phosphoenolpyruvate carboxykinase (GTP)

phosphoenolpyruvate carboxykinase (GTP)
phos·pho·enol·py·ru·vate car·boxy·ki·nase (GTP) (fos″fo-e″nol-piґroo-vāt kahr-bok″se-kiґnās) [EC 4.1.1.32] an enzyme of the lyase class that catalyzes the decarboxylation and phosphorylation of oxaloacetate to form phosphoenolpyruvate, using GTP as a phosphate donor. The enzyme occurs in both the mitochondria and cytosol of mammalian liver, and the reaction is part of the mechanism of gluconeogenesis. Deficiency of the enzyme in mitochondria or cytosol, an autosomal recessive trait, causes infant hypoglycemia. Called also phosphopyruvate carboxykinase.

Medical dictionary. 2011.

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