firecracker: hereditary fructose intolerance

[quickfeet]

Why do people with HFI get hypoglycemia?

"A deficiency in aldolase B leads to accumulation of phosphorylated fructose → available phosphate levels drop → gluconeogenesis and glycogenolysis are blocked."

Is this correct?

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[Apoplexy__]

That's what FA says too.

The metabolic step that gets blocked in fructose metabolism is right after the investment of an ATP, such that when the body tries to metabolize fructose, it just keeps tacking on these phosphates without ever getting any energy from it. Apparently, this phosphate investment is so substantial that it translates to an inability to invest the necessary ATP and phosphate groups to perform gluconeogenesis and glycogenolysis.

This is also why you don't see hypoglycemia in benign fructosuria, since the deficient step is the one that actually puts on the ATP (fructokinase).

And it's also why you get hypoglycemia in that one form of galactose intolerance.

 

[DrEnderW]

You should just post this in the FC thread instead of making a new thread every time you aren't sure about something.

Everyone on there will be familiar with the content and answer you.

 

[seminoma]

Why do people with HFI get hypoglycemia?

"A deficiency in aldolase B leads to accumulation of phosphorylated fructose → available phosphate levels drop → gluconeogenesis and glycogenolysis are blocked."

Is this correct?

Yes, it's correct. No phosphate = no ability to make glucose.