Protein Kinase, AMP Activated Gamma 2 (PRKAg2)

AAKG; AAKG2; CMH6; H91620p; WPWS; 5'-AMP-activated protein kinase subunit gamma-2

SPN	T425
HGNC	9386
MGI	1336153
PubMed	12015471
RGD	727782
UniProt	Q9UGJ0
Wiki	PRKAG2

AMP-activated protein kinase (AMPK) is a heterotrimeric protein composed of a catalytic alpha subunit, a noncatalytic beta subunit, and a noncatalytic regulatory gamma subunit. Various forms of each of these subunits exist, encoded by different genes. AMPK is an important energy-sensing enzyme that monitors cellular energy status and functions by inactivating key enzymes involved in regulating de novo biosynthesis of fatty acid and cholesterol. PRKAG2 is a member of the AMPK gamma subunit family and encodes a protein with four cystathionine beta-synthase domains. Mutations in this gene have been associated with ventricular pre-excitation (Wolff-Parkinson-White syndrome), progressive conduction system disease and cardiac hypertrophy. Alternate transcriptional splice variants, encoding different isoforms, have been characterized.

Reference

"Characterization of AMP-activated protein kinase gamma-subunit isoforms and their role in AMP binding."
Biochem. J. 346:659-669(2000) [PubMed] [Europe PMC] [Abstract]

"Molecular cloning, genomic organization, and mapping of PRKAG2, a heart abundant gamma-2 subunit of 5'-AMP-activated protein kinase, to human chromosome 7q36."
Genomics 70:258-263(2000) [PubMed] [Europe PMC] [Abstract]

"Complete sequencing and characterization of 21,243 full-length human cDNAs."
Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]

"The DNA sequence of human chromosome 7."
Nature 424:157-164(2003) [PubMed] [Europe PMC] [Abstract]

"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]

"CBS domains form energy-sensing modules whose binding of adenosine ligands is disrupted by disease mutations."
J. Clin. Invest. 113:274-284(2004) [PubMed] [Europe PMC] [Abstract]

"Regulation of AMP-activated protein kinase by a pseudosubstrate sequence on the gamma subunit."
EMBO J. 26:806-815(2007) [PubMed] [Europe PMC] [Abstract]

"Kinase-selective enrichment enables quantitative phosphoproteomics of the kinome across the cell cycle."
Mol. Cell 31:438-448(2008) [PubMed] [Europe PMC] [Abstract]

"Lys-N and trypsin cover complementary parts of the phosphoproteome in a refined SCX-based approach."
Anal. Chem. 81:4493-4501(2009) [PubMed] [Europe PMC] [Abstract]

"Large-scale proteomics analysis of the human kinome."
Mol. Cell. Proteomics 8:1751-1764(2009) [PubMed] [Europe PMC] [Abstract]

"Ulk1-mediated phosphorylation of AMPK constitutes a negative regulatory feedback loop."
Autophagy 7:696-706(2011) [PubMed] [Europe PMC] [Abstract]

"AMP-activated protein kinase in metabolic control and insulin signaling."
Circ. Res. 100:328-341(2007) [PubMed] [Europe PMC] [Abstract]

"AMP-activated/SNF1 protein kinases: conserved guardians of cellular energy."
Nat. Rev. Mol. Cell Biol. 8:774-785(2007) [PubMed] [Europe PMC] [Abstract]

"An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."
J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]

"Novel PRKAG2 mutation responsible for the genetic syndrome of ventricular preexcitation and conduction system disease with childhood onset and absence of cardiac hypertrophy."
Circulation 104:3030-3033(2001) [PubMed] [Europe PMC] [Abstract]

"Mutations in the gamma(2) subunit of AMP-activated protein kinase cause familial hypertrophic cardiomyopathy: evidence for the central role of energy compromise in disease pathogenesis."
Hum. Mol. Genet. 10:1215-1220(2001) [PubMed] [Europe PMC] [Abstract]

"Identification of a gene responsible for familial Wolff-Parkinson-White syndrome."
N. Engl. J. Med. 344:1823-1831(2001) [PubMed] [Europe PMC] [Abstract]

Erratum
N. Engl. J. Med. 345:552-552(2001)

Erratum
N. Engl. J. Med. 346:300-300(2002)

"Constitutively active AMP kinase mutations cause glycogen storage disease mimicking hypertrophic cardiomyopathy."
J. Clin. Invest. 109:357-362(2002) [PubMed] [Europe PMC] [Abstract]

"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. 76:1034-1049(2005) [PubMed] [Europe PMC] [Abstract]