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ABSTRACT: Objectives
Systemic sclerosis (SSc) has the highest mortality rate among the rheumatic diseases, with lung fibrosis leading as the cause of death. A characteristic of severe SSc-related lung fibrosis is its progressive nature. Although most research has focused on the pathology of the fibrosis, the mechanism mediating the fibrotic spread remains unclear. We hypothesized that extracellular vesicle (EV) communication drives the propagation of SSc lung fibrosis.Methods
EVs were isolated from normal (NL) or SSc-derived human lungs and primary lung fibroblasts (pLFs). EVs were also isolated from human fibrotic lungs and pLFs induced experimentally with transforming growth factor-β (TGFβ). Fibrotic potency of EVs was assessed using functional assays in vitro and in vivo. Transmis
SUBMITTER: Mouawad JE
PROVIDER: S-EPMC10756928 | biostudies-literature | 2023 Dec
REPOSITORIES: biostudies-literature