Frataxin deficiency drives cardiac dysfunction and transcriptional dysregulation in Friedreich ataxia iPSC model
Jarmon G. Lees, Haoxiang Zhang, Lebei Jiao, Anne M. Kong, Ren Jie Phang, Li Li, Nan Su, Sebastian Bass-Stringer, Hei-Yi H. Woo, Anthony S Mukhtar, Alice Pébay, Mirella Dottori, Louise A. Corben, Martin Bruce Delatycki, Roger E. Peverill, , Shiang Yong Lim
This study uses iPSCs from FRDA patients to model cardiac dysfunction, finding transcriptional dysregulation and phenotypes like increased cell size and mitochondrial stress.
Cell Death and Disease · Jun 23, 2026Peer-reviewedPathology