Comparison of Human and Experimental Pulmonary Veno-Occlusive Disease

What is it about?

Pulmonary veno-occlusive disease is a severe and rapidly fatal form of pulmonary hypertension that, albeit the established association with germline mutations in the EIF2AK4 gene (coding for GCN2), remains obscure with regard to pathophysiology and disease evolution. For the first time, we link human hereditary and experimental gene inactivation to GCN2-dependent protein overexpression within vascular lesions that primarily develop in the pre-capillary pulmonary vasculature and only secondarily in pulmonary veins.

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