Kostetskii Igor, Li Jifen, Xiong Yanming, Zhou Rong, Ferrari Victor A, Patel Vickas V, Molkentin Jeffery D, Radice Glenn L
Center for Research on Reproduction and Women's Health, University of Pennsylvania School of Medicine, Philadelphia, Pa 19104, USA.
Circ Res. 2005 Feb 18;96(3):346-54. doi: 10.1161/01.RES.0000156274.72390.2c. Epub 2005 Jan 20.
The structural integrity of the heart is maintained by the end-to-end connection between the myocytes called the intercalated disc. The intercalated disc contains different junctional complexes that enable the myocardium to function as a syncytium. One of the junctional complexes, the zonula adherens or adherens junction, consists of the cell adhesion molecule, N-cadherin, which mediates strong homophilic cell-cell adhesion via linkage to the actin cytoskeleton. To determine the function of N-cadherin in the working myocardium, we generated a conditional knockout containing loxP sites flanking exon 1 of the N-cadherin (Cdh2) gene. Using a cardiac-specific tamoxifen-inducible Cre transgene, N-cadherin was deleted in the adult myocardium. Loss of N-cadherin resulted in disassembly of the intercalated disc structure, including adherens junctions and desmosomes. The mutant mice exhibited modest dilated cardiomyopathy and impaired cardiac function, with most animals dying within two months after tamoxifen administration. Decreased sarcomere length and increased Z-line thickness were observed in the mutant hearts consistent with loss of muscle tension because N-cadherin was no longer available to anchor myofibrils at the plasma membrane. Ambulatory electrocardiogram monitoring captured the abrupt onset of spontaneous ventricular tachycardia, confirming that the deaths were arrhythmic in nature. A significant decrease in the gap junction protein, connexin 43, was observed in the N-cadherin-depleted hearts. This animal model provides the first demonstration of the hierarchical relationship of the structural components of the intercalated disc in the working myocardium, thus establishing N-cadherin's paramount importance in maintaining the structural integrity of the heart.
心肌细胞之间的端对端连接即闰盘维持着心脏的结构完整性。闰盘包含不同的连接复合体,使心肌能够作为一个合胞体发挥作用。其中一种连接复合体,即黏着小带或黏着连接,由细胞黏附分子N-钙黏蛋白组成,它通过与肌动蛋白细胞骨架相连介导强烈的同型细胞间黏附。为了确定N-钙黏蛋白在工作心肌中的功能,我们构建了一个条件性敲除小鼠,其N-钙黏蛋白(Cdh2)基因的外显子1两侧有loxP位点。利用心脏特异性他莫昔芬诱导型Cre转基因,在成年心肌中删除了N-钙黏蛋白。N-钙黏蛋白的缺失导致闰盘结构解体,包括黏着连接和桥粒。突变小鼠表现出中度扩张型心肌病和心脏功能受损,大多数动物在给予他莫昔芬后两个月内死亡。在突变心脏中观察到肌节长度缩短和Z线厚度增加,这与肌肉张力丧失一致,因为N-钙黏蛋白不再可用于将肌原纤维锚定在质膜上。动态心电图监测捕捉到自发性室性心动过速的突然发作,证实死亡本质上是心律失常性的。在N-钙黏蛋白缺失的心脏中观察到缝隙连接蛋白连接蛋白43显著减少。这个动物模型首次证明了工作心肌中闰盘结构成分的层次关系,从而确立了N-钙黏蛋白在维持心脏结构完整性方面的至关重要性。