why won't Galactose infusion increase the blood glucose in von Gierke disease?

[ketap]

hi, i am really confuse....is it true that galactose or fructose infusion or glucagon and epinephrine infusion won't increase the blood sugar in von gierke's patients?
why?
thx u

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[ABQ9988]

The final step in all those pathways is blocked. You can't convert G6P to glucose via G6Phosphatase.

 

[mmmcdowe]

The whole point of putting a phosphate on a glucose is phosphate charged glucose cannot exit the cell through the membrane due to charge. This gives the cell time to either store the g6p or utilize it in glycolysis.

 

[SOML]

The final step in all those pathways is blocked. You can't convert G6P to glucose via G6Phosphatase.

exactly. defective glycogenolysis / gluconeogenesis from deficient G6Pase

The whole point of putting a phosphate on a glucose is phosphate charged glucose cannot exit the cell through the membrane due to charge. This gives the cell time to either store the g6p or utilize it in glycolysis.

Yes that's true, also the conversion of G --> G6P formation effectively ↓ intracellular glucose concentrations thereby driving more glucose into the cell.. but the OP's query was as to why glucagon etc. wouldn't increase glucose levels in the case of V.Gierke's

 

[ketap]

hi, just realize how stupid i am for asking this, but now i understand after i reviewed your answers and the glycogen and glycolisis again..thx u very much,friends..