. Res."},{"key":"ref_18","first-page":"PA5043","article-title":"Functional study of polymorphisms in the promoter region of the endothelin-1 gene in Pulmonary Arterial Hypertension","volume":"54","author":"Villar","year":"2019","journal-title":"Eur. Respir. Soc."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"384","DOI":"10.1016\/j.stem.2018.01.010","article-title":"CD157 Marks Tissue-Resident Endothelial Stem Cells with Homeostatic and Regenerative Properties","volume":"22","author":"Wakabayashi","year":"2018","journal-title":"Cell Stem Cell"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"1784","DOI":"10.1161\/ATVBAHA.123.319839","article-title":"Angiogenesis Redux: An Overall Protective Role of VEGF\/KDR Signaling in the Microvasculature in Pulmonary Arterial Hypertension","volume":"43","author":"Zhong","year":"2023","journal-title":"Arterioscler. Thromb. Vasc. Biol."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"427","DOI":"10.1096\/fj.00-0343com","article-title":"Inhibition of the VEGF receptor 2 combined with chronic hypoxia causes cell death-dependent pulmonary endothelial cell proliferation and severe pulmonary hypertension","volume":"15","author":"Kasahara","year":"2001","journal-title":"FASEB J"},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"1","DOI":"10.2174\/0118715206356712241202112641","article-title":"Advances in VEGFR Inhibitors: A Comprehensive Review of Novel Anticancer Agents","volume":"25","author":"Jha","year":"2025","journal-title":"Anticancer. Agents Med. Chem."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"L250","DOI":"10.1152\/ajplung.00105.2024","article-title":"Pneumonectomy combined with SU5416 or monocrotaline pyrrole does not cause severe pulmonary hypertension in mice","volume":"327","author":"Sun","year":"2024","journal-title":"Am. J. Physiol. Lung Cell Mol. Physiol."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"474","DOI":"10.1165\/rcmb.2014-0045TR","article-title":"The role of vascular endothelial growth factor in pulmonary arterial hypertension. The angiogenesis paradox","volume":"51","author":"Voelkel","year":"2014","journal-title":"Am. J. Respir. Cell Mol. Biol."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"eabm5755","DOI":"10.1126\/scitranslmed.abm5755","article-title":"Endothelial FoxM1 reactivates aging-impaired endothelial regeneration for vascular repair and resolution of inflammatory lung injury","volume":"15","author":"Huang","year":"2023","journal-title":"Sci. Transl. Med."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"2168","DOI":"10.1161\/ATVBAHA.119.312826","article-title":"Endothelial-to-Mesenchymal Transition in Human Adipose Tissue Vasculature Alters the Particulate Secretome and Induces Endothelial Dysfunction","volume":"39","author":"Haynes","year":"2019","journal-title":"Arterioscler. Thromb. Vasc. Biol."},{"key":"ref_27","doi-asserted-by":"crossref","unstructured":"Singh, A., Bhatt, K.S., Nguyen, H.C., Frisbee, J.C., and Singh, K.K. (2024). Endothelial-to-Mesenchymal Transition in Cardiovascular Pathophysiology. Int. J. Mol. Sci., 25.","DOI":"10.20944\/preprints202405.0888.v1"},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"245","DOI":"10.1146\/annurev-physiol-032222-080806","article-title":"Endothelial to Mesenchymal Transition in Health and Disease","volume":"85","author":"Xu","year":"2023","journal-title":"Annu. Rev. Physiol."},{"key":"ref_29","doi-asserted-by":"crossref","unstructured":"Peng, Q., Shan, D., Cui, K., Li, K., Zhu, B., Wu, H., Wang, B., Wong, S., Norton, V., and Dong, Y. (2022). The Role of Endothelial-to-Mesenchymal Transition in Cardiovascular Disease. Cells, 11.","DOI":"10.3390\/cells11111834"},{"key":"ref_30","doi-asserted-by":"crossref","unstructured":"Yun, E., Kook, Y., Yoo, K.H., Kim, K.I., Lee, M.S., Kim, J., and Lee, A. (2020). Endothelial to Mesenchymal Transition in Pulmonary Vascular Diseases. Biomedicines, 8.","DOI":"10.3390\/biomedicines8120639"},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"223","DOI":"10.1093\/cvr\/cvae021","article-title":"Endothelial to mesenchymal transition: At the axis of cardiovascular health and disease","volume":"120","author":"Hall","year":"2024","journal-title":"Cardiovasc. Res."},{"key":"ref_32","doi-asserted-by":"crossref","unstructured":"Fang, J.S., Hultgren, N.W., and Hughes, C.C.W. (2021). Regulation of Partial and Reversible Endothelial-to-Mesenchymal Transition in Angiogenesis. Front. Cell Dev. Biol., 9.","DOI":"10.3389\/fcell.2021.702021"},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"477","DOI":"10.1038\/nrm.2017.36","article-title":"Vascular heterogeneity and specialization in development and disease","volume":"18","author":"Potente","year":"2017","journal-title":"Nat. Rev. Mol. Cell Biol."