|CIBERES (coordinator group)||Maria Molina Molina, IDIBELL|
Idiopathic Pulmonary Fibrosis (IPF) is a lethal interstitial lung disease, with no effective treatment and a mean survival time of 3-4 years from the diagnosis. While normal lungs have a reparative answer that leads to tissue restoration, different altered mechanisms depending on cell and extra cellular matrix (ECM) components induce the fibrotic-scared formation in IPF lungs. The improvement in the knowledge about key pathogenic pro-fibrotic pathways and the establishment of a new experimental model are crucial to find an effective therapeutic approach that could be translated in humans and combat the complexity of human lung fibrosis. The planned activities will include new and innovative research approaches focused on the investigation of pro-fibrotic cell behavior (microenvironment interactions, cell plasticity and metabolism), in order to find the main clues to inhibit the lung fibrotic process and to repair the damaged lung tissue. For these purposes normal and fibrotic alveolar epithelial cells (AECs), fibroblasts, endothelial and mesenchymal stem cells (MSCs) will be used and evaluated on conventional cell cultures, co-cultures and three-dimensional cell cultures.