WNT5a-Mediated Aberrant Actin Filament Dynamics Drive Cardiac Pathogenic Phenotypes in <i>LMNA</i> -Related Emery-Dreifuss Muscular Dystrophy
BACKGROUND: sequence variations lead to EDMD remain unknown. METHODS: L204P sequence variation was also generated. RESULTS: L204P heterozygous knock-in mice exhibited impaired cardiac function and developed cardiac arrhythmias in response to sympathetic stress. CONCLUSIONS: -related EDMD. Our findings indicate that activating WNT5a/RhoA and stabilizing actin assembly may serve as novel therapeutic strategies for this condition.
Authors
- Hangping Fan
- Lenan Zhuang (ORCID: https://orcid.org/0000-0001-9561-8396)
- Tingyu Gong
- Jiamin Jin (ORCID: https://orcid.org/0000-0003-2725-840X)
- Zongkuai Yang
- X W Liu
- Hao Wang (ORCID: https://orcid.org/0000-0001-7594-7387)
- Yuxuan Guo (ORCID: https://orcid.org/0000-0002-6009-4403)
- Jiuxiao Zhao
- Lingying Li
- Ping Liang (ORCID: https://orcid.org/0000-0001-6806-3735)
- Qing Ke
- Junhao Gong
- Xiaochen Wang
- Yuan Zhang (ORCID: https://orcid.org/0000-0003-4780-6039)
Institutions
- Zhejiang International Studies University (CN)
- Chinese Academy of Medical Sciences & Peking Union Medical College (CN)
- Peking University (CN)
- Sir Run Run Shaw Hospital (CN)
- Zhejiang Shuren University (CN)
- Communication University of Zhejiang (CN)
- Fu Wai Hospital (CN)
- Ministry of Public Health (MG)
- First Affiliated Hospital Zhejiang University (CN)
Publication Details
- Journal
- Circulation
- Published
- 2026-04-17
- DOI
- https://doi.org/10.1161/circulationaha.125.075604
- Primary Topic
- Nuclear Structure and Function
- Type
- article
- Field-Weighted Citation Impact
- 0.00