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REVIEW

Mechanisms of Human Arrhythmia Syndromes: Abnormal Cardiac Macromolecular Interactions

Peter J. Mohler¹ and Xander H. T. Wehrens²

¹ Departments of Internal Medicine and Molecular Physiology and Biophysics, Division of Cardiology, University of Iowa Carver College of Medicine, Iowa City, Iowa; and
² Departments of Molecular Physiology and Biophysics and Medicine, Division of Cardiology, Baylor College of Medicine, Houston, Texas wehrens{at}bcm.edu

Many cardiac ion channels exist within macromolecular signaling complexes, comprised of pore-forming subunits that associate with auxiliary subunits, regulatory enzymes, and targeting proteins. This complex protein assembly ensures proper modulation of channel activity and ion homeostasis. The association of genetic defects in regulatory and targeting proteins to inherited arrhythmia syndromes has led to a better understanding of the critical role these proteins play in ion channel modulation.