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TMEM43-S358L mutation enhances NF-κBTGFp signal cascade in arrhythmogenic right ventricular dysplasia/cardiomyopathy

查看全文 作　　者：Guoxing [1,2,3]Zheng;Changying [4]Jiang;Yulin [5]Li;Dandan [2]Yang;Youcai [5]Ma;Bing [2]Zhang;Xuan [2]Li;Pei [2]Zhang;Xiaoyu [2]Hu;Xueqiang [2]Zhao;Jie [5]Du;Xin [1,2]Lin 高影响力作者 机构地区：[1]Tsinghua University-Peking University Joint Center for Life Sciences,Beijing 100084,China;[2]Institute for Immunology,Department of Basic Medical Sciences,School of Medicine,Tsinghua University,Beijing 100084,China;[3]The 7th Affiliated Hospital of Sun Yat-Sen University,Shenzhen,Guangdong 510275,China;[4]Department of Molecular and Cellular Oncology,The University of Texas,MD Anderson Cancer Center,Houston,TX 77030,USA;[5]Beijing Anzhen Hospital,Capital Medical University,The Key Laboratory of Remodeling-Related Cardiovascular Diseases,Ministry of Education,Beijing Collaborative Innovation Center for Cardiovascular Disorders,Beijing Institute of Heart,Lung&Blood Vessel Disease,Beijing 100029,China高影响力机构 出　　处：《Protein & Cell》索引2019年第10卷第2期,共16页高影响力期刊 基　　金：grants from National Natural Science Foundation of China(81570211 to X.Lin);China Postdoctoral Science Foundation(023221010 to G.Zheng). 摘　　要：Arrhythmogenic right ventricular dysplasia/cardiomyopathy(ARVD/C)is a genetic cardiac muscle disease that accounts for approximately 30%sudden cardiac death in young adults.The Ser358Leu mutation of transmembrane protein 43(TMEM43)was commonly identified in the patients of highly lethal and fully penetrant ARVD subtype,ARVD5.Here,we generated TMEM43 S358L mouse to explore the underlying mechanism.This mouse strain showed the classic patholo.gies of ARVD patients,including structural abnormalities and cardiac fibrofatty.TMEM43 S358L mutation led to hyper-activated nuclear factor kB(NFkB)activation in heart tissues and primary cardiomy.ocyte cells.Importantly,this hyper activation of NF-κB directly drove the expression of pro-fibrotic gene,transforming growth factor beta(TGFβ),and enhanced downstream signal,indicating that TMEM43 S358L mutation up-regulates NF-κB-TGFβ signal cascade during ARVD cardiac fibrosis.Our study partially reveals the regulatory mechanism of ARVD development. 关 键 词：TMEM43 ARVD NF-ΚB TGFΒ FIBROSIS KNOCK-IN mouse
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