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Pulmonary vascular remodeling patterns and expression of general control nonderepressible 2 (GCN2) in pulmonary veno-occlusive disease

Archive ouverte | Nossent, Esther | CCSD

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Comparison of Human and Experimental Pulmonary Veno-Occlusive Disease

Archive ouverte | Manaud, Grégoire | CCSD

International audience. Pulmonary veno-occlusive disease (PVOD) occurs in humans either as heritable form (hPVOD) due to biallelic inactivating mutations of EIF2AK4 (encoding GCN2), or as a sporadic form at older ag...

Articles Clinical phenotypes and outcomes of heritable and sporadic pulmonary veno-occlusive disease: a population-based study

Archive ouverte | Montani, David | CCSD

International audience. BackgroundBi-allelic mutations of the EIF2AK4 gene cause heritable pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis (PVOD/PCH). We aimed to assess the eff ect of E...

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