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"670","DOI":"10.1161\/CIRCRESAHA.117.312310","article-title":"Clonal Expansion of Endothelial Cells Contributes to Ischemia-Induced Neovascularization","volume":"122","author":"Manavski","year":"2018","journal-title":"Circ. Res."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"233","DOI":"10.1016\/j.ccc.2019.11.002","article-title":"Platelet Activation and Endothelial Cell Dysfunction","volume":"36","author":"Parker","year":"2020","journal-title":"Crit. Care Clin."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"713","DOI":"10.1007\/s00109-022-02197-0","article-title":"Nets, pulmonary arterial hypertension, and thrombo-inflammation","volume":"100","author":"Santos","year":"2022","journal-title":"J. Mol. Med."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"57","DOI":"10.2214\/AJR.23.28996","article-title":"In Situ Pulmonary Arterial Thrombosis: Literature Review and Clinical Significance of a Distinct Entity","volume":"221","author":"Baranga","year":"2023","journal-title":"AJR Am. J. Roentgenol."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"3355","DOI":"10.1016\/j.jtha.2024.08.011","article-title":"Endothelial colony-forming cells in the spotlight: Insights into the pathophysiology of von Willebrand disease and rare bleeding disorders","volume":"22","author":"Laan","year":"2024","journal-title":"J. Thromb. Haemost."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"312","DOI":"10.1016\/j.advms.2018.04.013","article-title":"The role of platelets in the development and progression of pulmonary arterial hypertension","volume":"63","author":"Kazimierczyk","year":"2018","journal-title":"Adv. Med. Sci."},{"key":"ref_40","first-page":"1457","article-title":"Thromboxane and prostacyclin metabolites in pulmonary hypertension","volume":"327","author":"Rostagno","year":"1992","journal-title":"N. Engl. J. Med."},{"key":"ref_41","first-page":"100907","article-title":"Platelet-derived microparticles and their cargos: The past, present and future","volume":"19","author":"Guo","year":"2024","journal-title":"Asian J. Pharm. Sci."},{"key":"ref_42","doi-asserted-by":"crossref","unstructured":"Choi, G.Y., and Calder, P.C. (2024). The differential effects of eicosapentaenoic acid and docosahexaenoic acid on cardiovascular risk factors: An updated systematic review of randomized controlled trials. Front. Nutr., 11.","DOI":"10.3389\/fnut.2024.1423228"},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"286","DOI":"10.1161\/CIRCULATIONAHA.120.052318","article-title":"Integrated Single-Cell Atlas of Endothelial Cells of the Human Lung","volume":"144","author":"Schupp","year":"2021","journal-title":"Circulation"},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1016\/j.yjmcc.2024.10.004","article-title":"Panorama of artery endothelial cell dysfunction in pulmonary arterial hypertension","volume":"197","author":"Shen","year":"2024","journal-title":"J. Mol. Cell Cardiol."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"785","DOI":"10.1038\/s41586-020-2822-7","article-title":"Capillary cell-type specialization in the alveolus","volume":"586","author":"Gillich","year":"2020","journal-title":"Nature"},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"1308","DOI":"10.1161\/CIRCULATIONAHA.121.054071","article-title":"Role of Venous Endothelial Cells in Developmental and Pathologic Angiogenesis","volume":"144","author":"Lee","year":"2021","journal-title":"Circulation"},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"a006429","DOI":"10.1101\/cshperspect.a006429","article-title":"Endothelial cell heterogeneity","volume":"2","author":"Aird","year":"2012","journal-title":"Cold Spring Harb. Perspect. Med."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"311","DOI":"10.1007\/s10456-021-09797-3","article-title":"Endothelial cell plasticity at the single-cell level","volume":"24","author":"Pasut","year":"2021","journal-title":"Angiogenesis"},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"551","DOI":"10.1146\/annurev-physiol-031620-123956","article-title":"Metabolism in Pulmonary Hypertension","volume":"83","author":"Xu","year":"2021","journal-title":"Annu. Rev. Physiol."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"285","DOI":"10.1177\/2045893217701438","article-title":"The cancer theory of pulmonary arterial hypertension","volume":"7","author":"Boucherat","year":"2017","journal-title":"Pulm. Circ."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"13394","DOI":"10.1073\/pnas.1821401116","article-title":"PFKFB3-mediated endothelial glycolysis promotes pulmonary hypertension","volume":"116","author":"Cao","year":"2019","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"1425","DOI":"10.1161\/ATVBAHA.116.307695","article-title":"JMJD8 Regulates Angiogenic Sprouting and Cellular Metabolism by Interacting with Pyruvate Kinase M2 in Endothelial Cells","volume":"36","author":"Boeckel","year":"2016","journal-title":"Arterioscler. Thromb. Vasc. Biol."