The fifteen years of discoveries that shaped molecular electrophysiology: time for appraisal.
This article serves as an introductory overview to a thematic review series that will present the latest advancements in the field of inherited arrhythmias. This area of cardiac electrophysiology started approximately 15 years ago thanks to the contribution of Mark Keating and coworkers, who discovered the molecular basis of long QT syndrome. The field rapidly expanded when clinicians, molecular biologists, geneticists, and cellular electrophysiologists, who undertook an impressive collaborative effort to clarify the genetic basis of "cardiac channelopathies." As a result of this hard work, the paradigms for diagnosis and management of patients with inherited arrhythmogenic diseases were substantially modified, demonstrating once more the value of "translational research." As more and more genes have been implicated in the genesis of inherited arrhythmias, we keep broadening our understanding of the complexity of ion channels and their multifaceted regulatory processes. Despite the fact that several discoveries have already been made, the field is facing new challenges that are attracting young investigators who share with the pioneers the ambitious goal of finding new therapies and even a cure for these conditions.
Priori
Silvia G
SG
Division of Cardiology and Molecular Cardiology, Fondazione Salvatore Maugeri, Via S Maugeri 10/10 degrees, 27100 Pavia, Italy. silvia.priori@nyumc.org
D000818:Animals; D001145:Arrhythmias, Cardiac / Q000235:genetics / Q000503:physiopathology; D055724:Electrophysiological Phenomena / Q000235:genetics* / Q000502:physiology; D005820:Genetic Testing / Q000379:methods* / Q000639:trends*; D006801:Humans; D008133:Long QT Syndrome / Q000235:genetics* / Q000503:physiopathology*; D009154:Mutation / Q000235:genetics