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PULMOTENSION — Result In Brief

Project ID: 18725
Country: Germany

Pulmonary hypertension: from molecules to therapy

European scientists joined forces to find novel disease targets and therapies for pulmonary hypertension.
Pulmonary hypertension: from molecules to therapy
Pulmonary hypertension (PH) describes a group of conditions caused by increased blood pressure in the lung vasculature. The nature of these conditions can be hereditary, idiopathic or secondary leading to shortness of breath and cardiac failure. This is caused by extensive remodelling and loss of patency of lung vessels.

To investigate the underlying mechanisms in PH, the EU-funded 'Pulmonary hypertension: functional genomics and therapy of lung vascular remodelling' (Pulmotension) project integrated leading European centres in the field. Team members analysed different pathways involved in the vascular remodelling process, which takes place in the lung and is responsible for increased vascular resistance. The consortium used a range of technologies such as genetic analysis, functional genomics and proteomics to analyse clinical tissue bank material.

Study results identified distinct genes and causative mutations as novel therapeutic targets, fostering the development of new drugs which were assessed in pre-clinical animal models of PH. The main focus of the study was to reverse the remodelling process in PH patients.

The consortium undertook an extensive programme translating molecules to clinical practice and generated significant new knowledge on the underlying pathology of PH. New diagnostic and therapeutic approaches were evaluated and standardised throughout Europe, and two clinical trials were conducted.

Alongside the promotion of innovative therapies, study outcomes also greatly benefited the European infrastructure for scientific and technical competence in the field of PH.

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