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"e035174","DOI":"10.1161\/JAHA.123.035174","article-title":"G6pd(N126D) Variant Increases the Risk of Developing VEGFR (Vascular Endothelial Growth Factor Receptor) Blocker-Induced Pulmonary Vascular Disease","volume":"13","author":"Signoretti","year":"2024","journal-title":"J. Am. Heart Assoc."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"2451","DOI":"10.1161\/CIRCULATIONAHA.117.028034","article-title":"Identification of MicroRNA-124 as a Major Regulator of Enhanced Endothelial Cell Glycolysis in Pulmonary Arterial Hypertension via PTBP1 (Polypyrimidine Tract Binding Protein) and Pyruvate Kinase M2","volume":"136","author":"Caruso","year":"2017","journal-title":"Circulation"},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"109","DOI":"10.1161\/CIRCRESAHA.122.321554","article-title":"BMPR2 Mutation and Metabolic Reprogramming in Pulmonary Arterial Hypertension","volume":"132","author":"Cuthbertson","year":"2023","journal-title":"Circ. Res."},{"key":"ref_56","doi-asserted-by":"crossref","unstructured":"Song, Y., Jia, H., Ma, Q., Zhang, L., Lai, X., and Wang, Y. (2024). The causes of pulmonary hypertension and the benefits of aerobic exercise for pulmonary hypertension from an integrated perspective. Front. Physiol., 15.","DOI":"10.3389\/fphys.2024.1461519"},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"2110","DOI":"10.1161\/CIRCULATIONAHA.120.049922","article-title":"Fate and State of Vascular Smooth Muscle Cells in Atherosclerosis","volume":"143","author":"Miano","year":"2021","journal-title":"Circulation"},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"1715","DOI":"10.1161\/ATVBAHA.119.312131","article-title":"Smooth Muscle Cell Phenotypic Diversity","volume":"39","author":"Liu","year":"2019","journal-title":"Arterioscler. Thromb. Vasc. Biol."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"180","DOI":"10.1186\/s12964-022-00993-2","article-title":"How vascular smooth muscle cell phenotype switching contributes to vascular disease","volume":"20","author":"Cao","year":"2022","journal-title":"Cell Commun. Signal"},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"3592","DOI":"10.1111\/jcmm.13269","article-title":"microRNA let-7g suppresses PDGF-induced conversion of vascular smooth muscle cell into the synthetic phenotype","volume":"21","author":"Wang","year":"2017","journal-title":"J. Cell Mol. Med."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"2693","DOI":"10.1161\/ATVBAHA.121.316600","article-title":"Six Shades of Vascular Smooth Muscle Cells Illuminated by KLF4 (Kr\u00fcppel-Like Factor 4)","volume":"41","author":"Yap","year":"2021","journal-title":"Arterioscler. Thromb. Vasc. Biol."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"707","DOI":"10.1159\/000490024","article-title":"The CCL5\/CCR5 Axis Promotes Vascular Smooth Muscle Cell Proliferation and Atherogenic Phenotype Switching","volume":"47","author":"Lin","year":"2018","journal-title":"Cell Physiol. Biochem."},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"2154","DOI":"10.1016\/j.bbadis.2018.04.002","article-title":"Nesfatin-1 functions as a switch for phenotype transformation and proliferation of VSMCs in hypertensive vascular remodeling","volume":"1864","author":"Lu","year":"2018","journal-title":"Biochim. Biophys. Acta Mol. Basis Dis."},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"1152","DOI":"10.1016\/j.celrep.2018.03.043","article-title":"Cell Autonomous and Non-cell Autonomous Regulation of SMC Progenitors in Pulmonary Hypertension","volume":"23","author":"Sheikh","year":"2018","journal-title":"Cell Rep."},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"1545","DOI":"10.1161\/CIRCULATIONAHA.120.045750","article-title":"A Notch3-Marked Subpopulation of Vascular Smooth Muscle Cells Is the Cell of Origin for Occlusive Pulmonary Vascular Lesions","volume":"142","author":"Steffes","year":"2020","journal-title":"Circulation"},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"223","DOI":"10.1007\/s00109-017-1619-0","article-title":"FOXM1 promotes pulmonary artery smooth muscle cell expansion in pulmonary arterial hypertension","volume":"96","author":"Bourgeois","year":"2018","journal-title":"J. Mol. Med."},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"1289","DOI":"10.1038\/nm.3695","article-title":"Pro-proliferative and inflammatory signaling converge on FoxO1 transcription factor in pulmonary hypertension","volume":"20","author":"Savai","year":"2014","journal-title":"Nat. Med."},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"431","DOI":"10.1165\/rcmb.2011-0128OC","article-title":"Hypoxia-induced pulmonary arterial smooth muscle cell proliferation is controlled by forkhead box M1","volume":"46","author":"Raghavan","year":"2012","journal-title":"Am. J. Respir. Cell Mol. Biol."