title: Biology of cardiac arrhythmias: ion channel protein trafficking.
abstract: The mechanisms underlying normal and abnormal cardiac rhythms are complex and incompletely understood. Through the study of uncommon inheritable arrhythmia syndromes, including the long QT and Brugada syndromes, new insights are emerging. At the cellular and tissue levels, we now recognize that ion channel current is the sum of biophysical (gating, permeation), biochemical (phosphorylation, etc), and biogenic (biosynthesis, processing, trafficking, and degradation) properties. This review focuses on how heart cells process ion channel proteins and how this protein trafficking may be altered in some cardiac arrhythmia diseases. In this review, we honor Dr Harry A. Fozzard, a modern pioneer in cardiac arrhythmias, cell biology, and molecular electrophysiology. As a scientist and physician, his writings and mentorship have served to foster a generation of investigators who continue to bring this complex field toward greater scientific understanding and impact on humankind.
authors.affiliation: Section of Cardiovascular Medicine, Department of Medicine, University of Wisconsin-Madison, USA.
authors.forename: Brian P
authors.initials: BP
authors.lastname: Delisle
authors.forename: Blake D
authors.initials: BD
authors.lastname: Anson
authors.forename: Sridharan
authors.initials: S
authors.lastname: Rajamani
authors.forename: Craig T
authors.initials: CT
authors.lastname: January
mesh_terms: D001145:Arrhythmias, Cardiac / Q000235:genetics / Q000378:metabolism / Q000503:physiopathology*; D000072237:ERG1 Potassium Channel; D004721:Endoplasmic Reticulum / Q000502:physiology; D051638:Ether-A-Go-Go Potassium Channels; D030342:Genetic Diseases, Inborn / Q000235:genetics / Q000503:physiopathology; D020022:Genetic Predisposition to Disease; D006801:Humans; D015640:Ion Channel Gating / Q000502:physiology; D007473:Ion Channels / Q000737:chemistry / Q000235:genetics / Q000502:physiology*; D008133:Long QT Syndrome / Q000235:genetics / Q000503:physiopathology; D010766:Phosphorylation; D024642:Potassium Channels, Voltage-Gated / Q000737:chemistry / Q000235:genetics / Q000502:physiology; D011487:Protein Conformation; D017510:Protein Folding; D011499:Protein Processing, Post-Translational / Q000502:physiology; D021382:Protein Sorting Signals / Q000502:physiology; D021381:Protein Transport / Q000502:physiology*; D015398:Signal Transduction / Q000502:physiology
chemical_list: D000072237:ERG1 Potassium Channel; D051638:Ether-A-Go-Go Potassium Channels; D007473:Ion Channels; D024642:Potassium Channels, Voltage-Gated; D021382:Protein Sorting Signals