MiR-129-1-3p down-regulation promotes BAG cochaperone 3 (BAG3)-driven pro-fibrotic processes in primary fibroblasts from patients with recessive dystrophic epidermolysis bullosa.

ABSTRACT: Recessive dystrophic epidermolysis bullosa (RDEB) is a rare and severe skin fragility disease due to loss-of-function mutations in the COL7A1 gene. RDEB patients suffer from relentless mucocutaneous blisters that evolve into chronic wounds with inflammation and progressive fibrosis, which heavily impacts the disease course. RDEB-associated fibrosis is the result of a complex dialogue among different cell types, signalling pathways and epigenetic players that are still poorly characterized. The expression levels of over 750 mature microRNAs were investigated in primary fibroblasts from patients with RDEB (RDEB-FBs) and healthy donors using TaqMan Low-Density Arrays. Among deregulated molecules, only the miR-129-1-3p was down-regulated in RDEB-FBs. Therefore, the role of miR-129-1-3p

SUBMITTER: Nobili R 

PROVIDER: S-EPMC12441624 | biostudies-literature | 2025 Dec

REPOSITORIES: biostudies-literature