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1007\/s11882-024-01181-7","article-title":"Mechanisms of Bone Morphogenetic Protein 2 in Respiratory Diseases","volume":"25","author":"Wen","year":"2024","journal-title":"Curr. Allergy Asthma Rep."},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"L246","DOI":"10.1152\/ajplung.00011.2023","article-title":"Contribution of transient receptor potential canonical channels in human and experimental pulmonary arterial hypertension","volume":"325","author":"Masson","year":"2023","journal-title":"Am. J. Physiol. Lung Cell Mol. Physiol."},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"535","DOI":"10.1084\/jem.20101812","article-title":"Role for miR-204 in human pulmonary arterial hypertension","volume":"208","author":"Courboulin","year":"2011","journal-title":"J. Exp. Med."},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"175725","DOI":"10.1016\/j.ejphar.2023.175725","article-title":"Endothelial progenitor cell-derived exosomes inhibit pulmonary artery smooth muscle cell in vitro proliferation and resistance to apoptosis by modulating the Mitofusin-2 and Ras-Raf-ERK1\/2 signaling pathway","volume":"949","author":"Liu","year":"2023","journal-title":"Eur. J. Pharmacol."},{"key":"ref_73","doi-asserted-by":"crossref","unstructured":"Chan, G.H., Chan, E., Kwok, C.T., Leung, G.P., Lee, S.M., and Seto, S.W. (2022). The role of p53 in the alternation of vascular functions. Front. Pharmacol., 13.","DOI":"10.3389\/fphar.2022.981152"},{"key":"ref_74","doi-asserted-by":"crossref","unstructured":"Kr\u00fcger-Genge, A., Blocki, A., Franke, R.P., and Jung, F. (2019). Vascular Endothelial Cell Biology: An Update. Int. J. Mol. Sci., 20.","DOI":"10.3390\/ijms20184411"},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"500","DOI":"10.1096\/fj.01-0842com","article-title":"Vascular smooth muscle and nitric oxide synthase","volume":"16","author":"Buchwalow","year":"2002","journal-title":"FASEB J."},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"2293","DOI":"10.1161\/ATVBAHA.120.314152","article-title":"Role of Extracellular Vesicles in Pulmonary Arterial Hypertension: Modulation of Pulmonary Endothelial Function and Angiogenesis","volume":"40","author":"Khandagale","year":"2020","journal-title":"Arterioscler. Thromb. Vasc. Biol."},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1016\/j.yjmcc.2021.07.010","article-title":"MicroRNA-663 prevents monocrotaline-induced pulmonary arterial hypertension by targeting TGF-\u03b21\/smad2\/3 signaling","volume":"161","author":"Li","year":"2021","journal-title":"J. Mol. Cell Cardiol."},{"key":"ref_78","doi-asserted-by":"crossref","unstructured":"Tomaszewski, M., Stycze\u0144, A., Krysa, M., Michalski, A., Morawska-Michalska, I., Hymos, A., Wawer, J., Roli\u0144ska, A., Rahnama, M., and Urbanowicz, T. (2024). Lymphocyte Involvement in the Pathology of Pulmonary Arterial Hypertension. Int. J. Mol. Sci., 25.","DOI":"10.3390\/ijms252413455"},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"3409","DOI":"10.1038\/s41467-022-30633-9","article-title":"Identification, discrimination and heterogeneity of fibroblasts","volume":"13","author":"Lendahl","year":"2022","journal-title":"Nat. Commun."},{"key":"ref_80","doi-asserted-by":"crossref","unstructured":"Zhang, H., Li, M., Hu, C.J., and Stenmark, K.R. (2024). Fibroblasts in Pulmonary Hypertension: Roles and Molecular Mechanisms. Cells, 13.","DOI":"10.3390\/cells13110914"},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"3519","DOI":"10.1096\/fj.201903188R","article-title":"Origin and functional heterogeneity of fibroblasts","volume":"34","author":"LeBleu","year":"2020","journal-title":"FASEB J."},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"C416","DOI":"10.1152\/ajpcell.00169.2003","article-title":"Hypoxia induces differentiation of pulmonary artery adventitial fibroblasts into myofibroblasts","volume":"286","author":"Short","year":"2004","journal-title":"Am. J. Physiol. Cell Physiol."},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1111\/j.1067-1927.2005.130102.x","article-title":"Tissue repair, contraction, and the myofibroblast","volume":"13","author":"Chaponnier","year":"2005","journal-title":"Wound Repair. Regen."},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"675","DOI":"10.1161\/01.RES.0000243584.45145.3f","article-title":"Hypoxia-induced pulmonary vascular remodeling: Cellular and molecular mechanisms","volume":"99","author":"Stenmark","year":"2006","journal-title":"Circ. Res."