When cell teamwork turns toxic
- PMID: 40207910
- PMCID: PMC11984950
- DOI: 10.7554/eLife.106689
When cell teamwork turns toxic
Abstract
In pulmonary hypertension, a combination of metabolic and mechanical dysfunction leads to irreversible vascular damage.
Keywords: adventitial fibroblasts; cell biology; human; medicine; pulmonary artery; pulmonary hypertension; vascular smooth muscle cells.
© 2025, Khoury and Chan.
Conflict of interest statement
WK No competing interests declared, SC Consultant for Merck, Janssen, and United Therapeutics; director, officer, and shareholder in Synhale Therapeutics; has held grants from Bayer and United Therapeutics; has filed patent applications regarding metabolism and next-generation therapeutics in pulmonary hypertension (US 10,925,869 B2; PCT/US2015/029286; PCT/US2018/062013; US 11,773,391 B2; PCT/IB2023/055431)
Figures

Comment on
- doi: 10.7554/eLife.98558
References
-
- Crnkovic S, Valzano F, Fließer E, Gindlhuber J, Thekkekara Puthenparampil H, Basil M, Morley MP, Katzen J, Gschwandtner E, Klepetko W, Cantu E, Wolinski H, Olschewski H, Lindenmann J, Zhao YY, Morrisey EE, Marsh LM, Kwapiszewska G. Single-cell transcriptomics reveals skewed cellular communication and phenotypic shift in pulmonary artery remodeling. JCI Insight. 2022;7:20. doi: 10.1172/jci.insight.153471. - DOI - PMC - PubMed
-
- Crnkovic S, Puthenparampil HT, Mulch S, Biasin V, Radic N, Wilhelm J, Bartkuhn M, Rad EB, Wawrzen A, Matzer I, Mitra A, Leib R, Nagy BM, Sahu-Osen A, Valzano F, Bordag N, Evermann M, Hoetzenecker K, Olschewski A, Ljubojevic-Holzer S, Wygrecka M, Stenmark K, Marsh LM, de Jesus Perez V, Kwapiszewska G. Adventitial fibroblasts direct smooth muscle cell-state transition in pulmonary vascular disease. eLife. 2025;13:RP98558. doi: 10.7554/eLife.98558. - DOI - PMC - PubMed
-
- El Kasmi KC, Pugliese SC, Riddle SR, Poth JM, Anderson AL, Frid MG, Li M, Pullamsetti SS, Savai R, Nagel MA, Fini MA, Graham BB, Tuder RM, Friedman JE, Eltzschig HK, Sokol RJ, Stenmark KR. Adventitial fibroblasts induce a distinct proinflammatory/profibrotic macrophage phenotype in pulmonary hypertension. Journal of Immunology. 2014;193:597–609. doi: 10.4049/jimmunol.1303048. - DOI - PMC - PubMed
-
- Frid MG, McKeon BA, Thurman JM, Maron BA, Li M, Zhang H, Kumar S, Sullivan T, Laskowsky J, Fini MA, Hu S, Tuder RM, Gandjeva A, Wilkins MR, Rhodes CJ, Ghataorhe P, Leopold JA, Wang RS, Holers VM, Stenmark KR. Immunoglobulin-driven complement activation regulates proinflammatory remodeling in pulmonary hypertension. American Journal of Respiratory and Critical Care Medicine. 2020;201:224–239. doi: 10.1164/rccm.201903-0591OC. - DOI - PMC - PubMed
Publication types
MeSH terms
LinkOut - more resources
Full Text Sources
Medical