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肌节Z盘在心肌病发病机制中的作用。

Role of the sarcomeric Z-disc in the pathogenesis of cardiomyopathy.

作者信息

Frank Derk, Kuhn Christian, Katus Hugo A, Frey Norbert

机构信息

University of Heidelberg, Department of Internal Medicine III, Germany.

出版信息

Future Cardiol. 2007 Nov;3(6):611-22. doi: 10.2217/14796678.3.6.611.

Abstract

The Z-disc has traditionally been viewed as a structure required to maintain sarcomeric function and integrity. More recently, the sarcomeric Z-disc has also emerged as a nodal point in cardiomyocyte signaling and mechanotransduction. This notion is not only supported by several transgenic animal models, but also by the identification of mutations in various Z-disc proteins, resulting in dilated or hypertrophic cardiomyopathy in patients. This review will thus focus on the role of the sarcomeric Z-disc and its associated proteins in the pathogenesis of cardiomyopathy.

摘要

传统上,Z线被视为维持肌节功能和完整性所必需的结构。最近,肌节Z线也已成为心肌细胞信号传导和机械转导的一个节点。这一观点不仅得到了多种转基因动物模型的支持,还得到了各种Z线蛋白突变的鉴定结果的支持,这些突变会导致患者出现扩张型或肥厚型心肌病。因此,本综述将聚焦于肌节Z线及其相关蛋白在心肌病发病机制中的作用。

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