Von Gierke's disease primarily affects which metabolic process?

Von Gierke's disease, also known as glycogen storage disease type I, primarily affects gluconeogenesis. This condition is caused by a deficiency of the enzyme glucose-6-phosphatase, which is involved in the last step of gluconeogenesis and glycogenolysis. In individuals with this disease, the inability to convert glucose-6-phosphate to glucose leads to an accumulation of glycogen and a deficiency of free glucose in the bloodstream, particularly between meals.

The impact on gluconeogenesis is particularly significant because this pathway allows the body to produce glucose from non-carbohydrate sources, maintaining blood sugar levels during fasting. The inability to properly perform gluconeogenesis results in symptoms such as hypoglycemia, lactic acidosis, and hyperlipidemia, which are characteristic of Von Gierke's disease.

While other processes like glycolysis, glycogenesis, and glycogenolysis are also affected to some degree due to the metabolic dysfunction, it is the impairment of gluconeogenesis that is most central to the pathology of the disease. Therefore, understanding Von Gierke's disease involves recognizing its primary disruption in gluconeogenesis and how that impacts overall metabolic homeostasis.