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Old 12-29-2011
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Biochemistry Pyruvate kinase deficiency and ketogenic diet

just I cant understand why in FA, it says that if we have pyruvate kinase def . we treat by increase intake of ketogenic nutrients (ketogenic amino acids as leucine and lysine????
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Old 12-29-2011
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Because pyruvate kinase deficiency, so less pyruvate produced and also less acetyl-CoA produced from pyruvate. In this case, cell cannot generate enough ATP by running the citric cycle since the entering substance is scare. By giving ketogenic nutrients such as ketogenic amino acids, cell can use these items to produce acetyl-CoA which then could enter citric cycle to generate ATP. That's a way to compensate for defect in glycolysis pathway.
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Old 12-29-2011
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Default a little correction

It's pyruvate dehydrogenase deficiency not pyruvate kinase.
For more information read here;
Why does ketogenic amino acids help pyruvate dehydrogenase deficiency?
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Old 12-29-2011
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Quote:
Originally Posted by nativedoc2 View Post
its pyruvate dehydrogenase def not pyruvate kinase
PDH deficiency also contributes to less sufficient amount of acetyl-CoA generated, almost results in same consequence since the two enzymes function in the same pathway.
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Old 12-29-2011
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I think it should be PDH rather than Pyruvate kinase.Ketone bodies do increase acetyl co A bt remember one thing the problem in pyruvate kinase def. is haemolytic anemia and since in RBC there is no krebs cycle the ketone bodies will still nt produce energy.
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Old 12-30-2011
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Quote:
Originally Posted by mohitkmc View Post
I think it should be PDH rather than Pyruvate kinase.Ketone bodies do increase acetyl co A bt remember one thing the problem in pyruvate kinase def. is haemolytic anemia and since in RBC there is no krebs cycle the ketone bodies will still nt produce energy.
RBCs don't rely on krebs cycle to produce energy. Even if there is no ATP produced from RBCs, cells other than RBCs can still produce energy.
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Old 12-30-2011
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Quote:
Originally Posted by belindalimm View Post
RBCs don't rely on krebs cycle to produce energy. Even if there is no ATP produced from RBCs, cells other than RBCs can still produce energy.
Ya I knew abt that like for eg-liver which will be the main source of providing glucose bt if there will be Pyruvate kinase deficience it will also not be useful in preventing haemolysis by 2 methods 1. absence of one of the steps of substrate leve; phosphorylation 2.Increase in 2,3-BPG
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