Glycogen children with

This reaction is important because it provides oxaloacetate for the citric acid cycle when the supplies have run low because of the demands of biosynthesis. It is also the enzyme that catalyzes the first step in gluconeogenesis, the pathway that provides the body with needed glucose in times of starvation or periods of exercise that deplete glycogen stores. But somehow these descriptions don t fill us with a sense of the importance of this enzyme and its jobs. It is not until we investigate a case study of a child born with pyruvate carboxylase deficiency that we see the full impact of this enzyme. 

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