Mice with cardiac-restricted overexpression of Myozap are sensitized to biomechanical stress and develop a protein-aggregate-associated cardiomyopathy
详细信息 查看全文
- 作者：Derk Franka ; d ; 1 ; derk.frank@uksh.de" class="auth_mail ; Ashraf Y. Rangreza ; d ; 1 ; Reza Poyanmehra ; Thalia S. Seegerb ; Christian Kuhna ; d ; Matthias Edena ; Katharina Stiebelinga ; Alexander Bernta ; d ; Christine Grundc ; Werner W. Frankec ; Norbert Freya ; d ; norbert.frey@uksh.de" class="auth_mail
- 关键词：Intercalated disc ; Cardiomyopathy ; Transgenic mouse model ; SRF ; Protein aggregates
- 刊名：Journal of Molecular and Cellular Cardiology
- 出版年：July 2014
- 年：2014
- 卷：72
- 期：Complete
- 页码：196-207
- 全文大小：2317 K
文摘
- •
Newly generated Myozap-Tg mice developed cardiomyopathy with hypertrophy and LV dilation.
- •
Pressure overload caused exaggerated hypertrophy, further loss of contractility and LV dilation.
- •
Tg hearts showed protein aggregates composed of Myozap, desmoplakin and other ID proteins.
- •
The aggregates closely resembled the alterations observed in desmin-related cardiomyopathy.
- •
Tg mice revealed induction of autophagy and dysregulation of the UPR, associated with apoptosis.