Gene Symbol: Prkag2
Description: protein kinase AMP-activated non-catalytic subunit gamma 2
Alias: 5'-AMP-activated protein kinase subunit gamma-2, AMP-activated protein kinase gamma2 subunit, AMPK gamma 2, adenosine monophosphate-activated protein kinase gamma 2-subunit, protein kinase, AMP-activated, gamma 2 non-catalytic subunit
Species: rat
Products:     Prkag2

Top Publications

  1. Reiter A, Bolster D, Crozier S, Kimball S, Jefferson L. Repression of protein synthesis and mTOR signaling in rat liver mediated by the AMPK activator aminoimidazole carboxamide ribonucleoside. Am J Physiol Endocrinol Metab. 2005;288:E980-8 pubmed
    ..Overall, however, the results from both experimental models support a scenario in which AICAR directly represses protein synthesis and mTOR signaling in the liver through an AMPK-dependent mechanism. ..
  2. Gollob M, Seger J, Gollob T, Tapscott T, Gonzales O, Bachinski L, et al. Novel PRKAG2 mutation responsible for the genetic syndrome of ventricular preexcitation and conduction system disease with childhood onset and absence of cardiac hypertrophy. Circulation. 2001;104:3030-3 pubmed
    We recently reported a mutation in the PRKAG2 gene to be responsible for a familial syndrome of ventricular preexcitation, atrial fibrillation, conduction defects, and cardiac hypertrophy...
  3. Hardie D, Scott J, Pan D, Hudson E. Management of cellular energy by the AMP-activated protein kinase system. FEBS Lett. 2003;546:113-20 pubmed
    ..A survey of the range of downstream targets for this important signalling pathway is presented. ..
  4. Sidhu J, Rajawat Y, Rami T, Gollob M, Wang Z, Yuan R, et al. Transgenic mouse model of ventricular preexcitation and atrioventricular reentrant tachycardia induced by an AMP-activated protein kinase loss-of-function mutation responsible for Wolff-Parkinson-White syndrome. Circulation. 2005;111:21-9 pubmed
    We identified a gene (PRKAG2) that encodes the gamma-2 regulatory subunit of AMP-activated protein kinase (AMPK) with a mutation (Arg302Gln) responsible for familial Wolff-Parkinson-White (WPW) syndrome...
  5. Ahmad F, Arad M, Musi N, He H, Wolf C, Branco D, et al. Increased alpha2 subunit-associated AMPK activity and PRKAG2 cardiomyopathy. Circulation. 2005;112:3140-8 pubmed
    AMP-activated protein kinase (AMPK) regulatory gamma2 subunit (PRKAG2) mutations cause a human cardiomyopathy with cardiac hypertrophy, preexcitation, and glycogen deposition...
  6. Laforet P, Richard P, Said M, Romero N, Lacene E, Leroy J, et al. A new mutation in PRKAG2 gene causing hypertrophic cardiomyopathy with conduction system disease and muscular glycogenosis. Neuromuscul Disord. 2006;16:178-82 pubmed
    Mutations in the gene encoding the gamma2 subunit of AMP-activated protein kinase (PRKAG2) cause familial cardiac hypertrophy and electrophysiological abnormalities, with glycogen accumulation in the heart of affected patients...
  7. Cheung P, Salt I, Davies S, Hardie D, Carling D. Characterization of AMP-activated protein kinase gamma-subunit isoforms and their role in AMP binding. Biochem J. 2000;346 Pt 3:659-69 pubmed
    ..Labelling studies, using the reactive AMP analogue 8-azido-[(32)P]AMP, indicate that the gamma subunit may participate directly in the binding of AMP within the complex. ..
  8. Arad M, Benson D, Perez Atayde A, McKenna W, Sparks E, Kanter R, et al. Constitutively active AMP kinase mutations cause glycogen storage disease mimicking hypertrophic cardiomyopathy. J Clin Invest. 2002;109:357-62 pubmed
    Mutations in PRKAG2, the gene for the gamma 2 regulatory subunit of AMP-activated protein kinase, cause cardiac hypertrophy and electrophysiologic abnormalities, particularly preexcitation (Wolff-Parkinson-White syndrome) and ..
  9. Hardie D. The AMP-activated protein kinase pathway--new players upstream and downstream. J Cell Sci. 2004;117:5479-87 pubmed

More Information


  1. Burwinkel B, Scott J, Buhrer C, van Landeghem F, Cox G, Wilson C, et al. Fatal congenital heart glycogenosis caused by a recurrent activating R531Q mutation in the gamma 2-subunit of AMP-activated protein kinase (PRKAG2), not by phosphorylase kinase deficiency. Am J Hum Genet. 2005;76:1034-49 pubmed
    ..However, in three of five patients, we identified identical heterozygous R531Q missense mutations of the PRKAG2 gene, which encodes the gamma 2-subunit of AMP-activated protein kinase, a key regulator of energy balance...
  2. Li J, Coven D, Miller E, Hu X, Young M, Carling D, et al. Activation of AMPK alpha- and gamma-isoform complexes in the intact ischemic rat heart. Am J Physiol Heart Circ Physiol. 2006;291:H1927-34 pubmed
    ..Despite the pathophysiological importance of gamma(2)-isoform mutations, gamma(1)-isoform complexes account for most of the AMPK activity in the ischemic heart. ..
  3. Scott J, Ross F, Liu J, Hardie D. Regulation of AMP-activated protein kinase by a pseudosubstrate sequence on the gamma subunit. EMBO J. 2007;26:806-15 pubmed
    ..Binding of AMP causes a conformational change that prevents this interaction and relieves the inhibition. We present several lines of evidence supporting this hypothesis. ..