Frank Derk, Kuhn Christian, Katus Hugo A, Frey Norbert
University of Heidelberg, Department of Internal Medicine III, Germany.
Future Cardiol. 2007 Nov;3(6):611-22. doi: 10.2217/14796678.3.6.611.
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.
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。