Distinct Early Signaling Events Resulting From the Expression of the PRKAG2 R302Q Mutant of AMPK Contribute to Increased Myocardial Glycogen | Zendy

Karalyn D. Folmes | Zendy; Anita Y.M. Chan | Zendy; Debby P.Y. Koonen | Zendy; Thomas Pulinilkunnil | Zendy; István Baczkó | Zendy; Beth Hunter | Zendy; Stephanie Thorn | Zendy; Michael F. Allard | Zendy; Robert Roberts | Zendy; Michael H. Gollob | Zendy; Peter E. Light | Zendy; Jason R.B. Dyck | Zendy

Open Access

Distinct Early Signaling Events Resulting From the Expression of the PRKAG2 R302Q Mutant of AMPK Contribute to Increased Myocardial Glycogen

Author(s) -

Karalyn D. Folmes,

Anita Y.M. Chan,

Debby P.Y. Koonen,

Thomas Pulinilkunnil,

István Baczkó,

Beth Hunter,

Stephanie Thorn,

Michael F. Allard,

Robert Roberts,

Michael H. Gollob,

Peter E. Light,

Jason R.B. Dyck

Publication year - 2009

Publication title -

circulation cardiovascular genetics

Language(s) - English

Resource type - Journals

eISSN - 1942-325X

pISSN - 1942-3268

DOI - 10.1161/circgenetics.108.834564

Subject(s) - glycogen , glycogen synthase , transgene , ampk , endocrinology , biology , genetically modified mouse , medicine , downregulation and upregulation , gsk 3 , gene knockdown , glycogen debranching enzyme , signal transduction , phenotype , protein kinase a , microbiology and biotechnology , phosphorylation , gene , biochemistry

Humans with an R302Q mutation in AMPKgamma(2) (the PRKAG2 gene) develop a glycogen storage cardiomyopathy characterized by a familial form of Wolff-Parkinson-White syndrome and cardiac hypertrophy. This phenotype is recapitulated in transgenic mice with cardiomyocyte-restricted expression of AMPKgamma(2)R302Q. Although considerable information is known regarding the consequences of harboring the gamma(2)R302Q mutation, little is known about the early signaling events that contribute to the development of this cardiomyopathy.

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