Article
Comparison of Human and Experimental Pulmonary Veno-Occlusive Disease.
American journal of respiratory cell and molecular biology - 1 Jul 2020
Manaud Grégoire, Nossent Esther J, Lambert Mélanie, Ghigna Maria-Rosa, Boët Angèle, Vinhas Maria-Candida, Ranchoux Benoit, Dumas Sébastien J, Courboulin Audrey, Girerd Barbara, Soubrier Florent, Bignard Juliette, Claude Olivier, Lecerf Florence, Hautefort Aurélie, Florio Monica, Sun Banghua, Nadaud Sophie, Verleden Stijn E, Remy Séverine, Anegon Ignacio, Bogaard Harm Jan, Mercier Olaf, Fadel Elie, Simonneau Gérald, Vonk Noordegraaf Anton, Grünberg Katrien, Humbert Marc, Montani David, Dorfmüller Peter, Antigny Fabrice, Perros Frédéric
Abstract excerpt
Pulmonary veno-occlusive disease (PVOD) occurs in humans either as a heritable form (hPVOD) due to biallelic inactivating mutations of EIF2AK4 (encoding GCN2) or as a sporadic form in older age (sPVOD). The chemotherapeutic agent mitomycin C (MMC) is a potent inducer of PVOD in humans and in rats (MMC-PVOD). Here, we compared human hPVOD and sPVOD, and MMC-PVOD pathophysiology at the histological, cellular, and...
Topics
- Animals
- Disease Models, Animal
- Endothelial Cells
- Humans
- Hypertension, Pulmonary
- Lung
- Mutation
- Pulmonary Artery