Transgenic Mice Overexpressing Mutant <i>PRKAG2</i> Define the Cause of Wolff-Parkinson-White Syndrome in Glycogen Storage Cardiomyopathy

Abstract

Our data establish PRKAG2 mutations as a glycogen storage cardiomyopathy, provide an anatomic explanation for electrophysiological findings, and implicate disruption of the annulus fibrosis by glycogen-engorged myocytes as the cause of preexcitation in Pompe, Danon, and other glycogen storage diseases.