This study was supported by funding from the Alberta Heritage Foundation for Medical Research (AHFMR), the Canadian Institutes of Health Research (CIHR), and the Heart and Stroke Foundation of Canada. Dr. Light, Ph.D., received salary support as an AHFMR Scholar and CIHR New Investigator.
Familial Wolff-Parkinson-White Syndrome: A Disease of Glycogen Storage or Ion Channel Dysfunction?
Article first published online: 21 APR 2006
Journal of Cardiovascular Electrophysiology
Volume 17, Issue Supplement s1, pages S158–S161, May 2006
How to Cite
LIGHT, P. E. (2006), Familial Wolff-Parkinson-White Syndrome: A Disease of Glycogen Storage or Ion Channel Dysfunction?. Journal of Cardiovascular Electrophysiology, 17: S158–S161. doi: 10.1111/j.1540-8167.2006.00399.x
- Issue published online: 21 APR 2006
- Article first published online: 21 APR 2006
- familiar WPW;
- glycogen storage;
- ion channels;
- ventricular pre-excitation;
Wolff-Parkinson-White (WPW) syndrome is the most common cause of ventricular pre-excitation, a condition where, due to defects in the conduction pathway, all or part of the ventricle is excited earlier than would normally be expected, often leading to ventricular fibrillation and sudden cardiac death. It was recently discovered that many of the underlying mutations responsible for the familial form of WPW syndrome are located in the gene encoding for the regulatory γ2-subunit (PRKAG2) of the AMP-activated protein kinase. The cellular mechanisms for the observed arrhythmias are currently being studied and may involve glycogen storage with associated hypertrophy as well as alterations in the properties of cardiac ion channels such as voltage-gated sodium channel. It is the aim of this review to discuss our current knowledge of the cellular disturbances underlying the induction of arrhythmias in patients with PRKAG2 mutations.