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. This gene 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. [provided by RefSeq]

protein kinase, AMP-activated, gamma 2 non-catalytic subunit

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.

ATP biosynthetic processManual Assertion Based On ExperimentTAS:BHF-UCL
Cellular response to glucose starvationManual Assertion Based On ExperimentIBA:GO_Central
Fatty acid biosynthetic processIEA:UniProtKB-KW
Glycogen metabolic processManual Assertion Based On ExperimentIMP:BHF-UCL
Intracellular signal transductionManual Assertion Based On ExperimentIMP:BHF-UCL
Negative regulation of protein kinase activityManual Assertion Based On ExperimentIDA:BHF-UCL
Positive regulation of peptidyl-threonine phosphorylationManual Assertion Based On ExperimentIMP:BHF-UCL
Positive regulation of protein kinase activityManual Assertion Based On ExperimentIMP:BHF-UCL
Protein phosphorylationManual Assertion Based On ExperimentIBA:GO_Central
Regulation of catalytic activityManual Assertion Based On ExperimentIBA:GO_Central
Regulation of fatty acid metabolic processManual Assertion Based On ExperimentIMP:BHF-UCL
Regulation of fatty acid oxidationManual Assertion Based On ExperimentTAS:BHF-UCL
Regulation of glucose importManual Assertion Based On ExperimentTAS:BHF-UCL
Regulation of glycolytic processManual Assertion Based On ExperimentIMP:BHF-UCL
Sterol biosynthetic processManual Assertion Based On ExperimentTAS:BHF-UCL

cytoplasm
cytosol
extracellular space
nucleoplasm
nucleotide-activated protein kinase complex
nucleus

Wolff-Parkinson-White syndrome (WPWS):
A supernormal conduction disorder characterized by the presence of one or several accessory atrioventricular connections, which can lead to episodes of sporadic tachycardia.
Cardiomyopathy, familial hypertrophic 6 (CMH6):
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.
Glycogen storage disease of heart lethal congenital (GSDH):
Rare disease which leads to death within a few weeks to a few months after birth, through heart failure and respiratory compromise.

Phosphorylated by ULK1; leading to negatively regulate AMPK activity and suggesting the existence of a regulatory feedback loop between ULK1 and AMPK.