Profibrotic
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Related Articles from SNS
Anti-Melanoma Differentiation-Associated protein 5 Auto-Antibodies Promote a Profibrotic Phenotype in a Human Lung Fibroblast Cell Line
Anti-melanoma differentiation-associated protein 5 (anti-MDA5) autoantibodies identify a distinct dermatomyositis subset frequently associated with rapidly progressive interstitial lung disease (RP-ILD). While these antibodies are established disease markers, their direct contribution to pulmonary fibrosis is poorly defined. This study investigated the pathogenic effects of patient-derived polyclonal anti-MDA5 antibodies on IMR-90 human lung fibroblasts.
Alveolar Epithelial Cell Loss of the Mitochondrial Regulator TFAM Drives Progressive Lung Fibrosis
Idiopathic pulmonary fibrosis (IPF) is characterized by failed alveolar epithelial repair and progressive fibrotic remodeling. Although aberrant reprogramming of alveolar type 2 (AT2) cells and accumulation of transitional AT2 states are increasing recognized as central features of IPF, the epithelial-intrinsic mechanisms that initiate these pathogenic states remain incompletely understood. Here, we identify mitochondrial transcription factor A (TFAM), a regulator of mitochondrial DNA...
Alveolar niche disruption and aberrant epithelial reprogramming are early hallmarks of idiopathic pulmonary fibrosis
Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial lung disease in which the earliest cellular events driving fibrosis remain poorly defined. Here, we analyzed lung samples from three independent and unique cohorts of patients with early disease and preserved lung function (Florence, NIH, Forli), applying an integrated multimodal approach combining single-nucleus RNA sequencing, bulk transcriptomics, immunostaining, and spatial transcriptomics. Single-nuclear RNA sequencing of...