Desmoplakin mutations in cardiac fibroblasts cause TGF?1-mediated pathological fibrogenesis in desmoplakin cardiomyopathy via beclin-1 regulation
Chuanyu Wei, Shing Fai Chan, Ardan Muammer Saguner, Corinna Brunckhorst, Fırat Duru, Joseph Edward Marine, Cynthia A. James, Hugh G Calkins, Daniel Philip Judge, Weinian Shou, Huei-Sheng Vincent Chen
This study shows that DSP mutations in cardiac fibroblasts enhance TGFβ1-induced fibrosis by disrupting beclin-1 regulation and autophagy, leading to excessive collagen accumulation and fibrotic gene activation.
Circulation Research · Aug 19, 2026Peer-reviewedCell Biology