Increased &agr;2 Subunit–Associated AMPK Activity and PRKAG2 Cardiomyopathy

@article{Ahmad2005IncreasedS,
  title={Increased \&agr;2 Subunit–Associated AMPK Activity and PRKAG2 Cardiomyopathy},
  author={Ferhaan Ahmad and Michael Arad and Nicolas Musi and Huamei He and Cordula M. Wolf and Dorothy M. Branco and Antonio R. Perez-Atayde and David I. Stapleton and Deeksha Bali and Yanqiu Xing and Rong Tian and Laurie J. Goodyear and Charles I. Berul and Joanne S. Ingwall and Christine E. Seidman and Jonathan G. Seidman},
  journal={Circulation},
  year={2005},
  volume={112},
  pages={3140-3148}
}
Background— AMP-activated protein kinase (AMPK) regulatory &ggr;2 subunit (PRKAG2) mutations cause a human cardiomyopathy with cardiac hypertrophy, preexcitation, and glycogen deposition. PRKAG2 cardiomyopathy is recapitulated in transgenic mice overexpressing mutant PRKAG2 N488I in the heart (TG&ggr;2N488I). AMPK is a heterotrimeric kinase consisting of 1 catalytic (&agr;) and 2 regulatory (&bgr; and &ggr;) subunits. Two &agr;-subunit isoforms, &agr;1 and &agr;2, are expressed in the heart… 

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