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Pulmonary Hypertension: Functional Genomics and Therapy of Lung Vascular Remodelling


Pulmonary hypertension (PH) describes devastating diseases, comprising idiopathic and secondary forms, which cause breathlessness and premature death, representing a major burden on healthcare systems. Extensive lung vascular remodelling with loss of vessel patency is the underlying pathomechanism. PULMOTENSION integrates the top EU Centres in PH and lung vascular biology in a multidisciplinary approach, aiming to combat and finally cure PH. The expertise of all members extends from the initial discovery of gene mutations causative of PH to having established new therapeutic regimen of PH. We will uncover underlying molecular pathways, identify distinct targets for anti-remodelling therapy, foster drug development based on these targets, and test these new treatment options in preclinical and clinical trials. The presented project includes a) genetics of PH, b) functional genomics and proteomics to unravel the molecular pathways underlying vascular remodelling, c) characterisation of the cross-talk between different cells and signalling pathways involved, d) preclinical trials in PH models including transgenics, e) a European PH Tissue Bank and Registry, f) conversion of target discovery into drug design and exploitation, g) development of non-invasive clinical tools for monitoring lung vascular remodelling in patients, h) a Clinical Trial Network to conduct phase I/II(III) studies focussing on anti- and reversal of remodelling in PH patients, and i) promotion of a world-leading infrastructure for scientific and technical competence in the field of PH, including training and mobility programs, technology transfer in alliance with industry and exploitation facilities. Gender issues will be particularly addressed, as many PH forms have a female preponderance. Hence, this ambitious translational research programme will span from molecules to patients to the benefit of molecular understanding and new treatments of PH, a prototype disease of vascular remo

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Ludwigstrasse 23

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Participants (30)