ArticleNature cardiovascular research2024
Reversal of pulmonary veno-occlusive disease phenotypes by inhibition of the integrated stress response.
Article in Nature cardiovascular research, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 9 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
9 citing papers in PubMed.
- Alkylating Agent-Induced FANCL Impairment Damages Pulmonary Arterial, Venous, and Microvascular Endothelial Cells and Predisposes to PAH/PVOD.Advanced science (Weinheim, Baden-Wurttemberg, Germany) · 2026Article
- Interleukin-6 is critical in the development of pulmonary vascular disease inProceedings of the National Academy of Sciences of the United States of America · 2026Article
- GDF15 is a putative biomarker for distinguishing pulmonary veno-occlusive disease and pulmonary arterial hypertension.The Journal of clinical investigation · 2026Article
- Review
- Pulmonary veno-occlusive disease: Insights from animal models.Vascular pharmacology · 2025Review
- Essential role of protein kinase R in the pathogenesis of pulmonary veno-occlusive disease.JCI insight · 2025Article
- Bridging the species divide: The limits of rat models in capturing human PVOD mechanisms.Pulmonary circulation · 2024Article
- Reply to the letter to the editor entitled "Bridging the species divide: The limits of rat models in capturing human PVOD mechanisms" by Perros F. et al.Pulmonary circulation · 2024Article
- Mechanisms underlying age-associated exacerbation of pulmonary veno-occlusive disease.JCI insight · 2024Article
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14 authors.
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Abstract
Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary hypertension arising from EIF2AK4 gene mutations or mitomycin C (MMC) administration. The lack of effective PVOD therapies is compounded by a limited understanding of the mechanisms driving vascular remodeling in PVOD. Here we show that administration of MMC in rats mediates activation of protein kinase R (PKR) and the integrated stress response (ISR), which leads to the release of the endothelial adhesion molecule vascular endothelial (VE) cadherin (VE-Cad) in complex with RAD51 to the circulation, disruption of endothelial barrier and vascular remodeling. Pharmacological inhibition of PKR or ISR attenuates VE-Cad depletion, elevation of vascular permeability and vascular remodeling instigated by MMC, suggesting potential clinical intervention for PVOD. Finally, the severity of PVOD phenotypes was increased by a heterozygous BMPR2 mutation that truncates the carboxyl tail of the receptor BMPR2, underscoring the role of deregulated bone morphogenetic protein signaling in the development of PVOD.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.