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FLASH GENE
Symbol PCK2 contributors: mct - updated : 09-11-2014
HGNC name phosphoenolpyruvate carboxykinase 2 (mitochondrial)
HGNC id 8725
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
HOMOLOGY
interspecies homolog to murine Pck2
homolog to C.elegans r11a5.4
Homologene
FAMILY phosphoenolpyruvate carboxykinase (GTP) family
CATEGORY enzyme
SUBCELLULAR LOCALIZATION     intracellular
intracellular,cytoplasm,organelle,mitochondria,matrix
basic FUNCTION
  • involved in gluconeogenesis
  • forming phosphoenolpyruvate by decarboxylation of oxaloacetate
  • catalyses the irreversible conversion of oxaloacetate to phosphoenolpyruvate, an important initial step in hepatic gluconeogenesis
  • its enzymatic activity is half that of primary hepatocytes and is localized exclusively to the mitochondria
  • strong candidate to link mtGTP synthesis with insulin release through anaplerotic phosphoenolpyruvate cycling
  • possible role for PCK2 and G6PC in the DNA damage response
  • can play, as the cytosolic isoenzyme, an active role in glyceroneogenesis and gluconeogenesis
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism carbohydrate
    signaling
    a component
    INTERACTION
    DNA
    RNA
    small molecule metal binding, nucleotide,
  • GTP
  • has an absolute requirement for Mn2+ ions for activity
  • protein
  • PPARG01A, a critical transcription factor for PEPCK gene expression, is decreased upon UV-irradiation
  • cell & other
    REGULATION
    activated by ER stress triggers suppression of AMPK while increasing CEBPB and CREB1 expression which activates PCK2 gene transcription
    induced by glucagon
    repressed by insulin
    ZNF692 (represses gene expression of PCK2, a key enzyme of gluconeogenesis
    Other regulation of gene expression in the liver by KLF15
    ASSOCIATED DISORDERS
    corresponding disease(s) PCK2D
    related resource MITOP database
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional     --low  
    in hypoglycemia and liver impairment
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS