Phosphoenolpyruvate Carboxykinase Deficiency

Phosphoenolpyruvate carboxykinase is an important enzyme in gluconeogenesis. It is found in both the cytosol and mitochondria of the liver cells. The enzyme is regulated by insulin, glucocorticoids, cyclic adenosine monophosphate (cAMP) and diet to maintain glucose homeostasis.


This is a companion discussion topic for the original article at https://patient.info/doctor/congenital-disorders/phosphoenolpyruvate-carboxykinase-deficiency