Adventitial fibroblasts direct smooth muscle cell-state transition in pulmonary vascular disease.

ABSTRACT:

Background

Pulmonary vascular remodeling is a progressive pathological process characterized by functional alterations within pulmonary artery smooth muscle cells (PASMCs) and adventitial fibroblasts (PAAFs). Mechanisms driving the transition to a diseased phenotype remain elusive.

Methods

We combined transcriptomic and proteomic profiling with phenotypic characterization of source-matched cells from healthy controls and individuals with idiopathic pulmonary arterial hypertension (IPAH). Bidirectional cellular crosstalk was examined using direct and indirect co-culture models, and phenotypic responses were assessed via transcriptome analysis.

Results

PASMC and PAAF undergo distinct phenotypic shifts during pulmonary vascular remodeling, with limited shared features, s

SUBMITTER: Crnkovic S 

PROVIDER: S-EPMC11984959 | biostudies-literature | 2025 Apr

REPOSITORIES: biostudies-literature