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Old 03-10-2011
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Biochemistry von Gierke's Why Glucose does not work?

Does anyone know why giving glucose or fructose or galactose in von Gierke patient does not raise blood glucose level?
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Old 03-10-2011
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Originally Posted by usluipek View Post
Does anyone know why giving glucose or fructose or galactose in von Gierke patient does not raise blood glucose level?
giving glucose should perfectly raise blood gl level.

galactose forms gal-6-p > with the help of udp gal and udp gl forms gl-6-p(here again,the enzyeme def)

fructose is used in gluconeogenesis from fructose-6-p (after transforming) >gl-6-p .then you need the gl-6-phosphatase enzyme again.


hope it helps.
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Old 03-11-2011
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The enzyme deficient is Glucose 6- phosphatase, the enzyme required by the liver to release glucose in the blood.
if you give fructose or galactose, to these patients, first these sugars need to be converted into glucose by the liver & released into the blood. This would required the enzyme G-6-Phosphatase, which is deficient, so giving these sugars is not going to inc. the blood glucose.

Giving glucose to any body with or without von Gierke's should increase their blood levels.
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Old 03-11-2011
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Thank you all!
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