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Target intelligence / Profile preview
Protein kinase AMP-activated non-catalytic subunit gamma 2 (PRKAG2)
- Target
- PRKAG2
- Molecular classification
- Enzyme Subunit
01
Overview
The PRKAG2 gene encodes the gamma-2 regulatory subunit of AMP-activated protein kinase (AMPK), a key energy sensor in eukaryotic cells. AMPK monitors cellular energy status and regulates metabolic pathways to restore energy balance during stress. Mutations in PRKAG2 are associated with several cardiac conditions, including Wolff-Parkinson-White syndrome and familial hypertrophic cardiomyopathy. A specific mutation, Arg531Gln (R531Q), causes a severe congenital glycogen storage disease leading to cardiomegaly and heart failure.
- Other names
- 5'-AMP-activated protein kinase subunit gamma-2
02
Mechanism of action
Modulation of AMPK activity
03
Biological functions
Energy metabolism regulationResponse to cellular stressRegulation of AMPK activityCellular energy sensor
04
Disease associations
Wolff-Parkinson-White syndromeFamilial hypertrophic cardiomyopathyProgressive conduction system diseaseArrhythmiasCardiomegalyEarly-onset heart failureGlycogen storage disease
05
Safety considerations
Aberrant AMPK activityPathological glycogen storage within the heart tissue
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