Molecular genetic basis of sudden cardiac death.
This article outlines the up-to-date understanding of the molecular basis of disorders that cause sudden death. Several arrhythmic disorders that cause sudden death have been well-described at the molecular level, including the long QT syndromes and Brugada syndrome; this article reviews the current scientific knowledge of these diseases. Hypertrophic cardiomyopathy, a myocardial disorder that causes sudden death also has been well-studied. Finally, a disorder in which myocardial abnormalities and rhythm abnormalities coexist, arrhythmogenic right ventricular dysplasia, is described.
Department of Pediatrics (Cardiology), Texas Children's Hospital and Baylor College of Medicine, 6621 Fannin Street, FC. 430.09, Houston, TX 77030, USA. jtowbin@bcm.tmc.edu
Jeffrey A
JA
Towbin
D001145:Arrhythmias, Cardiac / Q000235:genetics / Q000503:physiopathology; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000235:genetics / Q000503:physiopathology; D024741:Cardiomyopathy, Hypertrophic, Familial / Q000235:genetics / Q000503:physiopathology; D002648:Child; D016757:Death, Sudden, Cardiac / Q000209:etiology*; D000072237:ERG1 Potassium Channel; D051638:Ether-A-Go-Go Potassium Channels; D006801:Humans; D051656:KCNQ Potassium Channels; D051657:KCNQ1 Potassium Channel; D008133:Long QT Syndrome / Q000150:complications* / Q000235:genetics / Q000628:therapy; D062554:NAV1.5 Voltage-Gated Sodium Channel; D015221:Potassium Channels / Q000502:physiology; D024642:Potassium Channels, Voltage-Gated; D015222:Sodium Channels / Q000502:physiology; D013577:Syndrome; D017180:Tachycardia, Ventricular / Q000235:genetics; D014927:Wolff-Parkinson-White Syndrome / Q000503:physiopathology
D000072237:ERG1 Potassium Channel; D051638:Ether-A-Go-Go Potassium Channels; D051656:KCNQ Potassium Channels; D051657:KCNQ1 Potassium Channel; C496909:KCNQ1 protein, human; D062554:NAV1.5 Voltage-Gated Sodium Channel; D015221:Potassium Channels; D024642:Potassium Channels, Voltage-Gated; C568320:SCN5A protein, human; D015222:Sodium Channels; C059983:potassium channel protein I(sk)