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Desmoplakin maintains gap junctions by inhibiting Ras/MAPK and lysosomal degradation of connexin-43.桥粒斑蛋白通过抑制 Ras/MAPK 和连接蛋白 43 的溶酶体降解来维持缝隙连接。
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本文引用的文献

1
Altered desmosomal proteins in granulomatous myocarditis and potential pathogenic links to arrhythmogenic right ventricular cardiomyopathy.肉芽肿性心肌炎中桥粒蛋白的改变及与致心律失常性右室心肌病的潜在致病联系。
Circ Arrhythm Electrophysiol. 2011 Oct;4(5):743-52. doi: 10.1161/CIRCEP.111.964890. Epub 2011 Aug 22.
2
Mutation analysis and evaluation of the cardiac localization of TMEM43 in arrhythmogenic right ventricular cardiomyopathy.TMEM43 基因突变分析及其在致心律失常性右室心肌病中心脏定位的评估。
Clin Genet. 2011 Sep;80(3):256-64. doi: 10.1111/j.1399-0004.2011.01623.x. Epub 2011 Jan 24.
3
The pathobiology of arrhythmogenic cardiomyopathy.心律失常性心肌病的病理生物学。
Annu Rev Pathol. 2011;6:299-321. doi: 10.1146/annurev-pathol-011110-130151.
4
Arrhythmogenic right ventricular cardiomyopathy.致心律失常性右室心肌病
Lancet. 2009 Apr 11;373(9671):1289-300. doi: 10.1016/S0140-6736(09)60256-7.
5
A new diagnostic test for arrhythmogenic right ventricular cardiomyopathy.一种用于诊断致心律失常性右室心肌病的新型检测方法。
N Engl J Med. 2009 Mar 12;360(11):1075-84. doi: 10.1056/NEJMoa0808138.
6
Characterization of the molecular phenotype of two arrhythmogenic right ventricular cardiomyopathy (ARVC)-related plakophilin-2 (PKP2) mutations.两种致心律失常性右室心肌病(ARVC)相关的桥粒斑菲素蛋白-2(PKP2)突变的分子表型特征
Heart Rhythm. 2008 Dec;5(12):1715-23. doi: 10.1016/j.hrthm.2008.09.009. Epub 2008 Sep 6.
7
N-cadherin haploinsufficiency affects cardiac gap junctions and arrhythmic susceptibility.N-钙黏蛋白单倍体不足影响心脏缝隙连接和心律失常易感性。
J Mol Cell Cardiol. 2008 Mar;44(3):597-606. doi: 10.1016/j.yjmcc.2007.11.013. Epub 2007 Dec 7.
8
Molecular composition of the intercalated disc in a spontaneous canine animal model of arrhythmogenic right ventricular dysplasia/cardiomyopathy.致心律失常性右室发育不良/心肌病自发性犬类动物模型中介入盘的分子组成
Heart Rhythm. 2007 Sep;4(9):1196-205. doi: 10.1016/j.hrthm.2007.05.025. Epub 2007 Jun 8.
9
Connexin43 remodeling caused by inhibition of plakophilin-2 expression in cardiac cells.心肌细胞中由于抑制桥粒斑蛋白-2表达导致的连接蛋白43重塑。
Circ Res. 2007 Sep 28;101(7):703-11. doi: 10.1161/CIRCRESAHA.107.154252. Epub 2007 Aug 2.
10
Suppression of canonical Wnt/beta-catenin signaling by nuclear plakoglobin recapitulates phenotype of arrhythmogenic right ventricular cardiomyopathy.核内桥粒斑珠蛋白对经典Wnt/β-连环蛋白信号通路的抑制重现了致心律失常性右室心肌病的表型。
J Clin Invest. 2006 Jul;116(7):2012-21. doi: 10.1172/JCI27751.

Gap junctions and arrhythmogenic cardiomyopathy.

作者信息

Asimaki Angeliki, Saffitz Jeffrey E

机构信息

Department of Pathology, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, MA 02215, USA.

出版信息

Heart Rhythm. 2012 Jun;9(6):992-5. doi: 10.1016/j.hrthm.2011.11.024. Epub 2011 Nov 16.

DOI:10.1016/j.hrthm.2011.11.024
PMID:22100709
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3358518/
Abstract
摘要