These authors should be regarded as co-first authors.
Overexpression of G100S mutation in PRKAG2 causes Wolff–Parkinson–White syndrome in zebrafish
Article first published online: 25 OCT 2013
© 2013 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd
How to Cite
Overexpression of G100S mutation in PRKAG2 causes Wolff–Parkinson–White syndrome in zebrafish., , , , , , , , , .
Nothing to declare.
- Article first published online: 25 OCT 2013
- Accepted manuscript online: 30 AUG 2013 10:40AM EST
- Manuscript Revised: 28 AUG 2013
- Manuscript Accepted: 28 AUG 2013
- Manuscript Received: 16 APR 2013
- National Natural Science Foundation. Grant Number: 81000038
- PRKAG2 cardiac;
The Wolff–Parkinson–White (WPW) syndrome was believed to be associated with PRKAG2 gene mutations. In this study, we verified the pathopoiesis of G100S mutation, a novel mutation only discovered in Chinese patients with WPW, in cardiac disorder. Similar to R302Q, when overexpressed PRKAG2 G100S mutant in zebrafish, we observed a thicker heart wall, detected a decreased AMPK enzymatic activity by tissue AMPK kinase activity colorimetric technique, as well as examined an increased glycogen storage in heart wall using the method for periodic acid-Schiff staining, in comparison with the zebrafish without exogenous PRKAG2 (mock) or with wild-type PRKAG2 (WT). Taken together, we concluded PRKAG2 G100S mutation might contribute to impair the AMP-activated protein kinase function, which resulted in increased cardiac glycogen storage, serving as a pathogenesis for WPW syndrome in Chinese.