},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"134","DOI":"10.1152\/physiol.00053.2005","article-title":"Role of the adventitia in pulmonary vascular remodeling","volume":"21","author":"Stenmark","year":"2006","journal-title":"Physiology"},{"key":"ref_86","doi-asserted-by":"crossref","unstructured":"Suzuki, Y., Kawasaki, T., Tatsumi, K., Okaya, T., Sato, S., Shimada, A., Misawa, T., Hatano, R., Morimoto, C., and Kasuya, Y. (2024). Transcriptome Analysis of Fibroblasts in Hypoxia-Induced Vascular Remodeling: Functional Roles of CD26\/DPP4. Int. J. Mol. Sci., 25.","DOI":"10.3390\/ijms252312599"},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"1977","DOI":"10.1007\/s00018-013-1478-y","article-title":"Development and pathologies of the arterial wall","volume":"71","author":"Seidelmann","year":"2014","journal-title":"Cell Mol. Life Sci."},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"575","DOI":"10.1038\/s41586-021-03549-5","article-title":"Cross-tissue organization of the fibroblast lineage","volume":"593","author":"Buechler","year":"2021","journal-title":"Nature"},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"1920","DOI":"10.1038\/s41467-020-15647-5","article-title":"Collagen-producing lung cell atlas identifies multiple subsets with distinct localization and relevance to fibrosis","volume":"11","author":"Tsukui","year":"2020","journal-title":"Nat. Commun."},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"114","DOI":"10.1038\/s41586-021-03569-1","article-title":"A molecular single-cell lung atlas of lethal COVID-19","volume":"595","author":"Melms","year":"2021","journal-title":"Nature"},{"key":"ref_91","doi-asserted-by":"crossref","unstructured":"Li, M., Riddle, S., Kumar, S., Poczobutt, J., McKeon, B.A., Frid, M.G., Ostaff, M., Reisz, J.A., Nemkov, T., and Fini, M.A. (2021). Microenvironmental Regulation of Macrophage Transcriptomic and Metabolomic Profiles in Pulmonary Hypertension. Front. Immunol., 12.","DOI":"10.3389\/fimmu.2021.640718"},{"key":"ref_92","doi-asserted-by":"crossref","first-page":"e148382","DOI":"10.1172\/jci.insight.148382","article-title":"Complement-containing small extracellular vesicles from adventitial fibroblasts induce proinflammatory and metabolic reprogramming in macrophages","volume":"6","author":"Kumar","year":"2021","journal-title":"JCI Insight"},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1161\/CIRCRESAHA.114.301633","article-title":"MicroRNA-124 controls the proliferative, migratory, and inflammatory phenotype of pulmonary vascular fibroblasts","volume":"114","author":"Wang","year":"2014","journal-title":"Circ. Res."},{"key":"ref_94","doi-asserted-by":"crossref","first-page":"1006","DOI":"10.1161\/CIRCULATIONAHA.114.008750","article-title":"Endothelial-to-mesenchymal transition in pulmonary hypertension","volume":"131","author":"Ranchoux","year":"2015","journal-title":"Circulation"},{"key":"ref_95","doi-asserted-by":"crossref","first-page":"2003957","DOI":"10.1183\/13993003.03957-2020","article-title":"Endothelial cells in the pathogenesis of pulmonary arterial hypertension","volume":"58","author":"Evans","year":"2021","journal-title":"Eur. Respir. J."},{"key":"ref_96","doi-asserted-by":"crossref","first-page":"108438","DOI":"10.1016\/j.pharmthera.2023.108438","article-title":"Pulmonary vascular fibrosis in pulmonary hypertension\u2014The role of the extracellular matrix as a therapeutic target","volume":"247","author":"Jandl","year":"2023","journal-title":"Pharmacol. Ther."},{"key":"ref_97","doi-asserted-by":"crossref","first-page":"RP98558","DOI":"10.7554\/eLife.98558.3","article-title":"Adventitial fibroblasts direct smooth muscle cell-state transition in pulmonary vascular disease","volume":"13","author":"Crnkovic","year":"2025","journal-title":"eLife"},{"key":"ref_98","doi-asserted-by":"crossref","first-page":"2505","DOI":"10.1161\/ATVBAHA.119.312907","article-title":"HIMF (Hypoxia-Induced Mitogenic Factor) Signaling Mediates the HMGB1 (High Mobility Group Box 1)-Dependent Endothelial and Smooth Muscle Cell Crosstalk in Pulmonary Hypertension","volume":"39","author":"Lin","year":"2019","journal-title":"Arterioscler. Thromb. Vasc. Biol."},{"key":"ref_99","doi-asserted-by":"crossref","first-page":"245","DOI":"10.1165\/rcmb.2021-0091TR","article-title":"Basement Membrane Extracellular Matrix Proteins in Pulmonary Vascular and Right Ventricular Remodeling in Pulmonary Hypertension","volume":"65","author":"Ambade","year":"2021","journal-title":"Am. J. Respir. Cell Mol. Biol."},{"key":"ref_100","doi-asserted-by":"crossref","first-page":"766","DOI":"10.1183\/09031936.00209911","article-title":"Matrix metalloproteinases and their inhibitors in pulmonary hypertension","volume":"40","author":"Chelladurai","year":"2012","journal-title":"Eur. Respir. J."},{"key":"ref_101","doi-asserted-by":"crossref","first-page":"L1002","DOI":"10.1152\/ajplung.00383.2014","article-title":"Compartment-specific expression of collagens and their processing enzymes in intrapulmonary arteries of IPAH patients","volume":"308","author":"Hoffmann","year":"2015","journal-title":"Am. J. Physiol. Lung Cell Mol. Physiol."