Question

1. Would frequent small feedings of cornstarch relieve the symptoms of type 0 glycogen storage disease? Explain.

2. The symptoms of type XI glycogen storage disease include hypoglycemia and hypergalactosemia. What does this tell you about the function of the GLUT2 glucose transport protein?

Answer 1

1) Frequent (every 3-4 hours in the day) small feedings of cornstarch should relieve the symptoms of type 0 glycogen storage disease. Because, type 0 Glycogen Storage Disease would be prevented by preventing low blood sugar (hypoglycemia) and it can be done by avoiding fasting. In our body, frequent (every 3-4 hours in the day) small feedings of cornstarch acts as a "slow release" form of glucose. Hence it may prevent hypoglycemia.

2) GLUT2 is a glucose transport protein. This allows glucose and galactose to exit tissues and enter the blood. But, in type XI glycogen storage disease, GLUT2 is non functional. Hence, glucose can not enter the blood and blood sugar level remains very low (Hypoglycemia). Galactose level remains high in blood (hypergalactosemia) due to some enzyme deficiencies.