IPFCELPLAST

Cell plasticity and microenvironment in lung fibrosis: Looking for its regulation as a potential treatment. IPFCELPLAST

InstitutionPrincipal Investigator
CIBERES (coordinator group) Maria Molina Molina, IDIBELL
CIBERES 
  • Ernest Sala, Hospital Son Espases-FISIB
  • Antoni Xaubet, Hospital Clínic de Barcelona-IDIBAPS
  • Julio Cortijo, Universidad de Valencia-Hospital Universitario de Valencia
  • Miriam Echevarría, Instituto de Investigación Biomédica de Sevilla-IBIS
SEPAR
  • Jose Antonio Rodriguez – Portal, PII-EPID, Hospital Virgen del Rocío de Sevilla
  • Julio Ancochea, Hospital La Princesa de Madrid
  • David Iturbe, Hospital Marqués de Valdecillas Santander
  • Diego Castillo, Hospital de Sant Pau Barcelona
  • Jaume Sauleda, Hospital Son Espases-FISIB
  • L. Molins. Hospital Clínic de Barcelona-IDIBAPS
CIBER-BBN
  • Jordi Aguiló, GBIO-CNM
  • Fernando Albericio, IRB
  • Bernardo Celda, GABBRMN-UVEG
  • Soledad Penadés LNB-CICBIO
  • Javier Pavia, GIB-UB

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.