Chapter 9:

Question 1

In diabetes mellitus glucose levels tend to be excessively high as a result of insulin resisitance or underproduction. In von Gierke’s disease, or glycogen storage disease type 1, there is a defect in the gluconeogenesis and glycogenolysis pathways. This defect prevents the synthesis of glucose from glucose-6-phosphate by glucose-6-phosphatase (type 1a), or the release of newly synthesized glucose from cells with a specialized membrane transporter (type 1b).

In a person with glycogen storage disease type 1a, what would be most likely to accumulate?

1. Glucose-6-phosphate.
2. Glucose-6-phosphatase.
3. Fructose-6-phosphate.
4. G3P.

Answer 1

1. Glucose-6-phosphate.