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.
Towbin
Jeffrey A
JA
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
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)