Article
Reversal of pulmonary veno-occlusive disease phenotypes by inhibition of the integrated stress response.
Nature cardiovascular research - 1 Jul 2024
Prabhakar Amit, Kumar Rahul, Wadhwa Meetu, Ghatpande Prajakta, Zhang Jingkun, Zhao Ziwen, Lizama Carlos O, Kharbikar Bhushan N, Gräf Stefan, Treacy Carmen M, Morrell Nicholas W, Graham Brian B, Lagna Giorgio, Hata Akiko
Abstract excerpt
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...
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