PKP2 orchestrates OXPHOS expression in cardiomyocytes via a PGC1α-dependent mechanism
Su Ji Han, Daphne van den Berg, Hesther de Ruiter, Hoyee Tsui, Eirini Kyriakopoulou, Tim Koopmans, Ilaria Perini, Jantine Monshouwer‐Kloots, Sebastiaan J. van Kampen, Anneline S.J.M. te Riele, Ilse R. Kelters, Monica Gianoli, B. M. T. Burgering, Eva van Rooij
This study identifies reduced oxidative phosphorylation (OXPHOS) in PKP2 mutant cardiomyocytes due to lower PPARGC1A expression, suggesting PPARGC1A modulation as a potential therapeutic target for arrhythmogenic cardiomyopathy.
Cardiovascular Research · Jul 6, 2026Peer-reviewedMolecular Biology