},{"key":"ref_102","doi-asserted-by":"crossref","first-page":"L173","DOI":"10.1152\/ajplung.00022.2024","article-title":"A comprehensive map of proteoglycan expression and deposition in the pulmonary arterial wall in health and pulmonary hypertension","volume":"327","author":"Mutgan","year":"2024","journal-title":"Am. J. Physiol. Lung Cell Mol. Physiol."},{"key":"ref_103","doi-asserted-by":"crossref","first-page":"216","DOI":"10.1093\/ehjci\/jex015","article-title":"Pulmonary arterial stiffness indices assessed by intravascular ultrasound in children with early pulmonary vascular disease: Prediction of advanced disease and mortality during 20-year follow-up","volume":"19","author":"Ploegstra","year":"2018","journal-title":"Eur. Heart J. Cardiovasc. Imaging"},{"key":"ref_104","doi-asserted-by":"crossref","unstructured":"Sun, W., and Chan, S.Y. (2018). Pulmonary Arterial Stiffness: An Early and Pervasive Driver of Pulmonary Arterial Hypertension. Front. Med., 5.","DOI":"10.3389\/fmed.2018.00204"},{"key":"ref_105","doi-asserted-by":"crossref","first-page":"1178","DOI":"10.1183\/13993003.00741-2015","article-title":"Pulmonary vascular resistance and compliance relationship in pulmonary hypertension","volume":"46","author":"Chemla","year":"2015","journal-title":"Eur. Respir. J."},{"key":"ref_106","doi-asserted-by":"crossref","first-page":"1016","DOI":"10.1016\/j.celrep.2015.09.049","article-title":"Matrix Remodeling Promotes Pulmonary Hypertension through Feedback Mechanoactivation of the YAP\/TAZ-miR-130\/301 Circuit","volume":"13","author":"Bertero","year":"2015","journal-title":"Cell Rep."},{"key":"ref_107","doi-asserted-by":"crossref","first-page":"e86987","DOI":"10.1172\/jci.insight.86987","article-title":"Distal vessel stiffening is an early and pivotal mechanobiological regulator of vascular remodeling and pulmonary hypertension","volume":"1","author":"Liu","year":"2016","journal-title":"JCI Insight"},{"key":"ref_108","doi-asserted-by":"crossref","first-page":"H1322","DOI":"10.1152\/ajpheart.00136.2018","article-title":"Role of extracellular matrix in the pathogenesis of pulmonary arterial hypertension","volume":"315","author":"Thenappan","year":"2018","journal-title":"Am. J. Physiol. Heart Circ. Physiol."},{"key":"ref_109","doi-asserted-by":"crossref","first-page":"495","DOI":"10.1002\/(SICI)1097-4652(199603)166:3<495::AID-JCP4>3.0.CO;2-K","article-title":"Exogenous leukocyte and endogenous elastases can mediate mitogenic activity in pulmonary artery smooth muscle cells by release of extracellular-matrix bound basic fibroblast growth factor","volume":"166","author":"Thompson","year":"1996","journal-title":"J. Cell Physiol."},{"key":"ref_110","doi-asserted-by":"crossref","first-page":"1702403","DOI":"10.1183\/13993003.02403-2017","article-title":"How does inflammation contribute to pulmonary hypertension?","volume":"51","author":"Kumar","year":"2018","journal-title":"Eur. Respir. J."},{"key":"ref_111","doi-asserted-by":"crossref","unstructured":"Zhang, Y., Li, X., Li, S., Zhou, Y., Zhang, T., and Sun, L. (2024). Immunotherapy for Pulmonary Arterial Hypertension: From the Pathogenesis to Clinical Management. Int. J. Mol. Sci., 25.","DOI":"10.3390\/ijms25158427"},{"key":"ref_112","doi-asserted-by":"crossref","first-page":"1701214","DOI":"10.1183\/13993003.01214-2017","article-title":"The inflammatory cell landscape in the lungs of patients with idiopathic pulmonary arterial hypertension","volume":"51","author":"Marsh","year":"2018","journal-title":"Eur. Respir. J."},{"key":"ref_113","doi-asserted-by":"crossref","first-page":"165","DOI":"10.1161\/CIRCRESAHA.113.301141","article-title":"Inflammation and immunity in the pathogenesis of pulmonary arterial hypertension","volume":"115","author":"Rabinovitch","year":"2014","journal-title":"Circ. Res."},{"key":"ref_114","doi-asserted-by":"crossref","first-page":"236","DOI":"10.1161\/CIRCRESAHA.108.182014","article-title":"Interleukin-6 overexpression induces pulmonary hypertension","volume":"104","author":"Steiner","year":"2009","journal-title":"Circ. Res."},{"key":"ref_115","doi-asserted-by":"crossref","first-page":"L609","DOI":"10.1152\/ajplung.00531.2016","article-title":"Central role of T helper 17 cells in chronic hypoxia-induced pulmonary hypertension","volume":"312","author":"Maston","year":"2017","journal-title":"Am. J. Physiol. Lung Cell Mol. Physiol."},{"key":"ref_116","doi-asserted-by":"crossref","first-page":"247372","DOI":"10.1155\/2014\/247372","article-title":"Effect of Th17 and Treg axis disorder on outcomes of pulmonary arterial hypertension in connective tissue diseases","volume":"2014","author":"Gaowa","year":"2014","journal-title":"Mediat. Inflamm."