Homo sapiens Protein: PRKAG2
Summary
InnateDB Protein IDBP-49674.6
Last Modified 2014-10-13 [Report errors or provide feedback]
Gene Symbol PRKAG2
Protein Name protein kinase, AMP-activated, gamma 2 non-catalytic subunit
Synonyms
Species Homo sapiens
Ensembl Protein ENSP00000287878
InnateDB Gene IDBG-49672 (PRKAG2)
Protein Structure
UniProt Annotation
Function AMP/ATP-binding subunit of AMP-activated protein kinase (AMPK), an energy sensor protein kinase that plays a key role in regulating cellular energy metabolism. In response to reduction of intracellular ATP levels, AMPK activates energy-producing pathways and inhibits energy-consuming processes: inhibits protein, carbohydrate and lipid biosynthesis, as well as cell growth and proliferation. AMPK acts via direct phosphorylation of metabolic enzymes, and by longer-term effects via phosphorylation of transcription regulators. Also acts as a regulator of cellular polarity by remodeling the actin cytoskeleton; probably by indirectly activating myosin. Gamma non-catalytic subunit mediates binding to AMP, ADP and ATP, leading to activate or inhibit AMPK: AMP-binding results in allosteric activation of alpha catalytic subunit (PRKAA1 or PRKAA2) both by inducing phosphorylation and preventing dephosphorylation of catalytic subunits. ADP also stimulates phosphorylation, without stimulating already phosphorylated catalytic subunit. ATP promotes dephosphorylation of catalytic subunit, rendering the AMPK enzyme inactive. {ECO:0000269PubMed:14722619}.
Subcellular Localization
Disease Associations Wolff-Parkinson-White syndrome (WPWS) [MIM:194200]: A supernormal conduction disorder characterized by the presence of one or several accessory atrioventricular connections, which can lead to episodes of sporadic tachycardia. {ECO:0000269PubMed:11407343, ECO:0000269PubMed:11748095}. Note=The disease is caused by mutations affecting the gene represented in this entry.Cardiomyopathy, familial hypertrophic 6 (CMH6) [MIM:600858]: A hereditary heart disorder characterized by ventricular hypertrophy, which is usually asymmetric and often involves the interventricular septum. The symptoms include dyspnea, syncope, collapse, palpitations, and chest pain. They can be readily provoked by exercise. The disorder has inter- and intrafamilial variability ranging from benign to malignant forms with high risk of cardiac failure and sudden cardiac death. CMH6 patients present Wolff-Parkinson-White ventricular preexcitation, enlarged myocytes without myofiber disarray, and glycogen- containing cytosolic vacuoles within cardiomyocytes. {ECO:0000269PubMed:11371514, ECO:0000269PubMed:11827995}. Note=The disease is caused by mutations affecting the gene represented in this entry.Glycogen storage disease of heart lethal congenital (GSDH) [MIM:261740]: Rare disease which leads to death within a few weeks to a few months after birth, through heart failure and respiratory compromise. {ECO:0000269PubMed:15877279}. Note=The disease is caused by mutations affecting the gene represented in this entry.
Tissue Specificity Isoform B is ubiquitously expressed except in liver and thymus. The highest level is detected in heart with abundant expression in placenta and testis.
Comments
Interactions
Number of Interactions This gene and/or its encoded proteins are associated with 21 experimentally validated interaction(s) in this database.
They are also associated with 1 interaction(s) predicted by orthology.
Experimentally validated
Total 21 [view]
Protein-Protein 20 [view]
Protein-DNA 0
Protein-RNA 0
DNA-DNA 1 [view]
RNA-RNA 0
DNA-RNA 0
Predicted by orthology
Total 1 [view]
Gene Ontology

Molecular Function
Accession GO Term
GO:0004679 AMP-activated protein kinase activity
GO:0004862 cAMP-dependent protein kinase inhibitor activity
GO:0005524 ATP binding
GO:0008603 cAMP-dependent protein kinase regulator activity
GO:0008607 phosphorylase kinase regulator activity
GO:0016208 AMP binding
GO:0019901 protein kinase binding
GO:0030295 protein kinase activator activity
GO:0043531 ADP binding
Biological Process
GO:0005977 glycogen metabolic process
GO:0006110 regulation of glycolytic process
GO:0006112 energy reserve metabolic process
GO:0006468 protein phosphorylation
GO:0006469 negative regulation of protein kinase activity
GO:0006633 fatty acid biosynthetic process
GO:0006754 ATP biosynthetic process
GO:0006853 carnitine shuttle
GO:0007050 cell cycle arrest
GO:0008286 insulin receptor signaling pathway
GO:0010800 positive regulation of peptidyl-threonine phosphorylation
GO:0016126 sterol biosynthetic process
GO:0019217 regulation of fatty acid metabolic process
GO:0035556 intracellular signal transduction
GO:0042304 regulation of fatty acid biosynthetic process
GO:0044255 cellular lipid metabolic process
GO:0044281 small molecule metabolic process
GO:0045860 positive regulation of protein kinase activity
GO:0046320 regulation of fatty acid oxidation
GO:0046324 regulation of glucose import
GO:0050790 regulation of catalytic activity
GO:0061024 membrane organization
GO:0071901 negative regulation of protein serine/threonine kinase activity
Cellular Component
GO:0005615 extracellular space
GO:0005654 nucleoplasm
GO:0005829 cytosol
GO:0031588 AMP-activated protein kinase complex
Protein Structure and Domains
PDB ID
InterPro IPR000644 CBS domain
PFAM PF00571
PRINTS
PIRSF
SMART SM00116
TIGRFAMs
Post-translational Modifications
Modification
Cross-References
SwissProt Q9UGJ0
PhosphoSite PhosphoSite-Q9UGJ0
TrEMBL C9JUG1
UniProt Splice Variant
Entrez Gene 51422
UniGene Hs.649300
RefSeq NP_057287
HUGO HGNC:9386
OMIM 602743
CCDS CCDS5928
HPRD 04119
IMGT
EMBL AB025580 AC006358 AC006966 AC074257 AC093583 AF087875 AJ249976 AK001887 BC020540 BC068598 BT007127
GenPept AAF03528 AAH20540 AAH68598 AAK00413 AAP35791 AAS02032 BAA84695 BAA91962 CAB65116