Pulmonary fibrosis (PF) is a lethal, progressive lung disorder affecting millions of patients worldwide, with limited therapeutic options. Inflammatory infiltration and fibroblast-to-myofibroblast activation leading to excessive extracellular matrix deposition underlie the pathogenesis of PF. While pathways promoting these cellular alterations have been described, endogenous factors counter-regulating such changes are barely known. Our recent observations suggest that the endothelial hormone C-type natriuretic peptide (CNP) may moderate pathological fibroblast-to-myofibroblast differentiation and thereby lung inflammatory infiltration and fibrosis. The proposed studies aim to unravel the mechanisms and clinical implications of this paracrine pathway by employing a novel genetic mouse model and cultured murine and human fibroblasts.
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