},{"key":"ref_117","doi-asserted-by":"crossref","first-page":"1671","DOI":"10.1161\/CIRCULATIONAHA.107.694778","article-title":"Identification of a novel role of T cells in postnatal vasculogenesis: Characterization of endothelial progenitor cell colonies","volume":"116","author":"Hur","year":"2007","journal-title":"Circulation"},{"key":"ref_118","doi-asserted-by":"crossref","unstructured":"Zhao, H., Song, J., Li, X., Xia, Z., Wang, Q., Fu, J., Miao, Y., Wang, D., and Wang, X. (2024). The role of immune cells and inflammation in pulmonary hypertension: Mechanisms and implications. Front. Immunol., 15.","DOI":"10.3389\/fimmu.2024.1374506"},{"key":"ref_119","doi-asserted-by":"crossref","first-page":"2","DOI":"10.1016\/j.jdermsci.2018.11.008","article-title":"Regulatory and effector B cells: Friends or foes?","volume":"93","author":"Matsushita","year":"2019","journal-title":"J. Dermatol. Sci."},{"key":"ref_120","doi-asserted-by":"crossref","first-page":"L710","DOI":"10.1152\/ajplung.00311.2016","article-title":"The mast cell-B cell axis in lung vascular remodeling and pulmonary hypertension","volume":"312","author":"Breitling","year":"2017","journal-title":"Am. J. Physiol. Lung Cell Mol. Physiol."},{"key":"ref_121","doi-asserted-by":"crossref","first-page":"209","DOI":"10.1164\/rccm.202009-3481OC","article-title":"Safety and Efficacy of B-Cell Depletion with Rituximab for the Treatment of Systemic Sclerosis-associated Pulmonary Arterial Hypertension: A Multicenter, Double-Blind, Randomized, Placebo-controlled Trial","volume":"204","author":"Zamanian","year":"2021","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_122","doi-asserted-by":"crossref","unstructured":"Ross, E.A., Devitt, A., and Johnson, J.R. (2021). Macrophages: The Good, the Bad, and the Gluttony. Front. Immunol., 12.","DOI":"10.3389\/fimmu.2021.708186"},{"key":"ref_123","doi-asserted-by":"crossref","first-page":"200ra117","DOI":"10.1126\/scitranslmed.3006674","article-title":"Blocking macrophage leukotriene b4 prevents endothelial injury and reverses pulmonary hypertension","volume":"5","author":"Tian","year":"2013","journal-title":"Sci. Transl. Med."},{"key":"ref_124","doi-asserted-by":"crossref","first-page":"430","DOI":"10.1161\/ATVBAHA.120.314639","article-title":"Altered Macrophage Polarization Induces Experimental Pulmonary Hypertension and Is Observed in Patients with Pulmonary Arterial Hypertension","volume":"41","author":"Zawia","year":"2021","journal-title":"Arterioscler. Thromb. Vasc. Biol."},{"key":"ref_125","doi-asserted-by":"crossref","first-page":"428","DOI":"10.1186\/s12931-024-03058-9","article-title":"T follicular helper cell is essential for M2 macrophage polarization and pulmonary vascular remodeling in hypoxia-induced pulmonary hypertension","volume":"25","author":"Li","year":"2024","journal-title":"Respir. Res."},{"key":"ref_126","doi-asserted-by":"crossref","first-page":"597","DOI":"10.1165\/rcmb.2016-0201OC","article-title":"Roles for the CX3CL1\/CX3CR1 and CCL2\/CCR2 Chemokine Systems in Hypoxic Pulmonary Hypertension","volume":"56","author":"Amsellem","year":"2017","journal-title":"Am. J. Respir. Cell Mol. Biol."},{"key":"ref_127","doi-asserted-by":"crossref","first-page":"1534","DOI":"10.1016\/j.jaci.2022.05.028","article-title":"Mast cell chymase regulates extracellular matrix remodeling-related events in primary human small airway epithelial cells","volume":"150","author":"Zhao","year":"2022","journal-title":"J. Allergy Clin. Immunol."},{"key":"ref_128","doi-asserted-by":"crossref","first-page":"1179","DOI":"10.1161\/CIRCRESAHA.111.257568","article-title":"PAR-2 inhibition reverses experimental pulmonary hypertension","volume":"110","author":"Kwapiszewska","year":"2012","journal-title":"Circ. Res."},{"key":"ref_129","doi-asserted-by":"crossref","unstructured":"Moriyama, H., and Endo, J. (2023). Pathophysiological Involvement of Mast Cells and the Lipid Mediators in Pulmonary Vascular Remodeling. Int. J. Mol. Sci., 24.","DOI":"10.3390\/ijms24076619"},{"key":"ref_130","doi-asserted-by":"crossref","first-page":"342","DOI":"10.1007\/s12016-019-08769-2","article-title":"Mast Cell Biology at Molecular Level: A Comprehensive Review","volume":"58","author":"Bielory","year":"2020","journal-title":"Clin. Rev. Allergy Immunol."},{"key":"ref_131","doi-asserted-by":"crossref","first-page":"452","DOI":"10.1086\/677359","article-title":"Imatinib in pulmonary arterial hypertension: C-Kit inhibition","volume":"4","author":"Farha","year":"2014","journal-title":"Pulm. Circ."},{"key":"ref_132","doi-asserted-by":"crossref","first-page":"e70015","DOI":"10.1096\/fj.202400338RR","article-title":"Classical dendritic cells contribute to hypoxia-induced pulmonary hypertension","volume":"38","author":"Mickael","year":"2024","journal-title":"FASEB J."},{"key":"ref_133","doi-asserted-by":"crossref","first-page":"665","DOI":"10.1165\/rcmb.2019-0443OC","article-title":"DNGR1-Cre-mediated Deletion of Tnfaip3\/A20 in Conventional Dendritic Cells Induces Pulmonary Hypertension in Mice","volume":"63","author":"Koudstaal","year":"2020","journal-title":"Am. J. Respir. Cell Mol. Biol."