PEAR1 and pulmonary fibrosis: Accordingly, functional analysis revealed an enrichment of activated fibroblast marker genes in ECM development and lung fibrosis (Fig. 2f, g), indicating that Pear1 deficiency reshapes mesenchymal components to a new pattern that favors the exaggeration of the disease through higher collagen deposition, decreased pulmonary function and poor prognosis (Fig. 1).