pyruvate dehydrogenase deficiency treatment

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StringBean

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So I was hoping someone out there could explain why the Tx for pyruvate dehydrogenase deficiency is an increase intake of ketogenic nutrients (high fat content). I know it's due to some simple link in the pathway but I can't seem to see it in my head (or on paper for that matter... Biochem is def my weakness).

Thanks 😀
 
hey...

Ketogenic AA feeds into the TCA via acetyl coa. Thus, it bypass Pyruvate and the pyruvate dehydrogenase. If you want a source, look at Lippincott's Bioch. the chap on AA metabolism...it has a little chart that shows you where and how all the AA's enter the TCA cycle.

hope this helps.
 
if i was that patients physician i would inject some pyruvate DH in his butt......that will make him feel better
 
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