},{"key":"ref_134","doi-asserted-by":"crossref","first-page":"2209","DOI":"10.1038\/s41590-024-02006-5","article-title":"The diverse roles of neutrophils from protection to pathogenesis","volume":"25","author":"Herro","year":"2024","journal-title":"Nat. Immunol."},{"key":"ref_135","doi-asserted-by":"crossref","first-page":"1177","DOI":"10.1016\/j.chest.2021.06.068","article-title":"Neutrophil Elastase: Finding Its Way from Mechanism, via Biomarker to Treatment Target in Pulmonary Hypertension?","volume":"160","author":"Bogaard","year":"2021","journal-title":"Chest"},{"key":"ref_136","doi-asserted-by":"crossref","first-page":"1442","DOI":"10.1016\/j.chest.2021.06.028","article-title":"Severe Pulmonary Arterial Hypertension Is Characterized by Increased Neutrophil Elastase and Relative Elafin Deficiency","volume":"160","author":"Sweatt","year":"2021","journal-title":"Chest"},{"key":"ref_137","doi-asserted-by":"crossref","first-page":"2078","DOI":"10.1161\/ATVBAHA.116.307634","article-title":"Neutrophil Extracellular Traps Promote Angiogenesis: Evidence from Vascular Pathology in Pulmonary Hypertension","volume":"36","author":"Aldabbous","year":"2016","journal-title":"Arterioscler. Thromb. Vasc. Biol."},{"key":"ref_138","doi-asserted-by":"crossref","unstructured":"Dierick, F., Solinc, J., Bignard, J., Soubrier, F., and Nadaud, S. (2021). Progenitor\/Stem Cells in Vascular Remodeling during Pulmonary Arterial Hypertension. Cells, 10.","DOI":"10.3390\/cells10061338"},{"key":"ref_139","doi-asserted-by":"crossref","first-page":"780","DOI":"10.1164\/rccm.200810-1662OC","article-title":"Evidence of dysfunction of endothelial progenitors in pulmonary arterial hypertension","volume":"180","author":"Toshner","year":"2009","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_140","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1016\/j.semcdb.2022.01.008","article-title":"Specification of the haematopoietic stem cell lineage: From blood-fated mesodermal angioblasts to haemogenic endothelium","volume":"127","author":"Ho","year":"2022","journal-title":"Semin. Cell Dev. Biol."},{"key":"ref_141","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1016\/j.yjmcc.2021.10.009","article-title":"Endothelial repair by stem and progenitor cells","volume":"163","author":"Wang","year":"2022","journal-title":"J. Mol. Cell Cardiol."},{"key":"ref_142","doi-asserted-by":"crossref","unstructured":"Perrotta, F., Perna, A., Komici, K., Nigro, E., Mollica, M., D\u2019Agnano, V., De Luca, A., and Guerra, G. (2020). The State of Art of Regenerative Therapy in Cardiovascular Ischemic Disease: Biology, Signaling Pathways, and Epigenetics of Endothelial Progenitor Cells. Cells, 9.","DOI":"10.3390\/cells9081886"},{"key":"ref_143","doi-asserted-by":"crossref","first-page":"475","DOI":"10.4103\/2045-8932.93547","article-title":"Pulmonary artery endothelium resident endothelial colony-forming cells in pulmonary arterial hypertension","volume":"1","author":"Duong","year":"2011","journal-title":"Pulm. Circ."},{"key":"ref_144","doi-asserted-by":"crossref","first-page":"116","DOI":"10.1164\/rccm.201006-0905OC","article-title":"C-kit-positive cells accumulate in remodeled vessels of idiopathic pulmonary arterial hypertension","volume":"184","author":"Montani","year":"2011","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_145","doi-asserted-by":"crossref","first-page":"2227","DOI":"10.1002\/j.2040-4603.2021.tb00178.x","article-title":"Lung Pericytes in Pulmonary Vascular Physiology and Pathophysiology","volume":"11","author":"Yuan","year":"2021","journal-title":"Compr. Physiol."},{"key":"ref_146","doi-asserted-by":"crossref","first-page":"447","DOI":"10.1002\/path.5152","article-title":"Targeting PDGF-mediated recruitment of pericytes blocks vascular mimicry and tumor growth","volume":"246","author":"Thijssen","year":"2018","journal-title":"J. Pathol."},{"key":"ref_147","doi-asserted-by":"crossref","first-page":"001634","DOI":"10.1099\/jgv.0.001634","article-title":"The emerging role of perivascular cells (pericytes) in viral pathogenesis","volume":"102","author":"Butsabong","year":"2021","journal-title":"J. Gen. Virol."},{"key":"ref_148","doi-asserted-by":"crossref","first-page":"766","DOI":"10.1161\/ATVBAHA.119.313715","article-title":"Lineage Tracing Reveals the Dynamic Contribution of Pericytes to the Blood Vessel Remodeling in Pulmonary Hypertension","volume":"40","author":"Bordenave","year":"2020","journal-title":"Arterioscler. Thromb. Vasc. Biol."},{"key":"ref_149","doi-asserted-by":"crossref","first-page":"31","DOI":"10.4103\/2045-8932.109912","article-title":"Dysfunctional resident lung mesenchymal stem cells contribute to pulmonary microvascular remodeling","volume":"3","author":"Chow","year":"2013","journal-title":"Pulm. 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