Meadows L S, Isom L L
Department of Pharmacology, The University of Michigan, 1301 MSRB III, Box 0632, 1150 W. Medical Center Dr. Ann Arbor, MI 48109-0632, United States.
Cardiovasc Res. 2005 Aug 15;67(3):448-58. doi: 10.1016/j.cardiores.2005.04.003.
Mutations in cardiac ion channels and their auxiliary subunits can lead to life-threatening cardiac arrhythmias. In recent years it has become apparent that ion channels are part of large, multi-protein complexes, comprising not only the ion channels and their auxiliary subunits, but also components of the cytoskeleton, regulatory kinases and phosphatases, trafficking proteins, extracellular matrix proteins, and possibly even other ion channels. Disruption of any member of a particular ion channel complex has the potential to disrupt the function of the associated channels, resulting in paroxysmal disease. Understanding the molecular composition of individual ion channel signaling complexes in heart may yield important insights into the molecular basis of cardiac arrhythmias and may suggest novel therapeutic approaches to treatment of these life-threatening conditions.
心脏离子通道及其辅助亚基的突变可导致危及生命的心律失常。近年来，越来越明显的是，离子通道是大型多蛋白复合物的一部分，不仅包括离子通道及其辅助亚基，还包括细胞骨架成分、调节激酶和磷酸酶、转运蛋白、细胞外基质蛋白，甚至可能还有其他离子通道。特定离子通道复合物中任何一个成员的破坏都有可能扰乱相关通道的功能，导致阵发性疾病。了解心脏中单个离子通道信号复合物的分子组成，可能会为心律失常的分子基础提供重要见解，并可能为治疗这些危及生命的疾病提出新的治疗方法。
