PCK1 is a main control point for the regulation of gluconeogenesis. It is a cytosolic enzyme, along with GTP, that catalyzes the formation of phosphoenolpyruvate from oxaloacetate, with the release of carbon dioxide and GDP. The expression of PCK1 can be regulated by insulin, glucocorticoids, glucagon, cAMP, and diet. Defects in PCK1 are a cause of cytosolic phosphoenolpyruvate carboxykinase deficiency. A mitochondrial isozyme of this protein also has been characterized (1).
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