Integrative Analysis of PRKAG2 Cardiomyopathy iPS and Microtissue Models Identifies AMPK as a Regulator of Metabolism, Survival, and Fibrosis

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• 作者：J. Travis Hinson¹ ; ² ; ¹³ ; ^{travis.hinson@jax.org} ; Anant Chopra³ ; ⁴ ; Andre Lowe¹ ; Calvin C. Sheng⁵ ; Rajat M. Gupta⁶ ; Rajarajan Kuppusamy⁷ ; John O'Sullivan⁸ ; Glenn Rowe⁹ ; Hiroko Wakimoto⁵ ; Joshua Gorham⁵ ; Kehan Zhang³ ; ⁴ ; Kiran Musunuru¹⁰ ; Robert E. Gerszten⁸ ; ¹¹ ; Sean M. Wu⁷ ; Christopher S. Chen³ ; ⁴ ; Jonathan G. Seidman⁵ ; Christine E. Seidman⁵ ; ⁶ ; ¹² ; ^{cseidman@genetics.med.harvard.edu}
• 关键词：AMP-activated protein kinase ; AMPK ; PRKAG2 ; hypertrophic cardiomyopathy ; TGF-beta ; fibrosis ; glycogen storage disease ; Wolff-Parkinson-White syndrome
• 刊名：Cell Reports
• 出版年：2016
• 出版时间：20 December 2016
• 年：2016
• 卷：17
• 期：12
• 页码：3292-3304
• 全文大小：4058 K
• 卷排序：17

文摘

PRKAG2 cardiomyopathy mutations activate AMPK in human iPS models AMPK transcriptionally regulates glucose handling and mitochondrial biogenesis AMPK enhances cardiac microtissue forces by increased myocyte survival AMPK inhibits TGF-beta 2 production and fibrosis in vivo