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Old 12-24-2011
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Biochemistry Lactic acidosis in von gierke

Why is there lactic acidosis in von gierke's disease?

Also why does administration of fructose increase blood glucose? Does it do it by means of gluconeogenesis?
Pls some one explain.
Thanks
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Old 12-24-2011
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Default hi indigo

hi, let me try my hands at this:

lactate can be converted to pyruvate which then continues into gluconeogenesis (cori cycle?) at some point in this process glu-6-phosphate is formed which should be broken down by glu-6-phosphatase which is lacking in von girkes. the bluid up of gluc-6-phosphatase will tell lactate dehydrogenase that theres no need to generate pyruvate from lactate which then builds up.

yur second question: i have a question myself does fructose increase blood gluc? i am not sure. however if it does then it does this by gluconeogenesis.
if you look at FA 2011 edition, page 103, the metabolism of fructose generates DHAP and glyceraldehyde. glyceraldehyde can be converted to glycerol. This glycerol is instead shuttled to the liver via the blood where it is:
  • phosphorylated by glycerol kinase to glycerol phosphate
  • converted to DHAP (dihydroxyacetone phosphate) which can participate in glycolysis or gluconeogenesis.
or maybe it does not even need to become glycerol phosphate, remember that DHAP and glyceraldehyde-3-p in the glycolytic pathway can just simply go back to become fruc-i,6-p in gluconeogenesis.

i hope i have made a little sense, i think i may have made it worse?
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Old 12-25-2011
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Thanks mate I think its a wonderful explanationMerry Christmas!!!
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Old 09-11-2012
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Default

Quote:
Originally Posted by indigo View Post
Why is there lactic acidosis in von gierke's disease?

Also why does administration of fructose increase blood glucose? Does it do it by means of gluconeogenesis?
Pls some one explain.
Thanks
Administration of Fructose or Galactose does NOT increase blood glucose. There is no G6 phosphatase !!!! Instead it causes lactic acidosis.

Source: UW Q
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sruthi (09-12-2012)



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