Glycogen Storage Disease

[MDApplicant88]

Advertisement - Members don't see this ad

For Type 0 disease the enzyme affected is the glycogen synthase. One of the manifestations is hypoglycemia. How does this happen? I would think that if glycogen synthase is defected glucose cannot be stored into glycogen and so glucose accumulates. Wouldn't that cause hyperglycemia?

 

[jfgavina]

You can't accumulate glucose! Its is either canalized to glycolysis and further oxidative phosphorylation, fat synthesis or glycogen synthesis. If you don't have the ability to store it as a form of glycogen when you're in the fasting state obviously you're going to get hypoglycemia because you can't make glucose out of glycogen! I didn't studied that disease, it is only a matter of logic

 

[Phlame217]

What glucose is available is going to get sequestered into cells due to insulin.

Without glycogen, the liver is severely limited on its ability to proceed in gluconeogenesis which leads to hypoglycemia.