Our human iPSC model captures early, clinically relevant features of FRDA cardiomyopathy and identifies PCDHGA10 as a disease-associated target within the γ-protocadherin family of calcium-dependent adhesion molecules. siRNA-mediated PCDHGA10 knockdown rescued cell survival, diastolic dysfunction, and mitochondrial ROS levels, implicating Ca2+-coupled and redox-linked phenotypes in cardiomyocyte dysfunction. These findings support further mechanistic study and therapeutic exploration of PCDHGA10
Tuesday, August 4, 2026
Cardiomyocyte Dysfunction is Modulated by PCDHGA10 in Friedreich Ataxia,
J. Lees, H. Zhang, L. Jiao, A. Kong, R. Phang, L. Li, N. Su, A. Mukhtar, S. Bass-Stringer, A. Pébay, M. Dottori, L. Corben, M. Delatycki, R. Peverill, S. Wilcox, J. Choi, J. Pullin, D. McCarthy, J. Napierala, M. Napierala, S. Lim, Cardiomyocyte Dysfunction is Modulated by PCDHGA10 in Friedreich Ataxia, Heart, Lung and Circulation, Volume 35, Supplement 3, 2026, Pages S709-S710, doi:10.1016/j.hlc.2026.07.1148.
