Early events marking lung fibroblast transition to profibrotic state in idiopathic pulmonary fibrosis.

ABSTRACT: Idiopathic Pulmonary Fibrosis (IPF) is an age-associated progressive lung disease with accumulation of scar tissue impairing gas exchange. Previous high-throughput studies elucidated the role of cellular heterogeneity and molecular pathways in advanced disease. However, critical pathogenic pathways occurring in the transition of fibroblasts from normal to profibrotic have been largely overlooked. We used single cell transcriptomics (scRNA-seq) from lungs of healthy controls and IPF patients (lower and upper lobes). We identified fibroblast subclusters, genes and pathways associated with early disease. Immunofluorescence assays validated the role of MOXD1 early in fibrosis. We identified four distinct fibroblast subgroups, including one marking the normal-to-profibrotic state transition. Ou

SUBMITTER: Jia M 

PROVIDER: S-EPMC10122312 | biostudies-literature | 2023 Apr

REPOSITORIES: biostudies-literature