Article
WNT5a-Mediated Aberrant Actin Filament Dynamics Drive Cardiac Pathogenic Phenotypes in LMNA-Related Emery-Dreifuss Muscular Dystrophy.
Circulation - 2 Jun 2026
Fan Hangping, Wang Xiaochen, Liu Xujie, Zhao Jiuxiao, Zhang Yuan, Yang Zongkuai, Wang Hao, Gong Junhao, Li Lingying, Jin Jiamin, Guo Yuxuan, Gong Tingyu, Zhuang Lenan, Ke Qing, Liang Ping
Abstract excerpt
BACKGROUND: Emery-Dreifuss muscular dystrophy (EDMD) is a rare genetic disorder characterized by early-onset joint contractures, progressive muscle atrophy, and cardiac abnormalities. Patients with EDMD carrying LMNA sequence variations often exhibit severe cardiac manifestations, including frequent atrioventricular block and ventricular tachycardia. Approximately 20% of those patients may ultimately require...
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